Talk:Gastrointestinal Tract - Abnormalities

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Cite this page: Hill, M.A. (2021, February 27) Embryology Gastrointestinal Tract - Abnormalities. Retrieved from


A case of congenital duodenal web causing duodenal stenosis in a down syndrome child: endoscopic resection with an insulated-tip knife

Gut Liver. 2011 Mar;5(1):105-9. Epub 2011 Mar 16.

Lee SS, Hwang ST, Jang NG, Tchah H, Choi DY, Kim HY, Ryoo E. Source Department of Pediatrics, Gachon University of Medicine and Science Graduate School of Medicine, Incheon, Korea.

Abstract A 35-month-old girl visited our hospital with repetitive vomiting and abdominal distention; this was especially aggravated after the introduction of solid and semisolid foods. At 5 months of age, the patient, who had Down's syndrome, had undergone surgery for ventricular septal defect, atrial septal defect, and patent ductus arteriosus, and had subsequently been frequently hospitalized for respiratory infections and other viral infectious diseases. After her admission, the abdominal distension improved with fasting and intravenous fl uid therapy. Radiograph from a small-bowel series revealed a thin fi lling defect with a dilated duodenal bulb in the distal region of the second portion of the duodenum, suggesting a duodenal web, and endoscopy revealed duodenal stenosis. We therefore performed endoscopic resection with an insulated-tip knife because of the history of prior operations, fasting problems after operations, and respiratory infections. Seven days later, scar formation was noted on the second portion of the duodenum, the scope passed well at the excision site, and no retained food material was noted on the follow-up endoscopy. After the procedure, the patient's abdominal distention and repetitive vomiting subsided, and she was discharged with the ability to eat eat an age-appropriate normal diet. There were no specifi c symptoms or other complications for 1 year after the procedure.

PMID: 21461083

Fig. 1 The findings of the gastrointestinal barium study. (A) A beak-like narrowing of the distal esophagus up to the gastroesophageal junction with mild passage disturbance, and a thin filling defect are noted on the distal region of the second portion of the duodenum. (B) Contrast is retained on the dilated stomach and duodenal bulb.

Fig. 3 Initial endoscopic findings. (A) A mucosal diaphragm is seen on the second portion of the duodenum, suggesting a duodenal web. (B) A mucosal diaphragm in the second portion of the duodenum shows a stenotic lumen.


Anomalous opening of the common bile duct into the duodenal bulb: endoscopic treatment

BMC Gastroenterol. 2007 Jul 5;7:26.

Disibeyaz S, Parlak E, Cicek B, Cengiz C, Kuran SO, Oguz D, Güzel H, Sahin B. Source Department of Gastroenterology, Turkiye Yüksek Ihtisas Hospital, Sihhiye, Ankara, Turkey. Abstract BACKGROUND: Anomalous biliary opening especially the presence of the ampulla of Vater in the duodenal bulb is a very rare phenomenon. We report clinical implications, laboratory and ERCP findings and also therapeutic approaches in 53 cases.

METHODS: The data were collected from the records of 12.158 ERCP. The diagnosis was established as an anomalous opening of the common bile duct (CBD) into the duodenal bulb when there is an orifice observed in the bulb with the absence of a papillary structure at its normal localization and when the CBD is visualized by cholangiography through this orifice without evidence of any other opening.

RESULTS: A total of 53 cases were recruited. There was an obvious male preponderance (M/F: 49/4). Demographic data and ERCP findings were available for all, but clinical characteristics and laboratory findings could be obtained from 39 patients with full records. Thirty-seven of 39 cases had abdominal pain (95%) and 23 of them (59%) had cholangitis as well. Elevated AP and GGT were found in 97.4% (52/53). History of cholecystectomy was present in 64% of the cases, recurrent cholangitis in 26% and duodenal ulcer in 45%. Normal papilla was not observed in any of the patients and a cleft-like opening was evident instead. The CBD was hook shaped at the distal part that opens to the duodenal bulb. Pancreatic duct (PD) was opening separately into the bulb in all the cases when it was possible to visualize. Dilated CBD in ERCP was evident in 94% and the CBD stone was demonstrated in 51%. PD was dilated in four of 12 (33%) cases. None of them has a history of pancreatitis. Endoscopically, Papillary Balloon Dilatation instead of Sphincterotomy carried out in 19 of 27 patients (70%) with choledocholithiazis. Remaining eight patients had undergone surgery (30%). Clinical symptoms were resolved with medical treatment in 16(32%) patients with dilated CBD but no stone. Perforation and bleeding were occurred only in two patients, which stones extracted with sphincterotomy (each complication in 1 patient).

CONCLUSION: The opening of the CBD into the duodenal bulb is a rare event that may be associated with biliary and gastric/duodenal diseases. To date, surgical treatment has been preferred. In our experience, sphincterotomy has a high risk since it may lead to bleeding and perforation by virtue of the fact that a true papillary structure is absent. However, we performed balloon dilatation of the orifice successfully without any serious complication and suggest this as a safe therapeutic modality.

PMID: 17610747


Duodenal stenosis, a new finding on congenital rubella syndrome: case description and literature review

J Infect. 2006 Nov;53(5):e207-10. Epub 2006 Mar 20.

