Chronic bowel obstruction from colonic stenosis in early infancy-A report of two cases.

Bowel obstruction in early infancy may result from a variety of congenital anomalies involving parts of the small and large bowel. However, in infancy, chronic bowel obstructions from congenital or acquired stenosis of the colon are rare and can cause diagnostic quandary. We present two cases of an eleven-week old male and a nine-week old male with massive abdominal distension and features of chronic bowel obstruction dating from neonatal period. In the first case investigations were inconclusive and laparotomy revealed isolated stenosis of the ascending colon. In the second case colonic stenosis was suspected preoperatively and a barium enema done showed multiple colonic stenosis confirming our working diagnosis. The diagnostic dilemmas encountered in managing the first patient are discussed to highlight the need for high index of suspicion of this condition in infants with chronic constipation. The way experience in managing the first case influenced diagnosis of the second case is also highlighted.

Congenital colonic atresia: report of one case.

Colonic atresia is a very rare cause of intestinal obstruction, and surgical management is the mainstay of therapy. A case of congenital colonic atresia is reported in a full-term neonate who presented with delayed passage of meconium, abdominal distention and bilious vomiting. The present case and the pertinent literature are discussed, with an emphasis on surgical management.

Congenital stenosis of the colon with foreign bodies. Case report.

BACKGROUND: Colonic stenosis is rarely seen in pediatric practice. Its incidence has been reported as 1/40,000 births. Ascending and transverse colon are the most common sites affected. Symptoms may be present at birth. CLINICAL CASE: We present the case of a 2 year, 11 month old male. Since birth, the patient had symptoms of constipation, abdominal pain and abdominal distension. X-ray of the abdomen showed a dilated colon and foreign bodies on the right side. Barium enema was normal. The stenotic segment was resected. Foreign bodies were removed and alimentary continuity was restored by end-to-end anastomosis. The pathologist found a 5-cm lumen at the stenosis. Histological ganglion cells were present throughout the specimen. The patient evolved favorably postoperatively and was discharged 1 week after surgery. The patient has been in good health for 2 years. CONCLUSIONS: Colonic stenosis is uncommon. Abu-Judeh reported on one case and a review of the literature revealed eight cases of congenital colonic stenosis since 1941. Symptoms are abdominal distension, vomiting and constipation with symptom remission, making diagnosis difficult. This is the first report involving foreign bodies. Barium enema and colonoscopy are necessary for diagnosis. Resection of the stenotic segment with end-to-end anastomosis or colostomy is the treatment of choice. Laparoscopy is an alternative procedure.

Estenosis congénita de colon con retención de cuerpos extraños: informe de un caso / Congenital stenosis of the colon with foreign bodies: case report

Introducción: La estenosis del colon es rara, con una incidencia de uno entre 40 mil nacimientos. El colon ascendente y transverso son los más afectados. Las manifestaciones clínicas pueden aparecer desde el nacimiento. Caso clínico: Niño de dos años 11 meses de edad, con cuadros periódicos de estreñimiento, dolor tipo cólico y distensión abdominal. La radiografía simple de abdomen mostró dilatación de asas y cuerpos extraños. El colon por enema fue normal. Por laparotomía exploradora se identificó estenosis en el colon transverso. Se practicó resección, extracción de cuerpos extraños y anastomosis término-terminal. El examen histopatológico mostró estenosis con lumen de 0.5 cm; las células ganglionares estuvieron presentes entre las capas musculares. A dos años de la cirugía, el paciente se encontraba asintomático. Conclusiones: La estenosis congénita de colon es poco frecuente. Abdu-Judeh informa un caso y refiere ocho descritos desde 1941. Las manifestaciones clínicas son estreñimiento, vómito y distensión abdominal, sin embargo, los pacientes pueden permanecer asintomáticos por periodos variables, lo que dificulta y retrasa el diagnóstico. No existe informe previo acerca de la retención de cuerpos extraños. El colon por enema y la colonoscopia son necesarios para el diagnóstico oportuno. El tratamiento electivo es la resección quirúrgica con anastomosis primaria o colostomía; una opción es la cirugía laparoscópica.

