Management Of Hirschsprung's Disease Research Articles

Combined Radial Incision and Steroid Injection for Refractory Colorectal Stenosis After Pull-Through Surgery in Hirschsprung Disease: An Innovative Conservative Treatment.

Anastomotic stenosis after pull-through surgery remains a challenge in the management of Hirschsprung disease. Based on the management of esophageal stenosis, we evaluated the efficacy of combined radial incision and steroid injection for the treatment of refractory colorectal anastomotic stenosis after pull-through. Combined radial incision and steroid injection is an alternative conservative treatment of refractory anastomotic stenosis after pull-though for Hirschsprung disease, avoiding a potential complicated redo pull-though surgery. We included patients with recto-sigmoid Hirschsprung disease that developed a refractory anastomotic stenosis after a laparoscopic-assisted Swenson pull-through at Robert-Debré Children University Hospital, Paris, France. Refractory stenosis was defined as obstructive symptoms associated with an anastomotic stenosis upon rectal exam without improvement after serial anal dilatations. Under general anesthesia, an injection of 10mg delayed-action steroid per quadrant was combined with a radial incision of the stenosis using monopolar cautery. Combined radial incision and steroid injection was performed in 4 children, for either early or late refractory stenosis. This resulted in improvement of refractory anastomotic stenosis, avoiding a redo pull-through in 75% of patients. One child presented with transient improvement after combined radial incision and steroid injection but developed recurrent stenosis despite additional combined radial incision and steroid injection and redo pull-through. The median follow-up was 29 months. We observed a clinical improvement in all the patients after combined radial incision and steroid injection. Steroids injection should be considered as a potential alternative therapy for anastomotic stenosis.

Comparative Statistical Analysis of Surgical Procedures in the Management of Hirschsprung Disease

Comparative Statistical Analysis of Surgical Procedures in the Management of Hirschsprung Disease

Outcome Analysis of Single-Stage Transanal Endorectal Pull Through in Selected Patients with Hirschsprung Disease

Background:Hirschsprung disease is a notable cause of neonatal intestinal obstruction and constipation in older children. Transanal endorectal pull through (TEPT) is a newer technique of definitive management as against staged procedures. The aim of our study is to evaluate the feasibility and outcome of the procedure in selected children with Hirschsprung disease managed by this technique with review of the literature.Materials and Methods:Medical records of 12 children who underwent single-stage TEPT in a tertiary care centre over a period of 3 years from 2015 to 2018 were reviewed and retrospectively analysed on the basis of age, investigations, intraoperative parameters, complications, functional outcome and hospital stay.Results:The median age at surgery was 9 months. Nine patients were boys. The median weight of patients was 7.5 kg. The transition zone was observed at the level of the rectosigmoid in eight patients (66.6%) and sigmoid colon in four patients (33.3%). The mean length of muscle cuff was 3 cm, the mean length of resected bowel was 25 cm, the median operative time was 105 min and the mean hospital stay was 8 days. Perianal excoriation (n = 2) and enterocolitis (n = 1) were complications encountered postoperatively; however, no patient had cuff abscess, anastomotic leak or stricture. Stool frequency initially at 2 weeks was average of six to ten times a day, which gradually reduced to two to three times a day by 3 months postoperatively. None of the patients had faecal soiling or constipation on follow-up.Conclusion:Single-stage transanal endorectal pull through is an effective technique in the management of Hirschsprung disease with minimal complications.

Su1763 – Coloanal Anastomosis for Operative Management of Hirschsprung's Disease in Adults

Su1763 – Coloanal Anastomosis for Operative Management of Hirschsprung's Disease in Adults

Management of Hirschsprung disease in Australia and New Zealand: a survey of the Australian and New Zealand Association of Paediatric Surgeons (ANZAPS).

To define the practice of management for Hirschsprung disease (HD) in Australia and New Zealand. Online survey of Australian and New Zealand Association of Paediatric Surgeons (ANZAPS) members. 56/80 (70%) members from 17 centres responded. 100% perform suction rectal biopsies; 40% perform a contrast enema. H&E (94%), ACHE (70%) and calretinin (75%). Primary pull-through (PT) is performed by 88% (100% by < 6/12months). The Soave-Boley PT is the preferred approach (85%), with laparoscopic assistance (77%) and muscle cuff division (93%). Routine post-operative dilatations are performed by 63% of respondents. If symptoms persist following PT, majority adopt a conservative approach (enemas/laxatives 90%; Botox 74%). If a long-segment is identified at PT, 60% fashion a stoma and delay definitive surgery. If total colonic aganglionosis is identified at PT, 76% fashion a stoma and delay definitive surgery. A dedicated bowel management program is available in 45% of centres with transition to adult services in 29%. A laparoscopic-assisted Soave-Boley PT is the most common technique for recto-sigmoid HD. Differences are noted in both the management of long-segment/total aganglionosis HD and post-operative management/follow-up.

The impact of variation in access to care on the management of Hirschsprung disease

IntroductionVariation in access to care has a significant impact on the disease management process and outcomes. Variable access to care might have similar effects on the management of Hirschsprung disease (HD). However, such variation has not been highlighted. Materials and MethodsAll patients referred to 3 academic centers (ACs) with HD were reviewed. Patient presentations, referral patterns, timing, and type of surgical intervention were compared between patients born in AC and those born in non-AC. Babies born with major congenital anomalies (MCAs) or total colonic HD were excluded. ResultsBetween 1998 and 2011, 129 patients were identified. After excluding 30 patients, 99 were split into 20 inborn patients (AC) and 79 outborn patients. Outborn patients more often presented with constipation (95% vs 65%, P = .001), whereas inborn patients presented with feeding intolerance or vomiting (75% vs 39%, P = .004). Outborn patients were diagnosed and had their pull-through (PT) at an older median age (in days) of 186 (1-2621) vs 4.5 (1-451) (P = .001) and 345 (11-2757) vs 92 (3-928) (P = .001), respectively. Moreover, inborn patients were more likely to undergo primary PT (75% vs 46%, P = .02) and avoid bowel diversion (95% vs 66%, P = .02). ConclusionVariation in access to care can have a significant impact on the quality of care delivery in HD. Limited access to AC is associated with staged PT in addition to delay in the diagnosis and management.

Management of Hirschsprung's disease ? an alternate approach

Management of neonatal Hirschsprung's disease based on diagnosis by histologic techniques and frozen acceptable method. However, the non-availability of histologic and histochemical techniques often necessitates alternate approaches. In 376 infants below the age of 2 months who were admitted with the presumptive diagnosis of Hirschsprung's disease, a barium enema was the key investigation. Histologic confirmation could be obtained after a colostomy was established. Manometry allowed the detection of any diagnostic errors. The overall error rate was approximately 4%. An alternate algorithm is suggested for the management of Hirschsprung's Disease, particularly in less well-developed centers.