Nephroblastomatosis- fetal ascites- macrosomia and Wilms tumor

Overview

A rare condition characterized by kidney abnormalities, macroxomia, endocrine pancreas abnormalities, large fetus and mental retardation

Symptoms

The list of signs and symptoms mentioned in various sources for Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor includes the 26 symptoms listed below: Large infant body Unusual facial features Fluid accumulation in abdomen Excess amniotic fluid Enlarged abdominal organs Undescended testes Bilateral renal hamartoma High blood insulin Mental retardation Round face Low muscle tone Small jaw Depressed nasal bridge Open mouth Anteverted upper lip Defective sternum Diaphragmatic hernia Capillary hemangiomas on skin Upswept front of hair Enlarged liver Ileac atresia Nephroblastomatosis Enlarged kidney Hyperplasia of islands of Langerhans Adrenal cortex cytomegaly Low blood sugar Note that Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor symptoms usually refers to various symptoms known to a patient, but the phrase Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor signs may refer to those signs only noticable by a doctor.

Causes

Hepatic, resulting in portal hypertension Hepatic cirrhosis: Extrahepatic biliary atresia, ╬▒1antitrypsin deficiency, galactosemia, tyrosinemia Portal vein thrombosis Cavernous transformation: Catheterization, dehydration, clotting disorder, omphalitis BuddChiari syndrome, due to neoplasm, collagen disease, hypercoagulopathy, OCP Arteriovenous fistula Fulminant hepatic failure (drugs, virus) Congenital hepatic fibrosis Lysosomal storage diseases (e.g., Gaucher) Bile ascites (bile peritonitis): Spontaneous rupture of the common bile duct o Renal Nephrotic syndrome Urinary ascites (due to bladder rupture) Obstructive uropathy: Congenital ascites may be seen with bilateral hydronephrosis Peritoneal dialysis Cardiac Congestive heart failure Chronic constrictive pericarditis Inferior vena cava web Erythroblastosis fetalis Peritonitis Tuberculous peritonitis Schistosomiasis (Mansoni) Tularemia Abscess Gastrointestinal disorders Infarcted bowel Bowel perforation Pancreatitis, ruptured pancreatic duct Proteinlosing gastroenteropathy o Chylous ascites Collection of lymph within the abdominal cavity; secondary to lymphatic obstruction from trauma, surgery, tumor, tuberculosis, or filariasis Gynecologic Ovarian tumors, cyst torsion or rupture Malignancy Leukemia, lymphoma, neuroblastoma Systemic lupus erythromatosus Ventriculoperitoneal shunt Hypothyroidism

Diagnosis

1. Is there associated dyspnea? If there is associated dyspnea, one should look for congestive heart failure, pulmonary emphysema, and other cardiopulmonary conditions. 2. Is there hepatomegaly? If there is associated hepatomegaly, certainly cirrhosis of the liver has to top the list of possibilities, but additional causes of ascites with hepatomegaly are constrictive pericarditis, the cardiomyopathies, Budd-Chiari syndrome, metastatic carcinoma, and hydatid cyst. 3. Is there edema of the lower extremities or significant proteinuria? Edema in the lower extremities along with significant proteinuria certainly suggests a nephrotic syndrome, whether it is due to glomerulonephritis, diabetes, or a collagen disease. It also suggests end-stage nephritis. If there is no significant proteinuria, then a primary peritoneal condition such as tuberculous peritonitis or peritoneal carcinomatosis must be considered. Remember, a large ovarian cyst can simulate ascites. 4. Is there a history of a primary tumor elsewhere? GI tumors may spread to the peritoneal surface and cause ascites, but a malignant melanoma may do the same thing.

Prognosis

The 'prognosis' of Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor usually refers to the likely outcome of Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor. The prognosis of Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor may include the duration of Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor, chances of complications of Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor, probable outcomes, prospects for recovery, recovery period for Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor, survival rates, death rates, and other outcome possibilities in the overall prognosis of Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor. Naturally, such forecast issues are by their nature unpredictable.

Treatment

Treatment is directed at underlying cause Bed rest, fluid, sodium restriction is the first line Diuretics: Careful use in selected cases o Chylous ascites High-protein, low-fat diet supplemented with medium-chain triglycerides Parenteral nutrition may be needed to decrease lymph flow and supplement nutrition Laparotomy may be indicated for failed dietary management, to seal leak site Surgical intervention: Bile or urine ascites Therapeutic paracentesis: May be repeated to relieve respiratory distress or impending umbilical rupture o Portacaval shunt or a peritoneovenous shunt (LeVeen) for intractable ascites Shunt between peritoneal cavity and superior vena cava Transjugular intrahepatic portosystemic shunt (TIPSS) for cirrhosis while awaiting transplantation