Short-Segment Hirschsprung's Disease

The clinical features and treatment of 15 children with short-segment Hirscbsprung's disease were reviewed. It accounted for 25% of all Hirschsprung's cases seen 1975–84. The symptomatology, which was generally mild, consisted of slowly progressive abdominal distension with one of three defaecating patterns: persistent or intermittent diarrhoea; 2–3 normal daily motions; and constipated stool passed infrequently. Diagnosis was based mainly on the clinical features and barium enema findings. Eight patients had formal sphincterectomy and 3 had rectal muscle biopsy; follow up to 2½ years in some of the patients with sphincterectomy showed satisfactory results. Factors that would expedite clinical diagnosis are discussed.

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Short segment Hirschsprung's disease as a cause of discrepancy between histologic, histochemical, and clinical features

Journal of Pediatric Surgery ◽

10.1016/s0022-3468(83)80543-0 ◽

1983 ◽

Vol 18 (2) ◽

pp. 167-171 ◽

Cited By ~ 11

Author(s):

C.W. Chow ◽

P.E. Campbell

Keyword(s):

Clinical Features ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Short Segment

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ROENTGENOGRAPHIC MANIFESTATIONS OF CONGENITAL MEGACOLON (HIRSCHSPRUNG'S DISEASE) IN EARLY INFANCY

PEDIATRICS ◽

10.1542/peds.18.2.227 ◽

1956 ◽

Vol 18 (2) ◽

pp. 227-238

Author(s):

Carroll Z. Berman

Keyword(s):

Small Bowel ◽

Barium Enema ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Ileocecal Valve ◽

Congenital Megacolon ◽

Inverted Position ◽

Proximal Half ◽

Fluid Levels

The roentgenographic findings in the first month of life were reviewed in a series of 23 infants with histologically proven [See FIG. 9. in source pdf] Hirschsprung's disease. The plain roentgenograms of 18 of the babies showed significant large bowel distention and 12 had prominent colonic fluid levels. In 15 of 19 of the patients which had been subjected to barium enema, the roentgenograms are diagnostic of congenital megacolon. In 5 of 7 of the infants contrast enemas in the first week of life demonstrated the characteristic deformity. The conclusions which appear justified from this investigation are: Congenital megacolon (Hirschsprung's disease) may be strongly suspected in the newborn period in a large majority of cases from the appearance of plain roentgenograms of the abdomen made in recumbent and erect projections. In some instances, roentgenograms in the inverted position supply additional or substantiating evidence of the disease. In lesions starting in the proximal half of the colon, the abdominal distention involves mainly small bowel, and the differentiation from low jejunal or ileal obstruction is usually not possible from the plain roentgenograms. Here examination by barium enema may be expected to establish the diagnosis. When the segment of achalasia begins above the ileocecal valve, the plain roentgenograms again indicate small bowel obstruction, but here barium enema does not demonstrate a significant contour or caliber abnormality of the colon. In this type of case, follow-up roentgenograms 24 and 48 hours after the enema will often show considerable retention of the barium thus suggesting the diagnosis which must be confirmed by biopsy. When the proximal end of the involved segment of colon is low in the rectum, the lesion may be very difficult to demonstrate by barium enema. The age factor alone, i.e., the performance of the examination very early in infancy, does not of itself preclude demonstration of the lesion of Hirschsprung's disease by barium enema.

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Is Single-stage Primary Transanal Endorectal Pull through (TEP) feasible for Short Segment Hirschsprung's Disease in Neonates?- Our Experience in a Tertiary Hospital

Journal of Surgical Sciences ◽

10.3329/jss.v18i2.43755 ◽

2019 ◽

Vol 18 (2) ◽

pp. 45-50

Author(s):

Md Shahjahan ◽

Kazi Md Noor ul Ferdous ◽

M Kabirul Lslam

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Anastomotic Stricture ◽

Pediatric Anesthesia ◽

Rectal Biopsy ◽

Tertiary Hospital ◽

Single Stage ◽

Short Segment ◽

Pull Through

Background: The surgical management of Hirschsprung's Disease (HD) includes so many procedures. TransanalEndorectal Pull through (TEP) represents the latest development in the concept of the minimally invasive surgery for HD. The purpose of this study was to evaluate the out come of the procedure in neonates. Methods: This retrospective study was carried out in a tertiary pediatric hospital during the period from January 2007 to December 2012 (5 years). The study included neonates of both sex, weight more than 2 kg, who were clinically suspected HD, radiologically transition zone at rectosigmoid and midsigmoid region and rectal biopsy proven HD and no evidence of sepsis or entrocolitis. Short segment HD with associated anomalies, and operated cases with less than 6 months' or irregular follow up were excluded. Results: During study period, single-stage transanalendorectal pull through (TEP) operation was done for short segment HD in 63 neonates, 9 patients were excluded from the study for irregular follow up. The mean operative time, mean blood loss, postoperative hospital stay, follow up period were 113 minutes, 20ml, 6.8 days and 19.6 months respectively. Transverse colostomy was needed in 6 patients for anastomotic leakage, thereafter developed anastomotic stricture, managed with regular anastomotic dilatation. Conclusion: Advancement in pediatric anesthesia, improvement of pediatric surgical expertise, perioperative management and nursing care has made single-stage primary transanalendorectal pull-through a feasible and safe surgical procedure for the treatment of short segment Hirschsprung's disease in neonate. Journal of Surgical Sciences (2014) Vol. 18 (2) : 45-50

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Diagnosis of Hirschsprung's Disease: Accuracy of Barium Enema Findings

Journal of the Korean Radiological Society ◽

10.3348/jkrs.1997.36.4.631 ◽

1997 ◽

Vol 36 (4) ◽

pp. 631

Author(s):

Sue Yun Yu ◽

Gye Yeon Lim ◽

Ji Yeong Yun ◽

Seong Tae Hahn ◽

Hak Hee Kim ◽

...

