Brain Injury and Medical Malpractice

The prevalence of brain injury in the United States is alarming as it is the second leading cause of disability in the country. Often referred to as the silent epidemic, approximately 3.17- 5.3 million Americans suffer from traumatic brain injuries, another 4.7 million have brain injuries from strokes, and another 500,000 have cerebral palsy (brain injury due to an event of oxygen deprivation). Causes of brain injury that may give rise to a medical malpractice lawsuit are further described below: 

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Brain Injury from Birth: a medical malpractice lawsuit may arise may when a child's brain is negligently deprived of oxygen during pregnancy, labor and delivery. This may result in the child later developing cerebral palsy, mental retardation, seizures, blindness, deafness, and learning disabilities. Oxygen deprivation that injures a baby's brain may arise from any of the following events: 

*Compression of the umbilical cord during delivery

*Maternal Infection present during the pregnancy or delivery

*Placental abruption or uterine rupture prior to birth

*Maternal high blood pressure during the pregnancy (preclampsia)

*Breeched vaginal position of the baby (feet first rather than head first)

*Improper administration or doasage of epidural or labor inducing drugs during the delivery

*Failure to timely perform an emergency c-section

*Fetal macrosomia (oversized baby) unable to navigate the birth canal 

Brain Injury in Adults and Children: a medical malpractice lawsuit may arise as a result of errors in diagnosis and treatment of a serious medical condition. A few of the causes of brain injury in children and adults that may involve medical malpractice include: 

*Medication errors

*Anesthesia errors

*Surgical errors

*Radiology errors 

*Emergency room errors

*Delay in diagnosis/treatment of heart attack or cardiac arrest

*Delay in diagnosis/treatment of a stroke, aneurysm, or blood clot

*Delay in diagnosis/treatment of meningitis or encephalitis

*Delay in diagnosis/treatment of a spreading infection or abscess

*Delay in diagnosis/treatment of internal bleeding

*Delay in diagnosis/treatment of hydrocephalus 

*Delay in diagnosis/treatment of diabetes/diabetic coma/insulin shock

In addition to the above mentioned causes of brain injury, many other errors involving patient treatment and care may give rise to a lawsuit.  One of the most common causes occurs when a hospital patient or nursing home patient falls (due to inadequate protective measures) and the patient suffers a traumatic brain injury.  In fact, falls are the leading cause of traumatic brain injury in our country surpassing even motor vehicle accidents. For a detailed guide to the incidence, prevalence, and epidemiology of brain injury, see The Essential Brain Injury Guide prepared under the auspices of the Brain Injury Association of America.     

Heart Defects In Newborns

Heart defects in newborns affect approximately 8 out of every 1000 births.  Causes can include genetic factors, such as those found in Down’s syndrome, or factors due to the developing baby’s environment. Certain medications, such as Paxil (Paroxetine), Zoloft (Sertraline), Wellbutrin (Bupropion), or Prozac (Fluoxetine) may be associated with cardiac birth defects in the babies of mothers who took these medications when pregnant. While some heart defects may only slightly affect a child’s quality of life, others can be very serious and may require surgical correction. Although causes of many heart defects are not known, improvements in prenatal diagnosis has led to better management and outcomes for those babies affected by these conditions.

The most common way to diagnose a cardiac defect prenatally is by sonogram. Many obstetricians order a screening sonogram for their patients during the 16th to 20th week of pregnancy. Part of the standard practice for the sonographer is to identify the four chambers of the baby’s developing heart.

If the four chambers are not seen, or appear abnormal, a more specialized test, called a fetal echocardiogram may be ordered. This test involves a more detailed sonogram examination of the developing baby’s heart. Abnormalities can be further evaluated by this test method.

There are many different cardiac anomalies that affect newborns. Only three (3) will be discussed here:

Ventricular Septal Defect (VSD)- refers to a hole between the ventricles, the two lower chambersof the heart. The mixing of the oxygenated and deoxygenated blood through the defect can cause symptoms. Sometimes, a small VSD may close up on its own; sometimes surgery is required.

Atrial Septal Defect (ASD)- involves a hole in the wall separating the atria, the upper chambers of the heart.

Pulmonary Stenosis- occurs when the pulmonary artery that leads from the right ventricle to the lungs is narrowed. This can obstruct this crucial bloodflow out of the heart and can cause the right ventricle to become over developed and enlarged in trying to overcome the obstructive narrowing.

If proper prenatal testing was not done, or if testing was not read or interpreted properly, medical malpractice may have occurred.  Allegations of medical malpractice may include a negligent delay in diagnosis and/or inadequate surgical treatment and care of the newborn's heart defect.   Lawsuits may also result if a baby was injured by medications used by the mother. Cardiac defects can cause serious health problems in the child and may also involve major surgery for correction. In addition, complications can occur due to the defect, especially if undiagnosed. It is very important that women at risk for having a baby with a heart defect have appropriate screening tests done. Risk factors for congenital heart defects may include prenatal exposure to the medications listed above. Also, factors such as a sibling or family history of congenital heart defects may warrant increased surveillance and testing during the pregnancy.

Increased Fluid Around the Heart May Cause Cardiac Tamponade

Pericardial effusion occurs when there is an abnormal amount of fluid around the heart.  The heart is normally surrounded by a thin membranous sac called the pericardium.  The space between the pericardium and the muscle that is the heart is referred to as the perciardial space.  Normal levels of pericardial fluid within the pericardial space are from 15 to 50 mL, or about 1-3 tablespoons.

iStock_000003834803XSmall.jpgAn effusion, therefore, represents  an abnormal accumulation of fluid in the pericardial space.   Because of the limited amount of space in the pericardial cavity, fluid accumulation will lead to increased intrapericardial pressure and this can negatively affect heart function.  Cardiac tamponade occurrs when there is a  large enough pericardial effusion causing enough pressure to adversely affect heart function.  This is an emergent life threatening condition.

Pericardial effusion symptoms may include difficulty breathing (dyspnea), shortness of breath when lying down (orthopnea), chest pain, cough, dizziness, low grade fever, rapid heart rate (tachycardia), and a feeling of anxiety  

Pericardial effusion may be caused by:

-a disturbed equilibrium between the production and re-absorption of pericardial fluid,

-a structural abnormality that allows fluid to enter the pericardial cavity

-inflammation of the pericardium (pericarditis)

-bacterial or viral infections

-injury to the heart from a medical procedure

-cancer

-heart attack

-autoimmune disorders

Unfortunately, pericardial effusion and cardiac tamponade may result from improper placement of a central venous catheter during a medical procedure involving an infant.  This may arise when there is an inadvertent perforation into the pericardial space by the CVC and fluids are artificially infused into the space thereby causing the tamponade. Upon recognition of this situation, emergent removal of this fluid via a needle inserted through the chest wall and into the pericardial space (pericardiocentesis) can improve the infant's chance of survival. It is suggested that routine radiography be performed to readily identify the CVC tip in all cases when these lines are placed into babies.    Increased awareness of this complication may decrease the mortality associated with CVC related pericardial effusions.   

Treatment depends on the underlying cause and the severity of the heart impairment. Pericardial effusion due to a viral infection sometimes goes away within a few weeks without treatment.  Some pericardial effusions remain small and never need treatment.  If the pericardial effusion is due to an autoimmune condition treatment with anti-inflammatory medications may help. If the effusion is compromising heart function and causing cardiac tamponade, it will need to be drained, most commonlyby a pericardiocentesis.  In some cases, surgical drainage may be required by cutting through the pericardium creating what is referred to as a pericardial window

Small Bowel Obstruction and Medical Malpractice

The small bowel is a long coiled hollow tube, called a tract, that is approximately twenty-five feet long. It includes the duodenum, jejunum and ileum.  A small bowel obstruction, also known as a small intestinal obstruction, is a mechanical or functional (paralytic) blockage of the intestinal tract, which prevents the normal transit of digestive products. It can occur at any level throughout the jejunum and ileum, and is considered a medical emergency when it occurs. The condition is often treated conservatively for the first several days; however, the patient must be monitored very closely for signs of clinical deterioration that can become life threatening.

iStock_000017493981XSmall.jpgMechanical obstruction is due to a mechanical barrier, such as an adhesive band from prior surgery, which creates a road block to the bowel.  On the other hand, functional obstruction is caused by an event that interferes with the nervous innervation of the bowel, such as electrolyte imbalances and metabolic disturbances. Functional bowel obstruction can be caused by a multitude of conditions whereas mechanical SBO is generally credited to a luminal, mural, or extra-mural mechanical barrier. A clinical syndrome exists called small intestinal pseudo-obstruction, which is characterized by manifestations of mechanical bowel obstruction in the absence of an obstructive lesion.

The symptoms of a mechanical small bowel obstruction include abdominal fullness and/or excessive gas, abdominal distention, pains and cramps in the stomach area (specifically the mid abdomen), vomiting, constipation (inability to pass gas or stool), diarrhea, and bad breath. Acute functional small bowel dilatation is referred to as adynamic or paralytic ileus. The symptoms of paralytic obstruction, in reference to the ileus, are abdominal fullness and/or excessive gas, abdominal distention, and vomiting after eating.  The pain less closely resembles the colicky type seen in mechanical obstruction, but may be just as severe

The diagnosis is determined by listening to the abdomen with a stethoscope. High-pitched, tinny and clanking sounds can be heard at the onset of mechanical obstruction.  If the blockage persists for too long or the bowel is significantly damaged, due to the stretching of the blood vessels supplying it thereby decreasing blood flow, bowel sounds will decrease and eventually become silent.  The hallmark of paralytic ileus is decreased or absent bowel sounds, which can create confusion in relation to the issue of etiology if this occurs.Diagnostic tests that demonstrate obstruction include plain radiographic film of the abdomen (usually in the flat and upright position), CT scan, barium enema and upper GI series with small bowel follow through

Treatment depends on the cause of the obstruction. In some cases, drastic measures are necessary to save a person’s life, while in others a strategy of watchful waiting is more appropriate. In general, more serious cases that require immediate treatment can be identified based on a patient’s vital signs and physical exam. If the person is very sick and appears to be on the brink of a serious event, surgery may be required to ensure the patient’s life.