BACKGROUND: Colonic stenosis is rarely seen in pediatric practice. Its incidence has been reported as 1/40,000 births. Ascending and transverse colon are the most common sites affected. Symptoms may be present at birth. CLINICAL CASE: We present the case of a 2 year, 11 month old male. Since birth, the patient had symptoms of constipation, abdominal pain and abdominal distension. X-ray of the abdomen showed a dilated colon and foreign bodies on the right side. Barium enema was normal. The stenotic segment was resected. Foreign bodies were removed and alimentary continuity was restored by end-to-end anastomosis. The pathologist found a 5-cm lumen at the stenosis. Histological ganglion cells were present throughout the specimen. The patient evolved favorably postoperatively and was discharged 1 week after surgery. The patient has been in good health for 2 years. CONCLUSIONS: Colonic stenosis is uncommon. Abu-Judeh reported on one case and a review of the literature revealed eight cases of congenital colonic stenosis since 1941. Symptoms are abdominal distension, vomiting and constipation with symptom remission, making diagnosis difficult. This is the first report involving foreign bodies. Barium enema and colonoscopy are necessary for diagnosis. Resection of the stenotic segment with end-to-end anastomosis or colostomy is the treatment of choice. Laparoscopy is an alternative procedure.

Congenital colonic stenosis: a case of late-onset.

PURPOSE: Colonic atresia and stenosis are rare causes of intestinal obstruction in the infant. Only 1.8%-15% of intestinal atresias occur in the colon. Congenital colonic stenosis is even less common than colonic atresia. Only 10 cases have been reported in Literature since 1966 and only one late-onset case has been reported in Literature until now. We describe the case of a 4-month-old baby coming to our attention because of an intestinal subocclusion due to a congenital colonic stenosis of the ascending colon. CASE REPORT: A 4-month-old baby came to our attention for persistent abdominal distension, reduction of bowl function and decaying of overall clinical conditions. A plain abdominal radiograph showed distended intestinal loops with air-fluid levels and no gas in the rectum. During the barium enema the contrast medium appeared to completely fill the lumen of the colon up to the ileo-cecal valve and Cecum appearing higher than normal. Beyond the ileo-cecal valve, the contrast medium showed an abnormal hypotonic dilatation of the small intestinal loops. Suspecting an organic intestinal obstruction, an explorative laparotomy was deemed necessary and at halfway in the ascending colon a stenosis was found. RESULTS: The post-operative course was uneventful and the patient is currently in good clinical conditions, has a normal diet and is thriving. CONCLUSION: Considering both the Literature and our own experience, it is wise to reckon the congenital colonic stenosis as a rare but possible cause of complete or partial intestinal obstruction not only in the newborn but also throughout the first year of life.

Pathology of chronic constipation in pediatric and adult coloproctology.

In colonic motility disorders, a pathohistological diagnosis based solely on formalin-fixed gut is often inconclusive. Classical histological techniques or immunohistochemistry represent a static staining. In contrast, native tissue submitted to enzyme histochemistry provides functional information about the effectiveness of the cellular performance. Routinely, a complementary set of reactions is performed and includes acetylcholinesterase (AChE), lactic and succinic dehydrogenase, as well as nitroxide synthase reactions. In this monograph, the whole spectrum of different anomalies of the colonic wall is illustrated in a systematic fashion: Hirschsprung's disease is characterized by an increase in AChE activity of parasympathetic nerve fibers of the rectosigmoid. In ultrashort Hirschsprung's disease, only enzyme histochemistry renders a reliable diagnosis possible in biopsies of the anal ring. Aganglionosis of the musculus corrugator cutis ani shows a localized increase of AChE activity in nerve fibers, similar to Hirschsprung's disease, not detectable in conventional histology. Immaturity, hypoganglionosis and neuronal dysganglionosis can be clearly recognized in dehydrogenase reactions. Enzyme histochemical reactions are complemented by picrosirius red staining for assessment of the collagen texture of the muscularis propria. Absence or intertenial interruption of the continuous connective tissue layer between circular and longitudinal muscle of the muscularis propria has been termed aplastic or atrophic desmosis, respectively. Many of the entities described are also observed in adults. Atrophic hypoganglionosis or atrophic desmosis with loss of the myenteric plexus connective tissue fascia is implied as a frequent cause of chronic constipation in adults. The essential contribution of a functional histopathological technique towards a reliable diagnosis of gut dysfunction in native tissue is extensively demonstrated in great detail in more than two hundred figures.

