Stenosis congenital

Severe congenital stenosis of the small bowel is usually accompanied by vomiting and abdominal distension, identical in severity to that seen in atresia. In less severe cases, these symptoms may be mild or even delayed for several days or weeks. Plain abdominal radiographs reveal dilatation of bowel loops proximal to the stenosis and normal or decreased quantity of air in the small bowel distal to the stenosis (McAlister et al. 1996). 

F7. Forbes, G. B., and Erganian, J. A., Parenteral administration of ammonium chloride for alkalosis of congenital Pyloric stenosis. A.M.A. J. Diseases Children 72, 649 (1946). 

The commonest cause of stenosis in patients below 60 years of age is a congenitally bicuspid aortic valve with fused commissures and/or dysplastic leaflets. Overtime calcification and fibrosis may cause the valve to become rigid and obstructive. Coexisting regurgitation of these valves is common. 

Mitral stenosis is seen typically as a consequence of chronic rheumatic fever. Isolated congenital mitral stenosis is very rare and not suitable for balloon valvuloplasty. Clinical symptoms depend on the degree of obstruction. Dyspnea, atrial fibrillation, embolic events, pulmonary edema, and right heart decompensation may occur and are all indications for treatment. Surgery and catheter intervention provide similar results. Balloon valvuloplasty produces best results in patients with little or no calcification of the mitral leaflets (20—23). 

Kan JS, White Rl Jr, Mitchell SE, et al. Percutaneous balloon valvuloplasty a new method for treating congenital pulmonary-valve stenosis. N Engl J Med 1982 307(9) 540-542. 

This is an autosomal dominant hereditary disorder characterised by a progressive loss of the bile ducts within the liver and narrowing of the bile ducts outside the liver. It is also associated with congenital heart disease, and in particnlar pulmonary stenosis. Symptoms are related to chronic cholestasis and include jaundice, pruritus, pale loose stools and poor growth within the first three months of life. The majority of children have a benign course and many cases go undetected however, there is an overall mortality of 20-30% due to progressive liver disease with the development of cirrhosis, cardiac disease or intercnrrent infection. 

Occlusion of the lacrimal puncta is called atresia when congenital and stenosis when it is acquired. Each produces true epiphora, although congenital cases tend to produce fewer clinical signs and symptoms than do acquired cases. 

Acquired causes may also be responsible for some of the congenital anomalies described above. Mention should be made of (1.) arterioportal fistulas, (2.) cavernous transformation of the portal vein (in portal vein thrombosis), (3.) fibrous obliteration of the portal vein, and (4.) cicatricial portal vein stenosis. These may also cause prehepatic hypertension, (s. tab. 14.2) (s. p. 246 )... 

In a case-control surveillance of congenital abnormahties in 22 865 women who had neonates or fetuses with congenital abnormalities, and 38 151 pregnant women who had neonates without any defects, treatment with nalidixic acid during pregnancy was associated with an increased risk of pyloric stenosis (29). Nalidixic acid should be avoided during pregnancy. 

ECHO remains the procedure of choice in the diagnosis and evaluation of a number of conditions such as valvular dysfunction (aortic and mitral stenosis and regurgitation and endocarditis), wall motion abnormalities associated with ischemia, and congenital abnormalities, such as ventricular or atrial septal defects. Images obtained from ECHO are used to estimate chamber wall thickness and left ventricle ejection fraction, assess ventricular function, and detect abnormalities of the pericardium such as effusions or thickening. 

Survey and its incidence continues to increase along with the growth of elderly population in Western societies (2). Aortic stenosis mostly occurs due to calcific degeneration of a normal trileaflet and less frequently in the setting of a congenital bicuspid valve. While still common in some parts of the world, rheumatic valve disease is now a rare cause in developed countries. 

Pyloric stenosis is congenital hypertrophy of the pylori, which presents with projectile vomiting and a palpable abdominal olive. ... 

Children with severe Congenital mitral stenosis pose a difficult surgical and medical management problem. The frequent occurrence of additional left-sided obstructive disease and intracardiac shunts favors a staged approach to management, involving both surgical and transcatheter interven-... 

We have reported the effect of inhaled nitric oxide in 10 patients (median age, 3.3 years range, 0.4-11.1 years) with symptomatic congenital mitral stenosis.These studies were performed during interventional cardiac catheterization or after surgery requiring the use of cardiopulmonary bypass. Six patients had additional left ventricular outflow tract obstruction (median, 25 mm Hg range, 15-50 mm Hg). Effective relief of the mitral gradient was present after intervention. 

Atz, A., Adatia, I., Moore, P., Jonas, R., and Wessel, D. (1994). Inhaled nitric oxide in congenital mitral stenosis with pulmonary hypertension. Pediatr. Res. 35, 29A... 

Pyloric stenosis may lead to obstruction of the lumen and usually presents beyond the neonatal period. However, it has been diagnosed in utero and can be seen in the neonatal period after administration of prostaglandin E to infants with ductus-dependent congenital heart disease. The stenosis is produced by central foveolar hyperplasia. On sonography, mucosal thickening often with polypoid or lobular appearance is observed, different from the muscular thickening observed in hypertrophic pyloric stenosis (Peled et al. 1992 Babyn et al. 1995) (Fig. 1.2). 

De Backer Al, De Schepper AM, Deprettere A et al (1999) Radiographic manifestations of intestinal obstruction in the newborn. JBR-BTR 82 159-166 De Lorimier A, Fonkalsrud E, Hays D (1969) Congenital atresia and stenosis of the jejunum and ileum. Surgery 65 819-827... 

Complications 89 Congenital Esophageal Stenosis 90 Esophageal Duplication 91 Esophageal Bronchus 91 Vascular Ring Abnormalities 93 Hiatal Hernia 94... 

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