To determine if there is any deterioration consistent with lack of blood flow, which leads to bowel ischemia, gangrene, perforation, septic shock, and death, it is imperative that the following steps be taken. The bowel must be decompressed with a long indwelling tube, all oral feeding must be stopped and IV therapy must be initiated with continuous monitoring and observation. Generally speaking, there is no reason anyone presenting to the emergency room with a small bowel obstruction should die in the hospital unless there are extenuating circumstances.

Deep Venous Thrombosis and Medical Malpractice

Deep venous thrombosis is the development of a blood clot in the large, deep veins of the lower leg and thigh. Thrombi can cause tissue injury due to vascular occlusion or distal embolization. However, venous obstruction can be offset by collateral blood vessels. Thrombi can also cause local pain and edema due to the blockage of blood flow. If the clot breaks off and travels through the blood, it is referred to as an embolism. An embolism can become trapped in the brain, lungs, or heart leading to major injury. Pulmonary embolus (PE) is a common complication and life threatening if not treated quickly with anticoagulants. PE presents with shortness of breath, chest pain, and cough with blood in sputum

iStock_000012053156XSmall.jpgDVT can occur with stasis or in hypercoaguable states. It is commonly seen following trauma, surgery, or burns, which contribute to decreased physical activity, damage to vessels, and release of procoagulant substances from tissues. Reduced physical activity causes a decline in the milking action of lower leg muscles and slows venous return. Risk factors for DVT include advanced age, bed rest, immobilization, smoking, birth control pills, family history of blood clots, fractures in the pelvis or legs, giving birth within the last 6 months, heart failure, and obesity.  To prevent DVT, patients should move their legs during long flights or when they are immobile for long periods of time.

Although many DVTs are asymptomatic, they can recur. Some individuals suffer from post-phlebitic syndrome, which involves chronic pain and swelling in the leg. The major symptoms of DVT include changes in a patient’s leg such as redness, increased temperature, pain, and tenderness. Diagnosis is based on the physical exam, which should demonstrate a red, swollen leg. Diagnostic tests include a D-dimer blood test along with other blood tests to check for hypercoagulability such as activated protein C resistance, anti-thrombin III levels, antiphospholipid antibodies, and genetic testing for mutations with a predisposition towards blood clots. Imaging studies of the legs include Doppler ultrasound, plethysmography, and radiography.

The primary treatment for DVT is anti-coagulants, also known as blood thinners. They prevent the formation of new clots and the growth of old clots. However, they cannot dissolve existing clots. Patients are more likely to bleed on these medications. Heparin is an IV administered anticoagulant given in a hospital setting.  Warfarin (Coumadin) is an oral anticoagulant that takes several days to work; thus, Heparin cannot be stopped until Warfarin is functioning at an effective dose for a minimum of two days. Many patients wear pressure stockings on their legs to improve blood flow and decrease their risk of DVT.  When medications are ineffective, patients may need to undergo surgery. A filter can be placed in the body’s largest vein to prevent thrombi from migrating to the lungs. Also, surgery may be necessary to remove large thrombi.  

Meningitis and Medical Malpractice

Meningitis is inflammation of the meninges, the membranes that cover the brain and spinal cord. It is commonly caused by infection, but other causes include chemical irritants, drug allergies, fungi, and tumors. Based on the clinical evolution of the illness and the type of inflammatory exudate present in the cerebral spinal fluid (CSF), infectious meningitis is categorized into acute pyogenic (usually bacterial), aseptic (usually viral), and chronic (usually tuberculosis, spirochetal, cryptococcal).

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Early diagnosis is essential for bacterial meningitis because it can result in death or brain damage if left untreated. In bacterial meningitis, a correlation exists between bacterial organism and age. The most likely organism in neonates may be Escherichia Coli or group B Streptococci. In the elderly, it may be Streptococcus Pneumonia or Listeria Monocytogenes. In young adults, it may be Neisseria Meningitides. In contrast, most viral infections are due to enteroviruses but only a small number of people who develop enteroviral infections present with meningitis. Other viral infections that can cause meningitis include mumps, herpes virus, measles, and influenza. Chronic meningitis can be caused by pathogens such as mycobacteria and spirochetes. Thus, medical attention is necessary to differentiate between bacterial, viral, and chronic meningitis.

Risk factors include individuals over the age of 60 or below the age of 5, diabetes mellitus, renal or adrenal insufficiency, hypoparathyroidism, cystic fibrosis, immunosuppression, HIV, crowding (military recruits and college residents), recent exposure to those with meningitis, etc. The symptoms have a rapid onset and include fever, chills, mental status changes, nausea, vomiting, photophobia, severe headache, and meningismus (stiff neck). Additional symptoms include agitation, bulging fontanelles, decreased consciousness, tachypnea, poor feeding or irritability in children, and opisthotonos (unusual posture, with head and neck arched backwards).

To confirm a diagnosis, a lumbar puncture (spinal tap) should generally be performed on anyone suspected of meningitis to sample and culture the CSF for abnormal cell counts, glucose, and protein. Other diagnostic tests include blood culture, chest x-ray, and MRI or CT scan of the head. The underlying cause of the meningitis needs to be determined to administer proper treatment and define the severity of each case. Unlike bacterial meningitis, viral meningitis usually does not involve treatment and patients generally recover within two weeks; however, in certain instances (such as with the herpes simplex virus) antiviral medications may be indicated.

Antibiotic treatment for bacterial meningitis is dependent on the underlying bacterium. By treating the most common types, the risk of dying is reduced to below 15%. Symptoms such as brain swelling, shock, and seizures are treated with other medications and intravenous fluids. Possible complications of meningitis include brain damage, subdural effusion, hearing loss, hydrocephalus, and seizures. To prevent contraction of meningitis, the meningococcal vaccination is recommended for populations at risk.

 

Oral Cancer and Medical Malpractice

Oral cancer is the uncontrollable growth of cells that invade and inflict damage in areas involving the lips, tongue, cheek lining, floor of the mouth, gingiva, and palate. Men are twice as likely to have oral cancer, specifically men over the age of 40. Risk factors include smoking/tobacco use; heavy alcohol use; chronic irritation from rough teeth, dentures, or fillings; human papilloma virus infection; family history; excessive sun exposure; taking immunosuppressants that weaken the immune system; and poor oral hygiene. Oral cancer can present with a sore, lump, or ulcer in the mouth that initially is painless; however, as the cancer progresses it may evolve into a burning sensation or pain. The area may appear pale colored or it can be dark and discolored. Other symptoms include dysphagia (difficulty swallowing), odynophagia (painful swallowing), chewing problems, speech difficulties, lymphadenopathy (swollen lymph nodes), and weight loss.

iStock_000018882292XSmall.jpgTo diagnose oral cancer, your physician or dentist will examine your oral cavity and if a suspicious lesion is identified then an oral brush biopsy may be performed. The test is painless and involves isolating and analyzing a small sample of tissue for abnormal cells. However, if the lesion is more concerning then a scapel biopsy is recommended to determine whether the area is malignant or benign. X-rays and CT scans may be utilized to determine if the cancer has metastasized. Other tests that may be conducted include endoscopy, barium swallow, or PET scan. 90% of oral cancers are squamous cell carcinomas. Squamous cells are thin, flat cells that line the lips and oral cavity. Squamous cell carcinoma often develops in areas of leukoplakia, white patches of cells that do not rub off. Other types of oral cancer include adenocarcinoma, lymphoma, melanoma, or teratoma.

If oral cancer is not diagnosed early, it can be life threatening. Treatment is dependent on the stage of the cancer which is determines by tumor size, lymph node involvement, and metastatsis. Surgery is recommended if the tumor is small enough and has not spread to the lymph nodes. Complications of surgery include disfigurement of the face, head, and neck. Often surgery is combined with radiation. Complications of radiation include dry mouth and dysphagia. When dealing with larger tumors, chemotherapy is recommended. Speech therapy is also essential to improve and retain movement, chewing, swallowing, and speech.

Depending on the presentation of the oral cancer, approximately 50% of individuals with oral cancer may survive greater than 5 years following diagnosis and treatment. If the cancer is identified early before significant metastasis, then the cure rate may be almost 90%; nevertheless, the majority of oral cancers have metastasized prior to diagnosis. One in four individuals with oral cancer will die due to delayed diagnosis and treatment. To prevent oral cancer, individuals should avoid smoking/tobacco, moderate or avoid alcohol use, and practice good oral hygiene

Advanced Diagnostic Imaging for Acquired Brain Injury

One of the first steps in evaluating brain injury is diagnostic imaging. Imaging refers to various methods of viewing the structures and processes residing in the brain. Some of the more familiar modalities are CT (or CAT) scans, which use X-rays to evaluate intracranial structures. MRI, Magnetic Resonance Imaging, uses magnetic fields to illustrate the brain. However, in cases of traumatic brain injury (TBI), more advanced methods may be needed for proper diagnosis.

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An MRI machine can use special software to perform a brain scan called Diffusion Tensor Imaging (DTI). This scan detects the diffusion of water across brain cells and highlights certain areas that may be associated with injury. These injuries may not be apparent on conventional MRI’s.

PET scan (Positron Emission Tomography) measures uptake and metabolism of glucose from a small radioactive “tagged” sample injected into the patient.  The scanner monitors this sample as the brain utilizes it. The metabolic uptake and usage may differ in patients who have suffered a brain injury. This helps define the extent and type of injury.

In SPECT (Single Photon Emission Computed Tomography), the tagged sample is not absorbed or utilized in the brain cells. Instead, it remains in the blood stream and demonstrates the blood movement or perfusion through the brain. Areas of brain injury or damage may not have normal blood flow so the SPECT scan helps define these areas.

Brain injuries can result from medical malpractice. A baby may suffer birth trauma during labor and delivery. A child may have a concussion or sports injury misdiagnosed or improperly evaluated by a physician. An individual injured in a car accident may not have the indicated testing done by the emergency room. Although scientific progress in imaging studies has improved the ability to diagnose and evaluate brain injuries, these tests need to be utilized in the appropriate situations so patient results and outcomes can improve.  