Keyword(s):

Barium Enema ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease

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The follow-up of the robotic-assisted Soave procedure for Hirschsprung’s disease in children

Journal of Robotic Surgery ◽

10.1007/s11701-021-01238-z ◽

2021 ◽

Author(s):

Tran Anh Quynh ◽

Pham Duy Hien ◽

Le Quang Du ◽

Le Hoang Long ◽

Nguyen Thi Ngoc Tran ◽

...

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Pediatric Patients ◽

Three Dimensional ◽

Dentate Line ◽

Robotic Arms ◽

Robotic Assisted ◽

The Mean ◽

Pull Through

AbstractRobotic surgery offers three-dimensional visualization and precision of movement that could be of great value to gastrointestinal surgeons. There were many previous reports on robotic technology in performing Soave colonic resection and pull-through for Hirschsprung’s disease in children. This study described the follow-up of the Robotic-assisted Soave procedure for Hirschsprung’s disease in children. Robotic-assisted endorectal pull-through was performed using three robotic arms and an additional 5-mm trocar. The ganglionic and aganglionic segments were initially identified by seromuscular biopsies. The rest of the procedure was carried out according to the Soave procedure. We left a short rectal seromuscular sleeve of 1.5–2 cm above the dentate line. From December 2014 to December 2017, 55 pediatric patients were operated on. Age ranged from 6 months to 10 years old (median = 24.5 months). The aganglionic segment was located in the rectum (n = 38), the sigmoid colon (n = 13), and the left colon (n = 4). The mean total operative time was 93.2 ± 35 min (ranging from 80 to 180 min). Minimal blood was lost during the surgery. During the follow-up period, 41 patients (74.6%) had 1–2 defecations per day, 12 patients (21.8%) had 3–4 defecations per day, and 2 patients (3.6%) had more than 4 defecations per day. Fecal incontinence, enterocolitis, and mild soiling occurred in three (5.4%), four (7.3%), and two pediatric patients, respectively. Robotic-assisted Soave procedure for Hirschsprung’s disease in children is a safe and effective technique. However, a skilled robotic surgical team and procedural modifications are needed.

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G324 Expression of neurones and neuronal precursors in the transition zone of short segment hirschsprung’s disease

10.1136/archdischild-2020-rcpch.280 ◽

2020 ◽

Author(s):

J Doherty ◽

S Chhabra ◽

M Smith ◽

D Edgar ◽

S Almond ◽

...

Keyword(s):

Transition Zone ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Short Segment ◽

Neuronal Precursors

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HIRSCHSPRUNG’S DISEASE

The Professional Medical Journal ◽

10.29309/tpmj/2010.17.02.2349 ◽

2010 ◽

Vol 17 (02) ◽

pp. 223-231

Author(s):

MUHAMMAD ZAFAR IQBAL ◽

Hammad Azam ◽

MUHAMMAD JAHANGIR ◽

Muhammad Tahir ◽

MUHAMMAD ANWAR ◽

...

Keyword(s):

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Case Series ◽

Abdominal Distension ◽

Functional Results ◽

International Studies ◽

Stool Incontinence ◽

Case Series Study ◽

Duhamel Procedure ◽

One Year

Introduction: Hirschsprung’s Disease is one of the most common congenital anomalies that Pediatric Surgeons manage. In spite of the various modifications of pull through procedures available, the long term functional results are less than ideal. However, Modified Duhamel Procedure is one which has relatively good functional results and that is the reason we have selected this procedure for Hirschsprung’s disease in Sheikh Zayed Hospital, Rahim Yar Khan. Objectives: To evaluate the outcome of patients after Modified Duhamel Procedure for Hirschsprung’s Disease. Design: This is case series study. Settings: Department of Pediatric Surgery Sheikh Zayed Medical College/ Hospital Rahim Yar Khan. Period: Seventeen cases were studied over a period of two year i.e. from November 2006 to December, 2008. Material and Method: Seventeen (17) cases from both sexes were operated for Hirschsprung’s Disease over a period of one year and Modified Duhamel Procedure was adopted for all these cases. All patients were diagnosed cases of rectosigmoidal aganglionosis and follow up was done over a period of one year according to a comprehensive Proforma. Four parameters like normal stool evacuation, abdominal distension, soiling and stool incontinence were followed and then the results were compared with other national and international studies. Results: Fever 19.4% (n=04), wound infection 19.4% (n=04), vomiting 9.52% (n=02), abdominal distension 4.76% (n=01), and bleeding per rectum 4.76% (n=01), were the immediate post operative complications. Fourteen patients (82.35%), used to pass stool once daily. Abdominal distension was observed occasionally in six patients (28.57%). Soiling was seen in five patients (29.41%). Out of Seventeen, stool incontinence was seen in only three patients (17.64%). Conclusion: Modified Duhamel Procedure with the help of linear cutter stapler device is quite safe, easy and less time consuming. Infact Modified Duhamel is a procedure of choice for Hirschsprung’s Disease.

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