Diamanti A, Pietrobattista A, Bevivino E, De Angelis P, Calce A, Dall'Oglio L, Gambarara M. Source Children's Hospital, Gastroenterology and Nutrition Unit, Piazza S. Onofrio 4, Rome, Italy.

Abstract Congenital rubella syndrome (CRS) continues to represent a public healthcare problem although an effective vaccination program. Gastrointestinal involvement is rather infrequent and the association of CRS with duodenal stenosis has been never reported. In this study a case of CRS with duodenal diaphragm is reported and the gastrointestinal diseases described in association with CRS are reviewed. A 10-month-old child affected by CRS with congenital hearth disease, perceptive deafness and microcephaly, was admitted because of vomiting and failure to thrive. An upper endoscopy demonstrated dilated proximal duodenum and a perforated diaphragm in the second segment of the duodenum. Endoscopic membranectomy was therefore performed. Two months later the patient was submitted to a further endoscopic evaluation that showed a partial diaphragm persistence and a second excision was performed. Follow-up one year after the first treatment showed good clinical conditions, reasonable physical growth and disappearance of vomiting. In conclusion we report the first case of CRS in association with duodenal stenosis. Duodenal stenosis in the absence of other intestinal localizations may be due to rubella capacity of infecting only small numbers of fetal cells but we cannot exclude that the duodenal stenosis in our patient be only a casual association.

PMID: 16546260

Cassart M, Massez A, Lingier P, Absil AS, Donner C, Avni F. Sonographic prenatal diagnosis of malpositioned stomach as a feature of uncomplicated intestinal malrotation. Pediatr Radiol. 2006 Feb 8;:1-3

Strouse PJ. Animal models of implantation. Reproduction. 2004 Dec;128(6):679-95. Disorders of intestinal rotation and fixation ("malrotation"). Pediatr Radiol. 2004 Nov;34(11):837-51.


Intestinal atresia and stenosis: a 25-year experience with 277 cases

Arch Surg. 1998 May;133(5):490-6; discussion 496-7.

Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Source Department of Pediatric Surgery, James Whitcomb Riley Hospital for Children, Indiana University Medical Center, Indianapolis 46202, USA. Abstract OBJECTIVE: To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcome in infants with intestinal atresia.

DESIGN: Retrospective case series.

SETTING: Pediatric tertiary care teaching hospital.

PATIENTS: A population-based sample of 277 neonates with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction was duodenal in 138 infants, jejunoileal in 128, and colonic in 21. Of the 277 neonates, 10 had obstruction in more than 1 site. Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%). Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%).

INTERVENTIONS: Patients with duodenal obstruction were treated by duodenoduodenostomy in 119 (86%), of 138 patients duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%) A duodenostomy tube was placed in 3 critically ill neonates. Patients with jejunoileal atresia were treated with resection in 97 (76%) of 128 patients (anastomosis, 45 [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web excision in 5 (4%), and the Bianchi procedure in 1 (0.8%). Patients with colon atresia were managed with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anastomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates.

MAIN OUTCOME MEASURES: Morbidity and early and late mortality.

RESULTS: Operative mortality for neonates with duodenal atresia was 4%, with jejunoileal atresia, 0.8%, and with colonic atresia, 0%. The long-term survival rate for children with duodenal atresia was 86%; with jejunoileal atresia, 84%; and with colon atresia, 100%. The Bianchi procedure (1 patient, 0.8%) and growth hormone, glutamine, and modified diet (4 patients, 1%) reduced total parenteral nutrition dependence.

CONCLUSIONS: Cardiac anomalies (with duodenal atresia) and ultrashort-bowel syndrome (<40 cm) requiring long-term total parenteral nutrition, which can be complicated by liver disease (with jejunoileal atresia), are the major causes of morbidity and mortality in these patients. Use of growth factors to enhance adaptation and advances in small bowel transplantation may improve long-term outcomes.

Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%).

Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%).

Classification of jejunoileal atresia (Gray and Skandalakis)

type I atresia - mucosal web

type II atresia - fibrous cord

type III atresia - complete separation type IIIa (mesenteric gap defect), type IIIb ("apple peel")

type IV atresia - multiple atresias


Initial classification

Gray SW, Skandalakis JE. Embryology for Surgeons. Philadelphia, Pa: WB Saunders Co; 1972:147-148. Modified

Martin LW, Zerella JT. Jejunoileal atresia: a proposed classification. J Pediatr Surg. 1976;11:399-403.

Grosfeld JL, Ballantine TVN, Shoemaker R. Operative management of intestinal atresia and stenosis based on pathologic findings. J Pediatr Surg. 1979;14:368-375.

The 2 major theories regarding the etiology of intestinal atresia are Tandler's concept (Tandler J. Zur Entwicklungsgeschichte des Menschlichen Duodenum in Fruhen Embryonalstadien. Morphol Jahrb. 1900;29:187-216) of a lack of revacuolization of the solid cord stage of intestinal development and the classic study by Louw and Barnard (Louw JH, Barnard CN. Congenital intestinal atresia: observations on its origin. Lancet. 1955;2:1065-1067) suggesting that a late intrauterine mesenteric vascular accident is the cause of most jejunoileal and colonic atresias.

duodenal atresia revacuolization is the probable cause for most cases

jejunoileal atresias occur as a result of intestinal volvulus, intussusception, internal hernia, or strangulation in a tight gastroschisis or omphalocele defect.

PMID: 9605910