Pulmonary Embolism and Medical Malpractice

Pulmonary embolism (PE) is a life threatening condition that affects over 600,000 people per year in the United States. Through the use of modern diagnostic tools such as multislice spiral CT and advancements in treatment, the mortality rate of PE has been reduced in recent years.

iStock_000000529518XSmall.jpgPE involves a blockage of an artery in the lungs from a clot that has traveled through the bloodstream from another part of the body. Usually the clot begins in the deep veins of the legs where it is referred to as deep vein thrombosis or DVT. The risk of PE increases during periods of immobilization such as long plane flights, surgery, pregnancy, and with conditions such as cancer and obesity. Additionally, estrogen based hormonal contraception (birth control) may increase the risk of PE.

Signs and symptoms of PE may include chest pain, shortness of breath, abnormal EKG (such as inverted T-waves), rapid heart rate, and increased respirations. PE may be preceded by leg pain due to DVT. A low grade fever may be present as well as a cough or hemoptysis (coughing up blood). If PE is suspected, a D-dimer blood test should be performed which measures the level of specific clotting related protein fragments in the blood.  The D-dimer test can help rule out PE if the blood test comes back within normal limits. However, If the D-dimer blood test comes back elevated for suspected PE, then radiographic imaging is indicated.

When available for imaging, the multi-slice spiral CT is favorable because it is non-invasive and is highly predictive of PE. If the patient has leg pain or other signs of DVT, then a ultrasound (doppler) of the veins in the legs may be performed as there is a high correlation between DVT and PE. But a negative leg doppler does not rule out PE. Other studies to help diagnose PE include include ventilation perfusion scanning and CTPA (CT Pulmonary Angiography-with contrast).

Treatment for PE depends on the severity or size of the blockage. For severe cases that are emergent, thrombolysis may be the preferred treatment- this involves the administration of clot busting medication such as tPA. In specific situations of severe emergent PE, surgery (embolectomy) may be an option to remove the clot. In most cases of PE, anticoagulants such as heparin and warfarin are started early as possible and used to treat the condition. Heparin or LMWH (low molecular weight heparin) are typically given initially followed by warfarin.  When indicated, an IVC filter may be placed in the patient to help prevent further clots from forming in the arteries of the lungs.

Brain Injuries and Birth

The brain is responsible for higher motor and sensory functions. It requires a constant source of oxygen in order to continue its vital functions. When the flow of oxygen is interrupted, the consequences can be devastating.  Severe injury can occur when the brain lacks the oxygen needed to continue functioning. A total lack of oxygen can be referred to as “anoxic” injury whereas a partial lack of flow can be referred to as “hypoxic” injury.

Brain injury can occur in an unborn baby if the flow of oxygen from the mother’s circulation to the baby’s is interrupted. This can happen from a number of causes. For example, the placenta can become partially detached from the wall of the uterus (placental abruption), which can interrupt the flow of oxygen to the baby. This complication can arise in pregnant patients with untreated or inadequately treated high blood pressure. Close monitoring of mother and baby, along with timely delivery and/or C-Section when needed can prevent these complications.

 

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Excessive contractions of the uterus (sometimes called “uterine hyperstimulation” or “tetanic contractions”) from labor, or from medicines used to accelerate labor, like pitocin, can cause problems with the baby’s oxygen supply. The rapid, powerful contractions of the uterus can prevent maternal oxygen from reaching the baby. If the flow of oxygen to the baby is interrupted, hypoxic or anoxic brain injury can occur. The consequences of this can be severe and may include seizures, brain damage, developmental delay, cerebral palsy, and other problems with motor or cognitive functions. Use of medications like pitocin must be closely monitored by dosage and effect on the mother and baby. If contractions are too strong or too frequent, the dosage may need to be decreased, the medicine may need to be stopped entirely, or special medication to reverse the effects may be given.

Brain injuries may occur in infants, children, and adults. Stroke, cardiac arrest, or choking can all interrupt the flow of oxygen to the brain. Patients who are undergoing surgery, or are in an intensive care unit in a hospital often have their oxygen levels monitored to be sure they are getting a sufficient supply. If they are not properly monitored or complications occur, hypoxic or anoxic brain injuries may result.

Medical malpractice that results in brain injury is devastating for the victim as well as families and loved ones. The extent of disability from hypoxic or anoxic brain injury varies greatly, but many people require extensive care and rehabilitation to regain function and improve their quality of life.

Stomach Cancer and Medical Malpractice

Stomach cancer is referred to as gastric cancer. The most common type of gastric cancer is called adenocarcinoma. This starts in the glandular tissue that composes the lining of the stomach and accounts for 90% to 95% of all gastric cancers. Other forms of gastric cancer include lymphomas, which involve the lymphatic system and sarcomas, which involve the connective tissue (such as muscle, fat, or blood vessels)iStock_000002366269XSmall.jpg.

Generally, the best chance for cure of gastric cancer is when it is found and treated at a very early stage. Unfortunately, the outlook is typically poor if the cancer is already at an advanced stage when discovered

Gastric cancer can develop in any part of the stomach and may spread throughout the stomach and to other organs, such as the esophagus, liver and lungs.  Gastric cancer is responsible for about 800,000 deaths worldwide per year.

The signs/symptoms include indigestion and/or heartburn, loss of appetite, especially for meat, abdominal discomfort or irritation, weakness and fatigue, bloating of the stomach, usually after meals, abdominal pain in the upper abdomen, nausea and occasional vomiting, diarrhra or constipation, weight loss, vomiting, blood in the stool, which will appear as black. This can lead to anemia, and dyspagia (trouble swallowing), which suggests a tumor in the upper protion of the stomach or extension of the gastric tumor into the esophagus.

The diagnosis is typically made when abnormal tissue seen in a direct visual (gastroscopic) examination is biopsied. This tissue is then examined under a microscope to check for the presence of cancerous cells. A biopsy, with subsequent histological analysis, is the only sure way to confirm the presence of cancer cells.

The clinical stages of stomach cancer are:

Stage 0. Limited to the inner lining of the stomach.

Stage I. Penetration to the second or third layers of the stomach (Stage 1A) or to the second layer and  nearby lymph nodes (Stage 1B).

Stage II. Penetration to the second layer and more distant lymph nodes, or the third layer and only nearby lymph nodes, or all four layers but not the lymph nodes.

Stage III. Penetration to the third layer and more distant lymph nodes, or penetration to the fourth layer and either nearby tissues or nearby or more distant lymph nodes.

Stage IV. Cancer has spread to nearby tissues and more distant lymph nodes, or has metastatized to other organs.

Treatment for gastric cancer depends on both the tissue type and the stage of the cancer. Treatment for adenocarcinoma may include:

1. Surgery- the goal of surgery is to remove all of the cancer and allow for a margin of healthy tissue,  when possible. The surgeon also removes lymph nodes surrounding the stomach to determine if they  have been invaded by cancer cells. Removing part of the stomach may relieve signs and symptoms of a  growing tumor in people with advanced stomach cancer. In this case, surgery generally does not cure the  cancer, but it can make the patient more comfortable. This is known as palliative therapy.

2. Radiation Therapy-uses high-powered beams of energy to kill cancer cells. It can be used before surgery (neoadjuvant radiation) to shrink a stomach tumor so it's more easily removed. Radiation therapy can also be used after surgery (adjuvant radiation) to kill any cancer cells that might remain.  Radiation is often combined with chemotherapy. In cases of advanced cancer, radiation therapy may be  used to relieve side effects caused by a large tumor. Radiation therapy can cause diarrhea, indigestion,  nausea and vomiting.

3. Chemotherapy-is a drug treatment that uses chemicals to kill cancer cells. Chemotherapy drugs can  kill cancer cells that may have spread beyond the stomach. Chemotherapy can be given before surgery  (neoadjuvant chemotherapy) to help shrink a tumor so it can be more easily removed. And it can be  given after surgery (adjuvant chemotherapy) to kill any cancer cells that might remain in the body.    Chemotherapy is often combined with radiation therapy. Chemotherapy may be used alone in patients  with advanced stomach cancer to help relieve signs and symptoms. Side effects of chemotherapy depend  on which drugs are used, and which drugs are used depends upon the type of cancer being treated.

4. Clinical Trials-are always being done at research cancer centers to study new treatments and new ways of using existing treatments. Participating in a clinical trial may give a patient a chance to try the  latest treatments. In some cases, researchers might not be certain of a new treatment's side effects.

Depending on the stage at diagnosis, the average 5 year survival rates for adenocarcinoma of the stomach are as follows:

Stage Ia - 71%

Stage Ib - 57%

Stage IIa - 45%

Stage IIb - 33%

Stage IIIa - 20%

Stage IIIb - 14%

 Stage IV - Less than 4%.

Medical malpractice may be seen when there is a significant and negligent delay in the diagnosis of gastric cancer. As the above shows, if the diagnosis is delayed, and the stage advances, the prognosis for 5 year survival rates becomes poor.  During patient screening, a doctor should be aware of the risk factors for gastric cancer that include smoking cigarettes, a diet high in salted, smoked, pickled meats or foods, stomach inflammation, a history of Helicobacter pylori infection, gastric polyps, pernicious anemia, and a family history of one or more relatives diagnosed with gastric cancer.  Screening for one type of adenocarcinoma called HDGC or Hereditary Diffuse Gastric Cancer includes genetic testing for the CDH1 gene mutation.  In families with the CDH1 gene mutation, intense surveillance may be appropriate and in some cases prophylactic gastrectomy.  

Aside from upper endoscopy, other helpful diagnostic tests for gastric cancers include a double contrast barium x-ray, ultrasound, or a CT scan; however, a negative barium x-ray study alone may give false assurance and is sometimes a reason for a delay in diagnosis. Research suggests that improvement in five-year survival requires not only improved awareness by patients, but especially improved diagnostic methods and screening programs by their doctors. 

Hydrocephalus and Medical Malpractice

Hydrocephalus is also known as "water in the brain."  It is a condition in which there is an abnormal accumulation of cerebrospinal fluid (CSF) in the ventricles, which are cavities in the brain.  In infants with hydrocephalus, CSF builds up in the central nervous system, causing the soft spot near the front of the scalp, known as the anterior fontanelle, to bulge and the head to be larger than expected. There is also a soft spot near the back of the head known as the posterior fontanelle. Early symptoms may include sundowning, which is when the eyes appear to gaze downward; irritability; seizures; separated scalp sutures; sleepiness; and vomiting.