The complete spectrum of neurocristopathy in an infant with congenital hypoventilation, Hirschsprung's disease, and neuroblastoma.

Neuroblastoma, Hirschsprung's disease, and central hypoventilation (Ondine's curse) are considered aberrations of neural crest cell growth, migration, or differentiation, and as such are considered to be under the general heading of neurocristopathy. Their combined occurrence in a newborn infant presenting with total colonic aganglionosis, central hypoventilation, and multifocal neuroblastoma had not been reported previously. A 2.3-kg white full-term girl required endotracheal intubation because of persistent apnea in the first hours of life. She had progressive abdominal distension and failure to pass meconium; a barium enema was performed, which showed microcolon with meconium pellets at the distal ileum. During laparotomy the distal ileum was found to be obstructed with inspissated meconium; an ileostomy and appendectomy were performed. The resected specimens were aganglionic. An additional 20 cm of aganglionic ileum was removed, and a normally innervated ileostomy was constructed. Numerous attempts at extubation failed because of apnea. The results of an extensive apnea workup, including electroencephalogram, magnetic resonance imaging (MRI), bronchoscopy, and pH probe study, were normal. Sleep studies showed congenital central hypoventilation syndrome, and the patient underwent a tracheostomy. At 3 months, an abdominal ultrasound examination performed within a septic workup showed a right suprarenal mass extending across the midline. Thoracic and abdominal MRI scans showed large bilateral adrenal and posterior mediastinal masses. The serum catecholamines and ferritin level were markedly elevated, suggestive of neuroblastoma. In light of the child's multiple problems, the family chose to forgo further workup (including a tissue biopsy) and therapy. In the following 2 months her tumor load rapidly progressed, and she died of respiratory insufficiency.(ABSTRACT TRUNCATED AT 250 WORDS)

Infants of diabetic mothers: radiographic manifestations.

The radiographic findings of a series of infants of diabetic mothers and a review of the literature are presented to illustrate the wide spectrum of abnormalities that may be seen with this condition. Congenital anomalies of the spine and skeletal, genitourinary, and cardiovascular systems and visceral situs inversus are significantly more frequent among infants of diabetic mothers than normal. The most specific anomaly is sacral agenesis. Renal vein thrombosis and adrenal hemorrhage are also more common and may be diagnosed by sonography. Over one-half of the cases of the small left colon are associated with maternal diabetes and may be diagnosed and treated with a contrast enema. The incidence of the respiratory distress syndrome is higher in infants of diabetic mothers than other premature infants, and the disease may occur in the presence of reliable indicators of lung maturity. Other common causes of dyspnea include cardiomyopathy, congenital heart disease, wet lung syndrome, hyperviscosity syndrome, and persistence of fetal circulation. Echocardiography is the most valuable adjunct in differentiating cardiac from pulmonary problems.

Neonatal small left colon syndrome. Occurrence in asymptomatic infants of diabetic mothers.

An unusually high incidence (40%) of maternal diabetes was observed in a series of 20 newborn infants who had low colonic obstruction and barium enema findings of a uniformly narrowed colon from the splenic flexure to the anus. This has been termed the "neonatal small left colon syndrome." We investigated the incidence of this colon configuration in gastrointestinally asymptomatic infants of diabetic mothers. Of 12 such infants examined by barium enema, six have shown this same narrowing of the left colon. The relationship of maternal diabetes to the small left colon is not yet clearly understood.