scholarly journals Familial Near-Total Intestinal Aganglionosis

2017 ◽  
Vol 6 (3) ◽  
pp. 62 ◽  
Author(s):  
Saida Hidouri ◽  
Hayet Zitouni ◽  
Jamila Chahed ◽  
Sana Mosbahi ◽  
Samia Belhassen ◽  
...  

Near total aganglionosis represents the most extreme and rare form of Hirschsprung's disease. It can affect more than one member of family. We report three cases of near total intestinal aganglionosis in a family presenting with intestinal obstruction at birth. All of them were operated and a jejunostomy was performed. Outcome was dismal.

PEDIATRICS ◽  
1983 ◽  
Vol 72 (4) ◽  
pp. 585-585
Author(s):  
S. R. POTTS ◽  
S. BROWN ◽  
B. T. SMYTH

To the Editor.— We wish to report our recent experience with the use of the decapeptide caerulein in managing a case of total intestinal aganglionosis. The child, a male born at term, had had multiple intestinal biopsies and had had two previous laparotomies (one for obstruction mistakenly thought to have been mechanical and the other for enterocolitis at which a double-barrelled enterostomy was fashioned) when a diagnosis of intestinal agangliosis was made at 31 days of age. Prior to the third intervention, Hirschsprung's disease was suspected but preoperative rectal suction biopsies although aganglionic did not show acetylcholinesterase staining.


2019 ◽  
Vol 23 (1) ◽  
pp. 52-53
Author(s):  
Gennady I. Chepurnoy ◽  
A. V. Leiga ◽  
V. B. Katsupeyev ◽  
M. G. Chepurnoy ◽  
N. A. Mikhanoshina ◽  
...  

There is presented a clinical observation of such rare form of Hirschsprung’s disease in a newborn -as terminal aganglionosis of the ileum and hypo-ganglionosis of the cecum, manifested by acute intestinal obstruction. Resection of the ileocecal angle and leaving the distal colon in the child’s body with good functional results in the long-term postoperative period made it possible to affirm the existence of high isolated segmental forms of Hirshprung’s disease.


Author(s):  
Manoochehr Ghorbanpour ◽  
Mohammad Ali Seyfrabie ◽  
Babak Yousefi

Objective. Patients undergoing Soave surgery for Hirschsprung's disease are at risk for some complications. The aim of this study was to investigate such short-term and long-term complications and evaluate the outcome of the operation in these patients. Methods. A case series study was carried out during the last 12 years, during 2007 to 2018 in Besat hospital of Hamadan. Data collection conducted using a checklist includes questions about demographic information, clinical features, and short-term and long-term complications, and consequences of post-operative surgery. The findings of the study were analyzed using SPSS software version 20 and appropriate statistical tests. P-value less than 0.05 was considered statistically significant. Results. A total of 55 children underwent Soave surgery during the last 12 years in Besat Hospital Hamadan, Iran. The mean age of the patients was 38±10 days during surgery, of which 56.4% were female. The mean hospital stay was 7.3 days. Also, the mean weight of children at birth was 2970±447 gr. Most of the patients were born as NVD (52.7%) and term (74.5%). The most common comorbidity was congenital heart disease. The most common short-term complication was intestinal obstruction in 14 patients (25.5%) and the most frequent long-term complication was intestinal obstruction and constipation (27.3% each cases). The mortality rate of patients in this study was 14.5% in total. Conclusions. One stage surgical procedure in Hirschsprung's disease is a safe and effective method, but care should be taken in choosing patients and patients should be monitored for possible complications, so that they can be considered and implemented for proper treatment.


2018 ◽  
Vol 06 (01) ◽  
pp. e94-e96
Author(s):  
Fabrizio Vatta ◽  
Alessandro Raffaele ◽  
Noemi Pasqua ◽  
Marco Brunero ◽  
Gloria Pelizzo ◽  
...  

AbstractHirschsprung's disease and, more rarely, intestinal duplication can both cause intestinal obstruction in neonates. The simultaneous occurrence of these two diseases is reported in only two studies, and in both cases, intestinal duplication was an incidental finding, as it had not determined clinical intestinal occlusion. This paper reports a unique case of coexistence of the two conditions, with both causing intestinal obstruction, delayed appropriate, and definitive surgical treatment.


2021 ◽  
Vol 25 (3) ◽  
pp. 209-213
Author(s):  
A. N. Smirnov ◽  
V. V. Кholostova ◽  
S. A. Voyna ◽  
V. S. Gladeeva ◽  
M. A. Amanova ◽  
...  

Introduction. Colon atresia is a rare form of congenital bowel obstruction. In scientific literature, there are isolated publications devoted to colon atresia, and only some authors discuss the membranous form of atresia.Material and methods. The article describes a rare clinical case of an incomplete colon membrane in an infant. At the age of 11 months, the child developed symptoms of acute intestinal obstruction. Irrigography was performed; Hirschsprung’s disease was suspected. At the first stage, the child was taken a floor-by-floor biopsy of the colon, and an ileostomy was put. Visually, Hirschsprung’s disease seemed to be present; however, histological examination turned aside this diagnosis. Given this, the colon membrane was suspected. Partial resection of the altered part of the sigmoid colon was performed with good clinical effect. Histological examination revealed an incomplete serous-muscular membrane of the colon.Conclusion. Colon atresia is a rare disease that presents a serious problem for diagnosis and treatment. Preoperative morphological verification of the diagnosis is important, even in case when Hirschsprung’s disease seems obvious. Preoperative diagnostics is important because surgical tactics may be considerably different in colon local lesions. 


2015 ◽  
Vol 22 (1) ◽  
pp. 65-68
Author(s):  
Nouf Y. Akeel ◽  
Jamal S. Kamal ◽  
Aisha M. Abdulrahman

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.  


2014 ◽  
Vol 21 (01) ◽  
pp. 020-026
Author(s):  
Muhammad Imran Ishfaq ◽  
Umar Farooq Ahmad ◽  
Saima Manzoor

Objective: To determine the frequency of Hirschsprung’s Disease as a cause ofneonatal intestinal obstruction, to compare the results of treatment with others and to suggestmeans and ways to improve the deficiencies in our circumstances. Place and duration of study:Nishtar hospital and Ibn-e-Siena Hospital, Multan from January, 2008 to June, 2012.Methodology: A total number of 115 patients from neonatal age to more than 5 years werediagnosed and treated for Hirschsprung’s Disease. Results: Hirschsprung’s Disease was acause of neonatal obstruction in 26 (22.6%) patients. Hirschsprung’s Disease was a cause ofrecurrent diarrhea in 37 (32.17%) patients. 76 (66.08%) patients presented with chronicconstipation. 27 patients underwent for pull-through operation. Conclusions: The overallcomplications rate and stooling pattern after Soave’s procedure are not significantly differentfrom others.


Sign in / Sign up

Export Citation Format

Share Document