In newborns with hydrocephalus, the head circumference rapidly enlarges and may surpass the 97th percentile when measured at pediatric visits.  If the skull bones have not yet firmly joined together at this age, bulging, firm anterior and posterior fontanelles may be present when the patient is in an upright position. The infant may exhibit fretfulness, poor feeding, and frequent vomiting.

As the hydrocephalus progresses, torpor, a state of sluggishness and reduced metabolism, sets in and the infant shows lack of interest in his/her surroundings. In a later stage, the upper eyelids become retracted and the eyes turn downwards (due to hydrocephalic pressure on the mesencephalic tegmentum and paralysis of upward gaze). Movements become weak and the arms may become tremulous.  There may be reduction of vision. Over time, the head becomes so enlarged that the growing child becomes bedridden and he/she is unable to lift or even move this huge object attached to its neck.

Hydrocephalus is diagnosed through clinical neurological evaluation and by using cranial imaging techniques such as ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), or pressure-monitoring techniques. The most common initial diagnostic test to determine hydrocephalus is an image of the brain (CT Scan or MRI) to identify the enlarged ventricles (spaces) within the brain that are typical of hydrocephalus.  

Hydrocephalus is often treated with the surgical placement of a shunt system. This system diverts the flow of CSF from a site within the central nervous system (CNS) to another area of the body where it can be absorbed.  A shunt is a flexible but sturdy tube composed of silicone and plastic.   A shunt system consists of a shunt tube, a catheter, and a valve. One end of the catheter is placed into one of the dilated a ventricles inside the brain.  Depending on the situation, it could also be placed into a cyst or somewhere near the spinal cord. The other end of the catheter is usually placed within the abdominal cavity.  It could also be placed into any area, such as the heart, where the extra cerebrospinal fluid can be drained and absorbed.  The valve located along the catheter maintains one-way flow and regulates the rate of CSF flow.

Hydrocephalus can be effectively treated with a shunt; however, there is still a potential for complications. The shunt can become blocked. Symptoms of blockage include headache and vomiting. There may be other problems with the shunt such as kinking, tube separation, or infection in the area of the shunt. Other complications may include infections such as meningitis or encephalitis, intellectual impairment, and nerve damage.  Untreated hydrocephalus has a 50-60% death rate. Survivors have varying degrees of intellectual, physical, and neurological disabilities.  

Spinal Cord Compression and Cauda Equina Syndrome

There are approximately 12,000 new cases of spinal cord compression annually in the United States with the average age of victims being 39.5 years old. Causes of spinal cord compression include trauma (such as auto accidents, falls, sports injury, epidural injection), spinal abscess, tumor, hematoma or blood clot, ruptured or herniated disk, and spinal stenosis. . Early symptoms may include the start of loss of movement or feeling in the arms or legs, back pain, and the loss of bowel or bladder function or control. Spinal cord compression of sudden onset constitutes a medical emergency, as the longer the duration of symptoms before cord pressure relief is obtained by surgical intervention (laminectomy), the greater the chance of permanent injury. Diagnosis is by clinical exam and other testing such as X-ray, CT, and MRI. MRI is usually the most accurate study to detect spinal cord compression as details of both bony and soft tissue abnormalities in the spinal column may be visualized. MRI may also be the preferred study if spinal cord injury occurs during pregnancy as MRI offers reduced radiation exposure to the fetus. However, after an accident, traction devices to immobilize the spine and life support equipment may preclude the use of MRI. 

The spinal column is comprised in descending order of the cervical, thoracic, lumbar, and sacral areas. The actual spinal cord ends at the level of L-1. Below the level of L-1, the cord branches into a bundle of spinal nerve roots from L-1 through L-5 and S-1 through S-5 that resembles a horses tail referred to as the cauda equina. When the nerve roots of the cauda equina are compressed permanent neurogenic injury may occur. This may be referred to as cauda equina syndrome (CES). Causes of compression of these nerve roots include the same types of causes as listed above for spinal cord compression. Symptoms of early onset of cauda equina syndrome include numbness in the groin (saddle anesthesia), loss of bowel or bladder function or control, weakness in the legs, and absence of ankle reflexes. Early diagnosis and treatment of CES is important as the longer the duration of symptoms, the more likely permanent neurological injury will occur such as paralysis and incontinence. Cauda equina syndrome of sudden onset is a medical emergency and treatment generally involves surgical decompression of the affected nerve roots. A laminectomy may be performed to relieve pressure on the nerve root in cases where there is a herniated or ruptured disk, hematoma, abscess, or tumor. 

In spinal cord and nerve root compression cases involving sudden onset of neurological symptoms, medical malpractice may arise when there has been an unreasonable delay in diagnosis and prompt surgical intervention by the doctors or hospital. Allegations of medical malpractice may also include a failure to diagnose and treat a vertebral fracture of the neck or back that required traction or immobilization which was not performed, thereby allowing pathological movement that resulted in spinal cord compression.

Leg Amputation and Medical Malpractice

Amputation is the surgical removal of all or part of an extremity. The most common amputation surgery is above or below the knee.  The indications for leg amputation include severe trauma, significant tumor in the bone or muscle, lack of blood circulation due to peripheral arterial disease, worsening or uncontrollable infection, failed management of acute compartment syndrome, failed management of Charcot’s degenerative osteoarthropathy, or debilitating extremity paralysis from infection or pressure-related complications. Failute to timely diagnose and treat infection, tumor, pressure sores, vascular disease, compartment syndrome, and Charcot's all may result in the unneccessary amputation of a leg and give rise to allegations of medical malpractice against negligent health care practitioners.  

iStock_000026640818XSmall.jpgThe amputation procedure varies depending on the extremity undergoing the operation. To determine the operation site and the amount of tissue to remove, the surgeon relies on the following factors: the patient’s pulse, skin temperature, areas of reddened skin, and sensitivity to touch in the affected extremity. The presence of a palpable pulse proximal to the level of amputation is a positive predictor for successful healing; however, the absence of a pulse does not necessarily reflect future wound healing failure. The level of the amputation is based on the extent of the damaged tissue, the healing potential of the area, and the rehabilitation potential of the patient. In addition to a thorough clinical examination, objective tests such as ankle pressures, toe pressures, transcutaneous oxygen measurements, and skin perfusion pressures are useful.  

The preoperative evaluation and preparation involves medical risk assessment, nutrition assessment, prosthetic and rehab consultation, and possibly a psychological consultation. The diseased tissue is removed along with any crushed bone and the maximal amount of healthy tissue is left behind. The blood vessels and nerves in the surrounding area are sealed off. Following the amputation, the site can be left open due the possibility of further amputation or covered with skin flaps and closed. The remaining muscles in the area are shaped so the end of the limb can be fitted for prosthesis, also known as an artificial limb. 

Thromboprophylaxis is recommended for all patients undergoing major lower extremity amputation because patients are at high risk for thromboembolism, the blocking of a blood vessel by a particle that has separated from a blood clot at the formation site. Antibiotic prophylaxis is typically recommended within one hour of skin incision for lower extremity amputation due to high risk for surgical site infection.

Generally, the patient undergoes physical rehabilitation soon after surgery and practice with the prosthesis can begin 10-14 days after surgery. The patient’s postoperative outcome is dependent upon preoperative functional status, comorbidities, and the level of amputation. Wound healing must be monitored and dressing changes performed. Patients with advanced diabetes, significant heart disease, or serious infection are at a greater risk of complications from the procedure. Possible complications include infection, joint contracture, necrosis, deep vein thrombosis, pulmonary embolism, hematoma, and wound opening. In addition, patients may experience phantom pain, a sense of pain in the amputated limb described as burning aching, or electric. Other causes of pain such as ischemia, infection, neuroma, or pressure related wounds should be excluded before determining the diagnosis as phantom pain.  

 

Appendicitis: Early Diagnosis and Treatment are Essential

Appendicitis is a medical emergency that requires immediate surgery to remove the appendix.  If left untreated, an inflamed appendix will eventually rupture.  When this happens intestinal contents (stool and bacteria) spill into the abdominal cavity causing infectious peritonitis, a serious and toxic inflammation of the abdominal cavity's lining (the peritoneum).  This condition can be fatal unless it is treated quickly with surgery and strong antibiotics.

SYMPTOMS: Usually pain is the first symptom, starting in the mid abdomen around the navel, and except in children below 3 years old, the pain tends to localize in the right lower quadrant within a few hours.  The abdominal wall becomes sensitive to gentle pressure, and the pain can be elicited through various tests the physician will use to bring it out.  One such sign is when the abdomen is gently pushed down and quickly released.  This is known as rebound tenderness and is a clinical sign the peritoneum is inflamed.  If the appendix is located beneath the cecum (first part of the colon), it may fail to elicit tenderness (silent appendix).  And if the appendix lies entirely within the pelvis, the region below the addomen, there could be a complete absence of the abdominal signs and symptoms. In such cases, a digital rectal exam will cause discomfort localized to the region of the appendix in the right lower quadrant.  Also, if the abdomen on palpation is rigid, which is known as involuntary guarding, there should be a strong suspicion of peritonitis requiring urgent surgical intervention.  The physician can perform certain other maneuvers, such as bending and rotating the right hip, and extending the hip in the prone position, which will bring about pain consistent with inflammation caused by appendicitis.  The next symptoms usually experienced are naussea and vomiting, as well as constipation.  Eventually as the inflammation progresses, fever will occur.  

BLOOD TESTS: When appendicitis is suspected, blood tests such as a CBC need to be done to try to confirm the diagnosis.  More than 80% of adults with appendicitis have a white blood cell count greater than 10,500 cells/mm3.  Another blood test commonly used is the determination of C-reactive protein (CRP),an acute-phase reactant synthesized by the liver in response to infection or inflammation.   

