Intestinal atresia and Hirschsprung's disease (original) (raw)
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Colonic Atresia and Hirschsprung's Disease: A Case Report from King Abdulaziz University Hospital
Journal of King Abdulaziz University - Medical Sciences, 2015
Atresia of the colon is a rare form among all types of intestinal atresia. Further, the association between colonic atresia and aganglionosis is extremely rare; it may be easily overlooked due to the associated distal microcolon. Management of a case of colonic atresia with distal aganglionosis presented with anastomotic leak due to unsuspected aganglionosis was discussed.
Colonic atresia and hirschsprung disease: a case report and review of the literature
Journal of Medical Case Reports, 2023
Background Colon atresia is one of the rarest congenital anomalies of the gastrointestinal tract, with an incident range of between 1 in 10,000 and 66,000 live births. Type I colonic atresia affects only the mucosal layer of the intestine and spares the intestinal wall and mesentery. Hirschsprung Disease is a rare association of Colon atresia and is usually diagnosed as a complication of atresia treatment. Case presentation This study reports a 14-h term white middle-eastern female infant with type I transverse colonic atresia complicated by the association of Hirschsprung disease and provides a brief literature review of the topic. She presented with poor feeding, weakness, and failure to pass meconium, and her abdominal X-ray showed complete distal bowel obstruction. The presence of Hirschsprung disease was realized after complications of atresia surgery. The infant underwent a total of three surgeries involving an end-to-end anastomosis of the atresia, colostomy formation following anastomosis leakage, and Hirschsprung surgery. The patient ultimately expired. Conclusions The association between colonic atresia and Hirschsprung disease poses a diagnostic and therapeutic challenge. Considering Hirschsprung disease as a possible association in colon atresia patients can facilitate proper decision-making in the course of treating colon atresia cases and achieving better outcomes.
Pediatric Surgery International, 2006
Colonic atresia (CA) is an infrequent cause of lower gastrointestinal obstruction in the neonate. Coexistence with aganglionosis of the colon (Hirschsprung's disease) has been reported but is generally not recognized in the neonatal period. We report another case and present a review of the literature. A boy with a lower gastrointestinal obstruction, caused by a CA type III, had creation of a proximal colostomy and a distal mucous fistula on the 1st day of life. In the preoperative work-up before restoring the continuity, rectal suction biopsies revealed the presence of Hirschsprung's disease. When the boy was 6 months old, a distal colectomy and reanastomosis were done. Creation of a colostomy and reanastomosis in a second procedure is recommended for treating a type III CA unless distal aganglionosis has been ruled out.
Ileal atresia associated with Hirschsprung disease (total colonic aganglionosis
Journal of Pediatric Surgery, 2005
One of the most common causes of small bowel obstruction in newborns is ileal atresia, and one of the most common causes of colonic obstruction in neonates is aganglionic megacolon (Hirschsprung disease). However, atresias of the small intestinal tract associated with Hirschsprung disease are extremely rare. We describe an infant born with both ileal atresia and Hirschsprung disease. This is the 19th known report of the case of an infant who had ileal atresia associated with Hirschsprung disease. D
Jejunal Atresia with Hirschsprung’s Disease: A Case Report
Journal of Regenerative Medicine & Biology Research, 2021
A rare case of congenital intestinal atresia associated with Hirschsprung disease was reported. A 0-day-old boy who was diagnosed to have intestinal atresia and he underwent intestinal anastomosis. The movement of the intestinal tract was slow after surgery. The abdominal distension is getting stronger. The radiograph and contrast enema revealed the possibility of Hirschsprung disease. The biopsy of rectum also revealed aganglionosis. Transverse colostomy was performed on the 145 th days after surgery of ileal atresia. The radical surgery for laparotomy-assisted trans anal pull-through technique was performed on the 376 th days after first surgery. Discussion was made as to its pathogenesis and timing of surgery.
Light and electronmicroscopy of the bowel in an unusual form of Hirschsprung's Disease
APMIS, 1989
In a histological and histochemical study of multiple biopsies of unaffected segments of the bowel from 15 patients with Hirschsprung's Disease (H.D.). the AChE or non-specific esterase and the NADPH tetrazolium reductase enzyme reactions proved to be useful in identification of myenteric plexus islands; and acid phosphatase for the delineation of individual neurones. In the affected segment (usually aganglionic), this myenteric plexus tissue was not reactive for esterase, but individual nerve fibres among muscle fibres of the two muscle coats showed the enzyme product in a third of the cases. Fine structural study of biopsies from a typical case of H.D.. showed normal looking axons and Schwann cytoplasm with terminals bearing both andrenergic and cholinergic vesicles in the unaffected colon, smooth muscle fibres with normal fine structure in all parts of the bowel. and loss of neurones with myenteric plexus replaced by nerve fibre groups in the affected rectosigmoid. One patient clinically presenting as a case of severe H.D., with histologically and histochemically normal myenteric and submucous ganglion cells, and not responding to resection of the bowel. showed degeneration of the unmyelinated axons with prominent Schwann cytoplasm, depleted cholinergic but persistent adrenergic vesicles, and markedly thinned and degenerating smooth muscle fibres and myofilaments, suggesting either a primary disorder of muscle tissue of the colon or. less likely. a denervation atrophy with secondary degeneration of the smooth muscle fibres.
Hirschsprung disease and other intestinal neuropathies in children
2020
Proper intestinal motility depends on interaction between muscles, nerve cells, and tendinous connective tissue of muscularis propria. Intestinal motility disorders refer to varied intestinal neuromuscular pathologies, including enteric neuropathies. The most common symptoms of these diseases are delayed passage of meconium in newborns and chronic constipation in infants and older children. If organic causes of clinical features are detected, a further multidisciplinary team approach for the management of these patients is recommended. Entities discussed in this review include Hirschsprung disease, hypoganglionosis, intestinal neuronal dysplasia, ganglioneuromatosis, and chronic intestinal pseudo-obstruction. Emphasis is given to the clinical symptoms and diagnostic features that distinguish these conditions enabling faster diagnosis and appropriate treatment.
Constipation and Dilated Bowel: Hirschsprung’s Disease Is Not Always the Case
Clinics and Practice
A case of a 2-month-old boy with constipation and a localized abdominal distension in the right abdomen and hypogastrium is presented. Plain radiograph, ultrasound exam as well as a barium enema were suggestive of Hirschsprung’s disease (HD) (ultrashort segment disease). Nevertheless, rectal suction biopsy was negative for neuronal abnormalities and unexpectedly on plain radiograph vertebral abnormalities were noticed. Subsequently magnetic resonance imaging of the spine and abdomen was performed, to evaluate possible spinal cord lesions and potential abnormalities of the perineal region musculature. A syringe throughout medullary cone was noted. The well-known issue, that the diagnosis of HD does not depend on the imaging (radiological) findings, was confirmed. The step-bystep diagnostic approach from the initial thought of ultrashort segment HD to the later imaging-based diagnosis of syringomyelia is discussed in this present paper.