1/10. A male newborn with colonic atresia and total colonic aganglionosis.A case of colonic atresia (CA) and aganglionosis is presented, which is probably the tenth in the English literature. The boy presented on day 3 of life with delayed passage of meconium, milk intolerance, and progressive abdominal distension. A barium enema study was complicated by barium peritonitis. Emergency peritoneal lavage was done and the intestinal obstruction was relieved by a proximal defunctioning ileostomy; type III CA (Grosfeld classification) was identified. The proximal atretic end was the blind-ending caecum and the distal atretic end commenced in the splenic area as a microcolon extending to the pelvis and replacing the normal colon. Total colonic aganglionosis (TCA) was confirmed by biopsies. This is the first case in the literature of TCA with CA documented before any attempts at colonic resection and anastomosis were made. This association highlights the recommendation for biopsy of the micro-colon and rectum when Type III CA (Grosfeld classification) is encountered in a newborn. The infant underwent a cholecystectomy and Duhamel-Martin operation at 14 months that was complicated by abdominal wound dehiscence; the ileostomy was closed at 22 months of age.- - - - - - - - - - ranking = 1keywords = meconium (Clic here for more details about this article) |
2/10. Failure to pass meconium: diagnosing neonatal intestinal obstruction.Timely passage of the first stool is a hallmark of the well-being of the newborn infant. Failure of a full-term newborn to pass meconium in the first 24 hours may signal intestinal obstruction. Lower intestinal obstruction may be associated with disorders such as Hirschsprung's disease, anorectal malformations, meconium plug syndrome, small left colon syndrome, hypoganglionosis, neuronal intestinal dysplasia and megacystis-microcolon-intestinal hypoperistalsis syndrome. Radiologic studies are usually required to make the diagnosis. In addition, specific tests such as pelvic magnetic resonance imaging, anorectal manometry and rectal biopsy are helpful in the evaluation of newborns with failure to pass meconium.- - - - - - - - - - ranking = 7keywords = meconium (Clic here for more details about this article) |
3/10. Hirschsprung's disease presenting with diffuse intestinal pneumatosis in a neonate.In the neonate, pneumatosis intestinalis is almost always associated with necrotizing enterocolitis. The manifestation of diffuse intestinal pneumatosis in Hirschsprung's disease has been reported rarely. It may occur as a result of Hirschsprung's disease complicated with enterocolitis. We report a two-day-old female baby born at term with the problems of failure to pass meconium, progressive abdominal distension and bile stained vomiting. There was an early roentgenographic presentation of pneumatosis intestinalis which might have led to a diagnosis of necrotizing enterocolitis. However, the intestinal pneumatosis resolved within 48 hours. After anorectal manometry and contrast enema examination, an ileostomy was performed at the age of 23 days, and multiple biopsies of intestine showed aganglionosis up to the ileum at the level of 85 cm above the ileocecal valve. Unfortunately, the patient developed short bowel syndrome after operation and died suddenly after an accidental choking at the age of three months. This case suggests that Hirschsprung's disease may have an unusual early roentgenographic presentation with diffuse intestinal pneumatosis in the first few days of life. Anorectal manometries and suction biopsies are crucial for further diagnosis.- - - - - - - - - - ranking = 1keywords = meconium (Clic here for more details about this article) |
4/10. Hirschsprung's disease, associated rare congenital anomalies.OBJECTIVE: Hirschsprung's disease may be associated with a number of congenital anomalies of which Down's syndrome and intestinal atresias are commonly encountered. The study aimed to assess the impact of rare associated anomalies on the diagnosis and management of Hirschsprung's disease. methods: A retrospective review of the clinical presentation, diagnosis and outcome of thirty five consecutive newly diagnosed cases of Hirschsprung's disease encountered over two years was performed. RESULTS: Besides Down's syndrome (two), intestinal atresia (one) and pigmentary ocular defects (two), three rare anomalies (Occipital meningocele, Calcific meconium cyst with anal stenosis, Malrotation) were encountered in four of thirty five cases. The clinical features, radiologic anatomy and gross morphology of the bowel were unconventional and the diagnosis was supported by intraoperative acetylcholinesterase staining of biopsies. Though the diagnosis was relatively delayed in these cases, the outcome has been comparable to the rest. The dilemma in their diagnosis and management and their possible pathoembryology is discussed. CONCLUSION: awareness of such associations and a specific investigative protocol is imperative for timely diagnosis and minimal morbidity in complex presentations of Hirschsprung's disease.- - - - - - - - - - ranking = 1keywords = meconium (Clic here for more details about this article) |