DIAGNOSTIC IMAGING: The plain film of the abdomen, known as a KUB is typically taken. Visualization of an appendicolith (a white colored defect in the right lower quadrant) in a patient with symptoms consistent with appendicitis is highly suggestive of appendicitis, but this occurs in fewer than 10% of cases. Another X-ray that may be utilized is the single-contrast Barium enema, which can be performed on an unprepared bowel. Absent or incomplete filling of the appendix with contrast barium coupled with pressure effect or spasm in the cecum suggests appendicitis. Though cheap in cost, once thick barium contrast is instilled, other more definitive tests will not be able to be performed. 

The appendix may be evaluated via transabdominal sonography. Many physicians believe that ultrasonography should be the initial imaging test in pregnant women and in children due to its safety. Abdominal CT scanning has become the most important imaging study in the evaluation of patients with appendicitis.  Advantages of CT scanning include its superior accuracy when compared with other imaging techniques, as well as its ready availability, noninvasiveness, and potential to reveal alternative diagnoses. MRI plays a relatively limited role in the evaluation of appendicitis due to high cost, long scan times, and limited availability.  The lack of dangerous radiation exposure makes it an attractive modality in pregnant patients where ultrasound is not helpful in making a diagnosis. 

In today’s day and age, the diagnosis of appendicitis should generally not be missed by an emergency room physician.  If in doubt a surgeon should be called to evaluate and remove the appendix, which can be done laparoscopically, so long as it remains locally inflamed or walled off, and before there is free perforation into the abdominal cavity.  A delay in diagnosis can result in lifelong complications affecting bowel function due to the scarring of the peritoneal lining.

BREAST CANCER AND MEDICAL MALPRACTICE

Breast cancer affects millions of women in America and some studies indicate up to one out of eight women will be diagnosed with breast cancer over the course of her lifetime. The survival rate of breast cancer that is detected and treated early is much better than when a malignant tumor is found after it haThumbnail image for Thumbnail image for iStock_000013080813XSmall.jpgs already spread to lymph nodes or surrounding tissue.  Therefore, it is important that every effort be made to diagnose and treat breast lesions as quickly as possible. 

One of the mainstays in breast cancer detection is mammography. A mammogram involves an X-ray taken of the breasts, usually taken from several angles. Some abnormalities on mammograms such as masses or calcifications can alert the healthcare provider and patient that additional diagnostic testing needs to be done. The mammogram is one of the main methods of screening for breast cancer. Other imaging methods are less commonly used for screening, such as sonography (ultrasound), and MRI. However, these diagnostic methods can be used to add to information already obtained from a mammogram study when a patient is evaluated and uncertainty or an abnormality exists.

Patients need to be informed about the need for breast cancer screening. The mammogram, ultrasound, or MRI tests need to be read and interpreted correctly in order to diagnose problems and treat them early. In the case of breast cancer, early detection and diagnosis is key to improved cure rates.  Special circumstances and risk factors (such as family history) may point to additional diagnostic testing to help identify breast cancer in those women at high risk. If tests are not interpreted correctly, or the results are not acted on in a timely or proper fashion by the healthcare provider, medical malpractice may be the result. 

Sometimes patients or healthcare providers feel a breast mass or lump on examination. This often requires further evaluation usually beyond just a “regular” screening mammogram. As noted above, additional mammogram views, ultrasound, and/or MRI can help characterize the type of mass. However, breast masses usually warrant a visit to a surgeon or breast specialist to evaluate the patient for biopsy. Breast biopsies can be much less invasive to patients than in years past. During a “stereotactic” breast biopsy, the doctor employs mammography to locate the specific abnormal area and then a special instrument is used to remove cells from that precise area. Ultrasound can also be used to identify an abnormal area that is then biopsied. These methods are particularly useful when a breast mass cannot be felt. Sometimes when a mass can be felt, or palpated, the biopsy can be done without using X-ray or ultrasound localization. 

Breast cancer screening can help identify women with breast cancer, as well as pre-cancerous lesions and benign breast conditions. Medical malpractice may occur as a consequence of failure to adequately evaluate, identify, screen, or diagnose breast cancer. Allegations of medical malpractice may include a failure by the internist to refer the patient to a breast specialist or surgeon for evaluation, failure by the radiologist to properly read a mammogram, ultrasound, or MRI, and/or failure by the pathologist to detect cancer in a breast biopsy that is indeed malignant. With improved methods of screening, diagnosis, and treatment of breast cancer, survival rates should continue to improve.

 

Congenital Diaphragmatic Hernia in Newborns

Hernia is when an organ or tissue protrudes through a weak area in the surrounding muscle or connective tissue. There are different types of hernias that generally vary by location. Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm that permits the abdominal viscera to herniate into the chest. The amount of herniated contents may be small or large and it generally contains parts of the intestine, spleen, or liver. CDH occurs during a critical period of lung development when bronchial and pulmonary artery branching occurs so lung compression by herniated bowel can lead to pulmonary hypoplasia, which is underdevelopment of the lungs. CDH occurs in 1 out of 2200 births and in 50% of CDH cases, there are other associated anomalies such as chromosomal abnormalities, congenital heart disease, and neural tube defects.

Within the first few hours of life, infants with CDH may present with mild or severe respiratory distress that may be incompatible with life. Symptoms include bluish skin due to lack of oxygen, rapid breathing, and fast heart rate. On physical exam, patients may have a barrel-shaped chest, scaphoid appearing abdomen due to loss of abdominal contents into the chest, and absence of breath sounds on the affected side. In the majority of patients with CDH, herniation occurs on the left; therefore, the heartbeat is displaced to the right due to a shift in the mediastinum. Right sided diaphragmatic hernias occur in 11% of cases and bilateral herniation only in 2%.

The level of respiratory distress depends on the severity of lung hypoplasia. In the prenatal period, lung hypoplasia can be determined using ultrasound to evaluate herniated contents and to measure the lung area to head circumference ratio. Postpartum, there is no specific test to quantify the amount of hypoplasia. The diagnosis of CDH is generally made prenatally by ultrasound; however, in those where CDH is not diagnosed in utero, it should be suspected in any full term infant presenting with respiratory distress and the diagnosis is made by chest x-ray showing herniation of abdominal contents into the hemithorax

For neonates diagnosed by prenatal ultrasound, the following steps for monitoring and intervention are recommended: twice weekly nonstress testing or biophysical profile testing at 33-34 weeks in addition to ultrasound examinations at 28, 30, 32, and 34-35 weeks to assess fetal growth and amniotic fluid volume. If the fetus experiences growth restriction or oligohydramnios, which is a deficiency of amniotic fluid, the baby should deliver early and betamethasone (a steroid medication that assists in preterm fetal lung development) should be given prior to delivery if the fetus is less than 34 weeks.  The best mode and gestational age for delivery of a fetus with CDH is undetermined; however, the suggested time for planned induction of labor is between 38-39 weeks.

In the delivery room, infants with problematic CDH should be immediately intubated and ventilated with low peak pressure to minimize lung injury. A nasogastric tube on continuous suction is placed in the stomach for decompression of the abdominal contents, which can help expand available lung tissue. In addition, the infant should have an umbilicial artery line for monitoring of blood gases and blood pressure and possibly an umbilical vein catheter for administration of fluids and medications. Blood pressure support should be given and an echo should be done to determine cardiac abnormalities in addition to the extent of pulmonary hypertension and shunting.

Extracorporeal membrane oxygenation (ECMO) has been used as part of  the treatment in some hospitals. ECMO operates as a heart-lung bypass system; thus, it does the job the heart and lungs would be doing. ECMO can be used temporarily while the infant’s condition stabilizes and improves.  Once the infant is stable, he or she can undergo surgical repair of the diaphragmatic hernia, where the stomach, intestines, and other abdominal organs are returned to the abdominal cavity. The hole in the diaphragm is also repaired. If the diaphragm is absent, an artificial diaphragm will be constructed and placed. Following the operation, the infant will require breathing support due to underdevelopment of the lungs. Once the infant is taken off ventilation (breathing machine), he or she may need oxygen and medications to assist with breathing for weeks to years. Thus, CDH requires long term follow up to monitor the infant’s condition to ensure no future complications such as lung infections or other associated congenital problems. The prognosis is generally good for infants with CDH and survival is greater than 80%.  

Normal Pressure Hydrocephalus and Medical Malpractice

iStock_000015502953XSmall.jpgCerebrospinal fluid (CSF) is a clear fluid that surrounds the brain and spinal cord. This fluid is continually produced and stored in ventricles, which are cavities of the brain. CSF cushions the brain, supplies the brain with nutrients, and removes wastes.  Excess fluid drained from the brain is absorbed by other tissues.

Hydrocephalus is a condition in which there is an abundance of CSF in the ventricles due to interference in proper drainage and absorption. To accommodate the extra fluid, the ventricles enlarge causing compression of  different parts of the brain.

Normal pressure hydrocephalus (NPH) is a type of hydrocephalus that occurs commonly in older adults. NPH is different from other types of hydrocephalus because it develops gradually. The slow enlargement of the ventricles causes the fluid pressure in the brain to not be as high as in other types of hydrocephalus. However, the enlarged ventricles still press on normal brain tissue

NPH often presents with the following clinical triad of findings: urinary incontinence, gait disturbance, and dementia, with urinary symptoms appearing later in the sequence.  Gait disturbance is often the initial and most prominent symptom of the triad. It may be progressive due to the expansion of the ventricular system. It generally presents as unsteadiness or impaired balance. Eventually, gait disturbances can lead to a need for canes or walkers. An increased tendency to fall backwards is also common. In the very late stages, the patient can progress to an inability to stand, sit, and rise from a chair. 

Dementia presents as apathy, forgetfulness, and a dullness of thinking. Memory problems are often predominant, which can contribute to a misdiagnosis of Alzheimer's disease. Although recall is severely impaired in NPH, recognition, a hallmark of Alzheimer’s, is either normal or slightly impaired.

Idiopathic NPH is normal pressure hydrocephalus occurring without a known cause; however, it can be attributed to any condition that blocks the flow of CSF such as bleeding from an abnormal blood vessel, an aneurysm, a closed head injury, meningitis or similar infections, and surgery on the skull.

The diagnosis of NPH is made by performing a lumbar puncture (spinal tap). If the initial pressure measurement is elevated, it points to a diagnosis of NPH. Clinical improvement after removal of CSF has a high predictive value for treatment success.  A CT scan of the brain will show enlarged ventricles, and an MRI may show certain technical findings consistent with enlarged ventricles and elevated cerebral pressures. Imaging alone cannot differentiate between NPH and other dementia causing conditions such as Alzheimer’s disease, Atherosclerotic vascular disease, and Parkinson’s disease.  

Treatment for NPH is surgical diversion of the excess CSF. A shunt is implanted to drain CSF from the intracranial ventricular system to a different location such as the peritoneal space in the abdomen. The most common shunts utilized today are called the ventriculoperitoneal (VP) and ventriculoatrial (VA) shunts.  Placement of a shunt is a neurosurgical procedure performed under general anesthesia, and usually takes less than an hour to complete. Cognitive impairment associated with dementia has been reported to improve in more than fifty percent of cases. Successful treatment for NPH is available; therefore, it is medicaly negligent to misdiagnose NPH as Alzheimer’s dementia, where responses to the latest therapy innovations are only temporary.   

Craniosynostosis and Medical Malpractice

An infant’s skull is comprised of boney plates separated by sutures.  Cranial sutures are strong, fibrous tissues that hold the bones together and they intersect in large soft spots known as fontanelles. The infant’s skull does not completely fuse until the age of two thereby granting the brain time to grow. Once the bones fuse, the sutures no longer remain flexible.  

Craniosynostosis is due to premature fusion of one or more cranial sutures.  It affects 1 in every 2000 to 2500 births worldwide. Premature closure of sutures restricts the growth of the skull perpendicular to the affected suture. To accommodate, the growing brain, the skull grows parallel to the affected suture resulting in a skull deformity. Craniosynostosis can involve one or multiple sutures; however, the sagittal suture is the most common. There is an increased incidence of craniosynostosis in multiple pregnancies and in presence of uterine abnormalities; however, craniosynostosis involving multiple sutures is often a product of a genetic syndrome such as Apert’s and Crouzon syndrome.

The different types of craniosynostosis are sagittal synostosis, frontal plagiocephaly, and metopic synostosis. Symptoms depend on the type of craniosynostosis but they may include no “soft spot” on the newborn skull, a raised hard ridge along the affected sutures, unusual head shape, and slow or no increase in head size over time as the infant grows. The diagnosis of craniosynostosis is based upon physical exam and radiographic studies such as X-ray and CT which can further illustrate structural abnormalities. Complications associated with craniosynostosis include increased intracranial pressure; inhibition of brain growth; impairment in cognition and neurodevelopment such as global development delay and poor feeding; and poor self-esteem and social isolation due to aesthetic abnormalities.

Management for this condition begins with a primary care provider recognizing an abnormal head shape and referring to a craniofacial team for evaluation, preferably within the first few weeks of life.  The craniofacial team develops a treatment plan, coordinates future care, and monitors the patient’s progress. Intervention involves surgical repair of craniosynostosis to prevent intracranial hypertension.  Signs of intracranial hypertension include papilledema, a beaten copper appearance on skull radiograph, or increased measured ICP requiring urgent decompression. 

The timing of surgery depends upon the severity of the condition and the child's health.  Sometimes, surgery is performed at 8-12 months of age because intracranial volume is sufficiently large and the child may better endure the stress of surgery. Also, the calvarium is thicker allowing more stable fixation. The different classifications of craniosynostosis require different surgical plans. Prognosis depends on how many sutures are involved and the child’s overall health.  Individuals with this condition who undergo surgery generally improve and do well, especially when it is not associated with a genetic syndrome.   

Wilson's Disease and Medical Malpractice

Wilson's disease is a rare autosomal recessive disorder, where copper accumulates in the body’s tissues and organs, specifically the liver, brain, and eyes. The body acquires and stores too much copper. Copper deposition causes damage, death, and scarring of tissues leading to dysfunction of organs. 

The incidence of Wilson’s disease is 1:300,000. If both parents possess an abnormal gene for Wilson’s disease, there is a 25% chance their child will have the disorder. The gene responsible for Wilson's disease is ATP7B. DNA testing is available for this gene; however, testing is complicated because different ethnic groups may have different mutations in this gene.

It is commonly seen in eastern Europeans, Sicilians, and southern Italians. Symptoms begin to present at age 4. Symptoms include abnormal posture of arms and legs, confusion or delirium, dementia, difficulty moving arms/legs, difficulty walking, emotional or behavioral changes, abdominal distention, personality changes, speech impairment, tremors of arms or hands, uncontrollable movements, hemoptysis, jaundice, etc.

Specific signs and tests are used to verify the diagnosis of Wilson’s disease. A slit lamp examination may demonstrate limited eye movement and Kayser-Fleischer rings, brown-colored rings around the iris. Physical examination may illustrate injury to the central nervous system such as loss of muscle control, coordination, memory, thinking and IQ. Other neurological signs may include muscle tremors and confusion.

In addition, the presence of liver or spleen disorders may be an indication for Wilson’s disease. Lab tests that assist in the evaluation for Wilson’s disease include: CBC, serum ceruloplasmin, serum copper, serum uric acid, and urine copper. If the patient possesses liver problems, one would expect to see the following picture: high liver enzymes (AST and ALT), high bilirubin, high PT and PTT, and low albumin. There are a myriad of other tests such as 24-hour urine copper test as well as abdominal and neurological imaging that can provide further evidence for or against the diagnosis.

Lifelong treatment is required to control Wilson's disease because it may cause fatal effects, such as loss of liver function and damage to the nervous system. In patients where the disorder is not fatal, symptoms may be disabling. The aim of treatment is to decrease the amount of copper in the body causing toxic effects.  Thus, medications known as chelators, which bind copper and remove it via the kidneys and guts, are utilized to treat this disorder. Unfortunately, certain medications that chelate copper, like penicillamine, can sometimes affect neurological function while others may not interfere with neurological function.

Also, a low-copper diet is recommended so individuals should avoid the following foods: chocolate, dried fruit, liver, mushrooms, nuts, and shellfish.  Individuals may want to drink distilled water because the majority of tap water flows through copper pipes. A liver transplant may be necessary in cases where the liver is severely damaged. In addition, people with severe neurological dysfunction may need special protective measures.

If left untreated, a variety of complications can result from Wilson’s disease including anemia, CNS damage, cirrhosis, liver necrosis, fatty liver, hepatitis, increased number of bone fractures and infections, jaundice, muscle atrophy, loss of ability to care for self and function independently, along with a number of other things. However, liver failure and injury to the central nervous system are the most common and dangerous effects. Wilson's disease is fatal if it goes undiagnosed and untreated.

Sigmoid Colon Volvulus and Medical Malpractice

The term volvulus is derived from the Latin word volve, which means to twist. A colonic volvulus occurs when a part of the colon twists on its mesentery, resulting in acute, subacute, or chronic colonic obstruction.  Volvulus involving the sigmoid colon is the most common, occuring in 75% of cases.  Sigmoid volvulus occurs when the last part of the large bowel just before the rectum (named for its “S” shape) twists on its self.  It is common in elderly men, but it is likely to occur in anyone with a redundant sigmoid colon. 

More than 60-70% of patients present with acute symptoms; the remainder present with subacute or chronic symptoms. A history of chronic constipation is common. The patient may describe previous episodes of abdominal pain, distension, and obstipation suggestive of repeated, subclinical episodes of volvulus.  With continued obstruction, nausea and vomiting can occur. The development of constant abdominal pain is ominous. It indicates there may be a closed loop obstruction with significant intraluminal pressure, which can lead to ischemic gangrene and bowel wall perforation.

Abdominal distension is generally massive and characteristically tympanitic over the gas-filled, thin-walled bowel loop. The presence of overlying or rebound tenderness raises the concern of peritonitis due to ischemic or perforated bowel. A patient with a history of acute volvulus episodes that spontaneously resolve can experience marked distention with minimal abdominal pain.

A radiographic film of the abdominal will demonstrate a huge air filled distended bowel frequently in the shape of an inverted “U,” with the convexity of the “U” facing the right upper abdominal quadrant.  A barium enema will show dilation in the sigmoid colon due to a twist. A physician may refer to an area of complete obstruction with some twisting as the “bird beak” sign.

CT scans can demonstrate crossing sigmoid transitions, tagged the X-marks-the-spot sign, and folding of the sigmoid wall by partial twisting, called the split-wall sign.  However, the most sensitive finding on CT is a sigmoid colon transition point, which is seen in 95% of scans, and a disproportionate enlargement of the sigmoid colon, noted in 86% of cases.

Colonoscopy or flexible sigmoidoscopy could be done to both confirm the diagnosis as well as attempt to treat the obstruction. Barium enemas can also reduce the obstruction when the pressure of the fluid rushing into the bowel unwinds it.

For treatment, the first step is to free the acute obstruction, and then to fix the redundant part of the bowel to prevent reoccurrence.  In up to 90% of patients with sigmoid volvulus, the condition recurs after untwisting with methods as noted above. For this reason, anyone with a sigmoid volvulus needs to undergo an operation during the same admission to either remove or fix down the excessive bowel length.

Once the diagnosis of sigmoid volvulus is confirmed, treatment must be immediate. A delay in treatment represents a greater likelihood of bowel wall death and gangrene. Up to 80% of people with this condition die from gangrene if intervention is delayed. In the event this diagnosis is missed and bodily injury results, the treating health care provider is at risk of a medical malpractice lawsuit.

Triple Negative Breast Cancer and Medical Malpractice

By dividing breast cancer into molecular breast cancer subtypes, physicians and researchers can devise better approaches and treatments for dealing with the disease. Most studies divide breast cancer into four major molecular subtypes: Luminal A, Luminal B, Triple-negative, and HER2/neu. Triple-negative breast cancer is characterized by tumors that lack estrogen, progesterone, and human epidermal growth factor receptors

iStock_000018505784XSmall.jpgHigher rates of triple negative breast cancer have been recorded in younger women, Hispanic and African American women, and women of lower socioeconomic class. Triple negative cancer tends to have a poorer prognosis than other types of breast cancer that have more available and effective treatments.