5/10. Neonatal intestinal obstruction simulating meconium ileus in infants with long-segment intestinal aganglionosis: radiographic findings that prompt the need for rectal biopsy.BACKGROUND: The initial clinical presentation and radiographic finding of microcolon in children with long-segment intestinal aganglionosis involving the entire colon, ileum and sometimes the jejunum can mimic meconium ileus. This makes the diagnosis difficult for the radiologist and surgeon. OBJECTIVE: To document and describe the clinical and radiographic findings in children with long-segment intestinal aganglionosis who are initially thought to have meconium ileus. MATERIALS AND methods: We reviewed the cases of six neonates with long-segment intestinal aganglionosis presenting as meconium ileus at our institutions between 1978 and 2002. We examined the clinical presentation and the radiographic, surgical, and pathologic findings. In addition, 17 cases from the literature were identified and are included in the discussion. RESULTS: A total of 23 cases were reviewed. Right lower quadrant intraluminal calcifications were noted on abdominal radiographs in all six neonates of our series and were described in 13 of the 17 neonates reported in the literature. Similarly, a microcolon was present in five of the six neonates of our series and in 14 of 16 historical neonates (one not reported). CONCLUSION: In a neonate with small-bowel obstruction and a microcolon, the presence of right lower quadrant intraluminal calcifications should raise the suspicion of long-segment intestinal aganglionosis even if the operative findings are typical of meconium ileus and a biopsy should be performed.- - - - - - - - - - ranking = 8keywords = meconium (Clic here for more details about this article) |
6/10. Hirschsprung's disease, a rare precipitating factor in neonatal perforated Meckel's diverticulum.Perforation of Meckel's diverticulum (MD) during the neonatal period may occur in the presence of distal colon obstruction. Herein, we describe a unique case of a 2-day-old infant that presented with pneumoperitoneum, in which a perforated MD was induced by distal intestinal obstruction secondary to total colonic aganglionosis. In the setting of neonatal perforated MD found intraoperatively, the determination of the possible precipitating etiology is necessary. The clinical history of delayed passage of meconium is emphasized, and either a rectal or colon biopsy is recommended intraoperatively to avoid overlooking the associated presence of Hirschsprung's disease.- - - - - - - - - - ranking = 1keywords = meconium (Clic here for more details about this article) |
7/10. Total intestinal aganglionosis.Total Intestinal Aganglionosis is a rare, uniformly fatal condition with absence of ganglia from the duodenum to the rectum. A neonate with this extreme form of Hirschsprung's disease is presented with a review of 12 previously reported cases. Smooth muscle strips from this infant's gastrointestinal tract demonstrated viable cholinergic receptors, absence of intrinsic neuronal innervation, and colonic contractile activity to the purported peptidergic neurotransmitter cholecystokinin. Four aspects of total intestinal aganglionosis are noteworthy: (1) one-third of patients presented between four and eight days of age after passing meconium on the first day of life; (2) at laparotomy, no intestinal distention, obstruction, or transition zone was evident; (3) hypertrophic nerve fibers seen in classic Hirschsprung's disease were absent in one-quarter of patients; and (4) a high incidence of affected siblings occurred in the previously reported cases. This form of Hirschsprung's disease may represent a distinct entity with autosomal recessive inheritance and significant risk for recurrence.- - - - - - - - - - ranking = 1keywords = meconium (Clic here for more details about this article) |
8/10. 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)- - - - - - - - - - ranking = 3keywords = meconium (Clic here for more details about this article) |
9/10. Inflammatory pseudocyst associated with trisomy 21 and Hirschsprung's disease.A 15-month-old black male with trisomy 21 and Hirschsprung's disease developed an inflammatory abdominal pseudocyst 1 month after Swenson pull-through repair. Although abdominal pseudocyst associated with ventriculoperitoneal shunts, peritoneal dialysis catheters, meconium peritonitis, and pancreatitis have been reported, this is the first report of a pseudocyst developing 1 month after a surgical repair of Hirschsprung's disease.- - - - - - - - - - ranking = 1keywords = meconium (Clic here for more details about this article) |
10/10. Anorectal malformation presenting as Hirschsprung's disease: a case report.A 3.8 kg female baby, who was born on 7-2-92 by normal vaginal delivery at a zonal hospital in the Southern Highlands of tanzania, was initially noted to have had an "ectopic" anus (an ano-perineal fistula) but was otherwise normal. She had delay in the passage of meconium upto the third day, and by the fifth day had developed marked abdominal distension. Following a period of unsuccessful saline "enemas" she had to undergo a right transverse colostomy in the fourth week (which relieved the distension) for what was then presumed to be Hirschsprung's disease. She was referred to the National Referral hospital in Dar es Salaam a year later, where she was found to have an entirely intra-pelvic sacrococcygeal teratoma in association with a "low" anorectal malformation. This unusual case is of interest because of the rare association of intrapelvic sacrococcygeal teratoma with anorectal malformation which led to the initial mis-diagnosis of hirschsprung's disease.- - - - - - - - - - ranking = 1keywords = meconium (Clic here for more details about this article) |
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