Studies have demonstrated that triple-negative breast cancer possesses a higher probability for recurrence and spreading beyond the breast. This risk is greater within the first few years post-treatment but over time the risk becomes similar to other types of breast cancer. In addition, triple-negative tends to present as a higher grade cancer, meaning that a greater number of cancer cells are abnormal in appearance

Although surgical and radiation treatments are similar for different types of breast cancer, drug treatments usually vary. Many therapies for breast cancer target cellular receptors. A receptor is a molecule generally located on the surface of cells that is involved with chemical signaling. When a molecule binds to a receptor, it causes the cell to perform certain actions such as division or multiplication. Targeted drug treatments like tamoxifen or Herceptin, designed to treat HER2 positive breast cancer, have not been developed for triple negative. A patient with triple-negative does not express estrogen, progesterone, and HER2/neu receptors so therapy against these receptors is ineffective.

Standard treatment for triple-negative breast cancer is chemotherapy. Current treatments include anthracylines, taxanes, ixabepiplone, platinum agents, and biologic agents. Patients undergoing chemotherapy can experience side effects such as vomiting, nausea, alopecia, mucositis, myelosuppression, etc. Surgery and radiation are additional avenues of treatment. Currently, cancer experts are studying several promising drug strategies targeted for triple negative breast cancer such as PARP inhibitors. 

Septic Arthritis and Medical Malpractice

Septic arthritis, also known as reactive arthritis and bacterial arthritis, is inflammation of a joint caused by infection. When it develops, it represents a medical emergency. It develops when bacteria or fungus is transmitted through an individual's bloodstream to a joint. Individuals can develop septic arthritis at any age but it is less common between the ages of three to adolescence.

The majority of acute cases involve bacteria such as staphylococcus or streptococcus. However, children who develop septic arthritis tend to be infected with Group B streptococcus or Haemophilus influenza, if they have not been vaccinated. The number of cases that progress to a chronic infection are less common. Chronic infections are generally caused by organisms such as Mycobacterium tuberculosis and Candida albicans.

Overall, the most common sites of infection are the hip and knee joints. The following are considered risk factors for septic arthritis: artificial joint implants, bacterial infection, chronic disease (diabetes, rheumatoid arthritis, and sickle cell disease), intravenous drug use, immunosuppressive medications, and recent joint surgery or arthroscopy.

Septic arthritis generally presents with a rapid onset of symptoms. Patients develop a fever in addition to joint swelling, redness, and pain in the infected joint. Patients may also experience pseudoparalysis, which is an inability to move the extremity possessing the infected joint. To diagnose septic arthritis, physicians will aspirate the joint fluid to check the cell count, look for crystals under the microscope, and perform a gram stain and culture. In addition, other tests include a blood culture and an X-ray of the affected joint.

Possible complications include joint degeneration and permanent joint damage, which can occur if appropriate treatment is delayed. If antibiotics are promptly given to treat the infection the patient has a good prognosis. If fluid accumulates in the joint, aspiration may be necessary. Aspiration involves a needle being inserted into the joint to drain off the excess fluid. Only severe cases require surgery to remedy the infected joint fluid; however, if a prosthetic joint is affected, it may need to be replaced

Pituitary Adenomas and Medical Malpractice

A pituitary adenoma is generally a benign, slow growing tumor that occurs in the pituitary gland. The pituitary gland is a small, bean shaped structure that lies at the base of the brain. It has a central role in the regulation of hormones that affect the body such as Adrenocorticotropic hormone (ACTH), Growth hormone (GH), Prolactin, and Thyroid-stimulating hormone (TSH).

Approximately 1 in 1,000 individuals have pituitary adenomas. They are generally not cancerous but may invade nearby structures.  They are classified based on size. A microadenoma is less than 1 cm in diameter whereas a macroadenoma is larger than 1 cm in size.

iStock_000017548218XSmall.jpgBased on whether the pituitary adenoma is a hormone-producing or hormone-inactive tumor, the patient will present with different symptoms. Hormone-producing tumors will make excessive amounts of an active hormone so symptoms present as a hormonal imbalance. The three most common hormone-producing adenomas are Prolactinomas, Growth hormone-secreting pituitary adenoma, and ACTH-secreting pituitary adenoma.

If the patient has a large hormone-inactive or hormone-producing tumor, it may compress surrounding brain structures due to its size.  Large pituitary tumors may compress the pituitary gland contributing to pituitary failure, which can lead to sexual dysfunction, inadequate body cortisol levels, and hypothyroidism. Other possible presentations relating to compression of brain structures include visual loss, headache, the “stalk effect”, and pituitary apoplexy.  The “stalk effect” is due to the compression of the pituitary stalk, the structure connecting the brain to the pituitary gland, which leads to a mild elevation in the hormone prolactin. Higher levels of prolactin in females can contribute to irregular menstrual cycles. Pituitary apoplexy presents with an abrupt headache and visual loss. It can occur under two different situations. The pituitary adenoma can bleed internally causing a sudden increase in size or the tumor can outgrow its blood supply and the dead tissue will swell.

Pituitary adenomas can be diagnosed based on endocrine function testing, imaging, and visual field testing. Often times, visual problems may be the only symptom present.  Optometrists and ophthalmologists should seriously consider the possibility of a pituitary tumor when the patient presents with an unexplained loss of visual field (especially peripheral vision), double vision, or blurred vision as the growth of a pituitary tumor can cause compression on the visual pathway (retina, optic nerve, etc). A delay in diagnosis of a pituitary adenoma may result in permanent loss of vision due to the damage caused by this compression. Endocrine function testing evaluates cortisol, follicle-stimulating hormone, lutenizing hormone, insulin growth factor-1, prolactin, testosterone/estradiol, and thyroid hormone levels. The preferred method of imaging is MRI, which screens for adenomas larger than 4 mm.

Treatment for pituitary adenomas depends on the presence of hormone production, size of the tumor, invasion of the tumor into surrounding structures, and the age and health of the patient. Drug therapy is used to treat hormone-producing tumors. For example, bromocriptine and cabergoline are used to treat tumors secreting prolactin because these medications decrease prolactin levels and tumor size. Pituitary adenomas that require surgery are usually minimally invasive techniques, where the tumor is removed through the nose.  On the other hand, radiation therapy involves high doses of radiation being delivered to the tumor. It is a treatment utilized for pituitary adenomas that cannot be controlled by drug therapy or surgical intervention. Patients have the best outlook when the entire tumor can be removed. 

Lumbar Puncture - Indications and Usage

Lumbar puncture is a procedure performed in the lower back area, where a needle is inserted between two vertebrae to remove a sample of cerebrospinal fluid (CSF). The patient flexes his/her back to widen the spaces between the vertebrae so it is easier for the physician to access the region. The back is washed with antiseptic soap or iodine and covered with a sterile sheet. A local anesthetic is used to numb the area and then a thin hollow needle is inserted through the spinal membrane and into the spinal canal. During this portion of the procedure, the patient generally feels pressure. The CSF pressure is measured, a small amount of fluid is removed, and the pressure is measured again. The needle is removed and the site is bandaged. The total procedure takes about 45 minutes.

Thumbnail image for Thumbnail image for iStock_000016020161XSmall.jpgLumbar puncture is used to collect CSF for analysis to help in diagnosing conditions such as subarachnoid hemorrhage (typically caused by ruptured aneurysm or traumatic brain injury), meningitis (inflammation of the membranes around the brain), and cancers of the brain or spinal cord. Lumbar Puncture may also be utilized to inject anesthetic medications, chemotherapeutic drugs, contrast material, or radioactive substances into CSF. 

Lumbar Puncture may be used to identify increased or decreased CSF pressure.  Increased CSF pressure can be caused by increased intracranial pressure as is seen with traumatic brain injury, ruptured aneurysm, and sometimes hydrocephalus. On the other hand, decreased CSF pressure can be caused by spinal cord tumor, shock, fainting, or diabetic coma.  Normal CSF appears clear and colorless. When an infection is present, the CSF may look cloudy and be yellow or pink in color. Infection may be suspected if there is an increased level of white blood cells and/or protein. Increased CSF glucose indicates hyperglycemia whereas decreased CSF glucose may reflect hypoglycemia, bacterial or fungal infection, tuberculosis, or meningitis. If tumor cells are detected, the patient may have cancer in the brain, spinal cord, or CSF.  If there are increased gamma globulin levels, the patient may be suffering from multiple sclerosis, neurosyphilis, or Guillan-Barre syndrome. The analysis of the CSF by the lab and the measuring of CSF pressure recorded from the lumbar puncture help in determining a diagnosis in many serious clinical situations.  

Hemochromatosis and Medical Malpractice

Primary Hemochromatosis occurs when too much iron builds up in the body. It is usually caused by a specific genetic problem that causes too much iron to be absorbed. If there is too much iron in the diet, the extra iron is absorbed in the gastrointestinal tract and builds up in the body tissues, particularly the liver, heart and pancreas. The result is damage to these organs. Primary hemochromatosis is the most common genetic disorder in the United States, affecting an estimated 1 of every 200 to 300 Americans.

Hemochromatosis affects more men than women. It is particularly common in Caucasians of western European descent. Symptoms are often seen in men between the ages of 30 and 50 and in women over 50, although some people may develop problems by age 20. There is increased risk if a relative has or had the condition.

Symptoms include severe fatigue (74%), impotence (45%), and arthralgia (44%), abdominal pain, decreased sex drive, lack of menstruation in women, hypothyroidism, and bronzing of the skin. Clinical manifestations include liver disease, skin pigmentation, diabetes mellitus joint damage, (arthropathy), impotence in males, and cardiac enlargement, with or without heart failure or conduction defects. The most common presentation is a patient with an enlarged liver (hepatomegaly), skin pigmentation, and arthritis.

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Along with an elevated serum iron level, other abnormal lab values include an elevated transferin saturation, which measures the amount of iron bound to transferrin, a protein that transports iron, an elevated ferritin, a test used to evaluate the body's iron stores, and an abnormal liver profile (a group of blood tests used to evaluate liver function). Commonly alanine amino transferase (ALT) or alkaline phosphatase are elevated.  When iron stores increase in the pancreas, pancreatitis leading to potentially fatal diabetes can occur. Iron overload damages the liver causing jaundice, which leads to fatal hepatic cirrhosis. The heart is another organ that excess iron can damage, causing it to fail.

Fortunately, primary hemochromatosis has a relatively simple treatment, and these complications, if caught early enough, can usually be reversed by treatment of the iron overload causing them. This treatment in an otherwise-healthy person consists of regularly scheduled phlebotomies (bloodletting). When first diagnosed, the phlebotomies may need to be performed fairly frequently, perhaps as often as once a week, until iron levels can be brought to within the normal range. Once iron and other markers are within the normal range, phlebotomies may be scheduled every other month or every three months depending upon the patient's rate of iron absorbtion.

For those patients unable to tolerate routine bloodletting, or whose blood count is not high enough to allow for it, there is a chelating agent, known as desferoxamine (deferoxamine) that can be used. Chelating agents such as desferoxamine bind with iron in the bloodstream and enhances its elimination from the organs involved via the urine and feces. Typical treatment for chronic iron overload requires subcutaneous injection over a period of 8–12 hours daily.

Primary hemochromatosis must be considered in any patient presenting with an enlarged liver. It also must be suspected if any one liver blood test, seen on any routine metabolic profile, is elevated without an explanation. The same is true for elevated fasting blood sugar levels. This is a disorder that can be successfully treated. And if caught early, a patient can expect to live a normal lifespan. Once the heart, pancreas or liver are damaged, life expectancy is considerably diminished.  Failure by a doctor to timely diagnose hemochromatosis before permanent damage to any bodily organ occurs, may constitute medical malpractice.

Premature Birth May Result in Injury to the Newborn

Premature birth can often result in complications for the newborn. Preterm or premature birth refers to babies born before 37 weeks of a normal 40 week long pregnancy

Some complications that can affect babies born prematurely are lung complications from lack of pulmonary maturity or development. Eye problems can result in vision difficulties or blindness. Premature babies may be more prone to infections and intestinal problems. They also are at increased risk for bleeding in the brain which can have devastating effects. Conditions ranging from learning disabilities to cerebral palsy and seizures are more common in babies born prematurely.

Thus it is extremely important that premature labor be diagnosed in a timely fashion by the patient’s health care provider. Steps can be taken to treat the underlying cause of the preterm labor. For example, if a maternal infection is present that is related to the preterm contractions , the infection needs to be diagnosed and treated. Certain conditions, such as twins, increase the risk of premature labor. Also, some anatomic abnormalities of the cervix or uterus can elevate the risk as well.

Although it may not be possible to prevent premature birth, certain steps can be taken to prolong the time until delivery. This may give time for medications to be administered that may significantly improve the newborns outcome by enhancing lung development or decreasing the risk of intestinal problems. Infections that are properly diagnosed and treated may likewise improve the premature baby’s situation.

Medical malpractice may occur when a pregnant patient’s symptoms, such as premature contractions, bleeding, or rupture of membranes are not properly evaluated and managed. If needed interventions such as medication to improve lung function, or antibiotics, are not given in cases where they are indicated, malpractice may have occurred. An evaluation by attorneys with experience and knowledge of these complex medical and legal issues can help clarify issues for victims of medical malpractice.

Ventricular Septal Defect in the Newborn

A ventricular septal defect (VSD) refers to a hole in the ventricular septum, the wall dividing the left and right ventricles of the heart. VSDs are the most common congenital cardiac anomalies. They are found in30-60% of all newborns with a congenital heart defect, or about 2-6 per 1000 births. During heart formation, when the heart begins life as a hollow tube, it begins to partition, forming a septa. If this does not occur properly it can lead to an opening being left within the ventricular septum.

As noted above, during ventricular contraction some of the blood from the left ventricle is forced into the right ventricle from where it enters the lungs through the pulmonary arteries and then reenters the left ventricle via the pulmonary veins and left atrium.  This refluxing of blood causes volume overload on the left ventricle.  This increased blood volume also increases pressure in the right venticle eventually causing pulmonary hypertension with its associated symptoms. This effect is more noticeable in those with larger defects, who may present with shortness of breath, poor feeding and failure to thrive in infancy.   

Because structural cardiac anomalies are estimated to occur in 8 of every 1,000 live births, it is imperative they be discovered before birth. Cardiovascular anomalies, such as VSD, are frequently associated with other congenital anomalies because the heart is among the last organs to develop completely in the embryo. The guidelines for routine prenatal evaluation of both the American College of Radiology and the American Institute of Ultrasound in Medicine require evaluation of the fetal heart.  To not do so, may be consistent with medical negligence or medical malpractice.

The ultrasound view that is most commonly used is the four-chamber view of the heart.  This allows for the assessment of abnormalities involving both right and left ventricles.  Normally, both ventricles are approximately the same size. The left ventricle is posterior and to the left of the right ventricle.  The wall between them, known as the interventricular septum separates the right ventricle from the left ventricle.  A normal septum goes from the cardiac apex to the atrial septum. Formation of the interventricular septum begins at approximately 28 days gestation.  A VSD results from maldevelopment of the embryonic  septum.

A large VSD is easily diagnosed on the four-chamber view alone. However, color Doppler ultrasonography may be needed to demonstrate smaller defects, and some really small defects may not be detected until after birth.  However, most of these close spontaneously.  Fetal position is a major factor in the detection of VSD.  For accurate diagnosis, it is critical that the ultrasound technician positions the transducer so the ultrasound beam is correctly directed relative to the septum.  Not only can a VSD be missed, but a pseudo-VSD may result if performed incorrectly.  

A small ventricular septal defect may never cause any problems. Larger defects can cause a wide range of disabilities, from mild to life-threatening.  If pulmonary hypertension develops due to a large ventricular septal defect that goes untreated, over time, permanent damage to the lung arteries develops and the pulmonary hypertension becomes irreversible.  This complication is known as Eisenmenger's syndrome, and may occur in early childhood, or it can develop slowly over many years.  Other complications that occur are heart failure, endocarditis, stroke, and heart arrhythmias which can lead to fatal complications. Becoming pregnant is also of concern due to the additional risks of pregnancy to women with VSDs undetected before birth and throughout childhood.   

Though no treatment is necessary for small VSDs, larger VSDs demand certain precautions be taken, often with the baby being born in a tertiary care center and directly placed into the care of a pediatric cardiologist to determine if medical or surgical therapy is necessary to prevent the complications that will occur.  That is why it is imperative that the diagnosis of VSD be made prior to birth. 

Hypertrophic Pyloric Stenosis

Hypertrophic pyloric stenosis (HPS) in infants is a narrowing of the pylorus, the lower part of the stomach through which food and other stomach contents pass to enter the small intestine. When an infant has HPS, the muscles in the pylorus have enlarged resulting in narrowing within the pyloric channel preventing food from emptying out of the stomach.

HPS is a form of gastric outlet obstruction, which means a blockage from the stomach to the intestines. It affects three out of every thousand babies born in the United States. It's about four times more likely to occur in firstborn male infants and also has been shown to run in families.  HPS occurs more commonly in Caucasian infants than in babies of other ethnic backgrounds.

Despite numerous hypotheses, the exact cause of HPS is not fully understood. It is believed that these babies are not born with it, but that the progressive thickening of the pylorus occurs after birth. A baby will start to show symptoms when the pylorus is so thickened that the stomach can no longer empty properly.

The thickening of the pyloric muscles may be a due to a combination of several factors.  The use of erythromycin in the first 2 weeks of life has been associated with HPS, and there is also a connection in babies whose mothers took this antibiotic at the end of pregnancy or during breastfeeding.

Symptoms of HPS include:

 Projectile vomiting- the forceful ejection of milk or formula up to several feet away, within 30 minutes after feeding. Vomiting may be mild at first and gradually become more severe. The vomit may sometimes contain blood. Babies are usually not ill-looking or febrile. In the early stage of the disease they remain hungry and suck vigorously after episodes of vomiting.

Persistent hunger- Babies often want to eat soon after vomiting

Stomach contractions- These are noticeable wave-like contractions that move across the upper abdomen (peristalsis) soon after feeding but before vomiting. This is caused by stomach muscles trying to force food past the outlet of the pylorus

Dehydration- prolonged delay in diagnosis can lead to dehydration. The infant with cry without tears or become lethargic. Diapers need to be changed less often and won’t be as wet as wet as they should be.

Changes in bowel movements- since food is prevented from reaching the intestines, babies with this condition may be constipated.

Weight problems- babies will not gain weight, and can sometimes even lose weight.  Parents often report trying several different baby formulas because they (or their physicians) assume vomiting is due to intolerance.

There are other conditions that can resemble HPS, such as gastroesophageal reflux disease (GERD).  This usually begins at eight weeks of age, with excess spitting up, or reflux, occurring after feedings. However, the majority of infants with GERD do not experience projectile vomiting, and although they may have poor weight gain, they tend to have normal stools.

In infants with gastroenteritis, which is an inflammation in the digestive tract that can be caused by viral or bacterial infection, their symptoms may also  resemble HPS. Vomiting and dehydration are seen with both conditions; however, infants with gastroenteritis will often have diarrhea with loose, watery, or sometimes bloody stools. Diarrhea usually isn't seen with HPS.  

Once HPS is diagnosed, early consultation with a surgeon familiar with neonatal care is warranted because treatment is essentially surgical.  This is especially important if the child requires transfer to another facility for surgical care. The American Pediatric Surgical Association offers guidelines for appropriate consultation and transfer of small infants. Good outcome has been shown to depend on the quality of preoperative correction of fluid and electrolyte abnormalities, availability of a pediatric anesthetist, and training level of the surgeon.

Pyloromyotomy, the surgical procedure in which an incision is made in the longitudinal and circular muscles of the pyloris is the procedure of choice. Laparoscopic pyloromyotomy has a significantly shorter recovery time compared with open pyloromyotomy. However, open pyloromyotomy has a higher efficacy and fewer complications.  Endoscopic pyloromyotomy, another alternative, can be performed as an outpatient procedure. Recently, endoscopic balloon dilatation of HPS after failed pyloromyotomy has been used with greater frequency.