scholarly journals Mo1736 INITIAL EXPERIENCE WITH PER-RECTAL ENDOSCOPIC MYOTOMY (PREM) FOR HIRSCHSPRUNG’S DISEASE: MEDIUM AND LONG-TERM OUTCOMES OF THE FIRST CASE SERIES OF A NOVEL THIRD SPACE ENDOSCOPIC PROCEDURE

2020 ◽  
Vol 91 (6) ◽  
pp. AB474
Author(s):  
Amol Bapaye ◽  
Parag Dashatwar ◽  
Siddharth Dharamsi ◽  
Sheetal V. Biradar ◽  
Vishnu Biradar ◽  
...  
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Third Space ◽  
Case Series ◽  
Endoscopic Procedure ◽  
Initial Experience ◽  
Long Term Outcomes ◽  
First Case ◽  
Endoscopic Myotomy

A novel third space endoscopic procedure, per-rectal endoscopic myotomy, for Hirschsprung’s disease: Medium and long-term outcomes

Endoscopy ◽  
2020 ◽  
Author(s):  
Amol Bapaye ◽  
Parag Dashatwar ◽  
Vishnu Biradar ◽  
Shital Biradar ◽  
Rajendra Pujari
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Third Space ◽  
Stool Frequency ◽  
Segment Length ◽  
Anal Stenosis ◽  
Current Standard ◽  
Aganglionic Segment ◽  
Endoscopic Myotomy

Introduction Hirschsprung’s disease (HSCR) is congenital aganglionosis affecting hindgut and presents with constipation. Surgical pull-through is current standard but has morbidity. Per-rectal endoscopic myotomy (PREM) is a novel third space endoscopy technique for treating SS-HSCR. Methods Retrospective series of SS-HSCR patients diagnosed on history, contrast enema, rectal biopsies, anorectal manometry and treated by PREM. Aganglionic segment mapped pre-PREM. PREM performed using third space endoscopy principles. Objective – to compare stool frequency and unit laxative (UL) usage pre and post-PREM. Results N = 9; duration 4-years. Mean age – 7.5 (± 5.2) years; 7 males. Mean aganglionic segment length – 6.3 (± 4.4) cm. Mean procedure time – 96.1 (±37.9) minutes. Mean LOS – 2.5 (±0.7) days. Median follow-up –17 months (9 – 58). Stool frequency – pre – 1/4.4 (±1.5) vs. post – 1/1.2 (±0.4) days (p = 0.0004). Mean UL usage – pre – 5.4 (±4.9) vs. post – 0.4 (±0.7) units (p = 0.0002). No laxatives in 6 (66%). Single AE (anal stenosis) – dilatation. Conclusions PREM is a safe and effective minimally invasive procedure for SS-HSCR and provides long-term response.

Long-Term Outcomes and Quality of Life in Patients after Soave Pull-Through Operation for Hirschsprung's Disease: An Observational Retrospective Study

2017 ◽  
Vol 28 (05) ◽  
pp. 445-454 ◽  
Author(s):  
Tania Mahler ◽  
Martine Dassonville ◽  
Dinh Truong ◽  
Annie Robert ◽  
Philippe Goyens ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Fecal Incontinence ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Bowel Function ◽  
Long Term Outcomes ◽  
Neurologic Impairment ◽  
Pull Through

Introduction Patients after pull-through operation for Hirschsprung's disease (HD) are at high risk of defecation disorders. This study aimed at investigating their long-term outcomes and quality of life (QoL) in comparison with controls. Patients and Methods Patients older than 5 years operated on for HD were interviewed to complete detailed questionnaires on bowel function. Patients without neurologic impairment were enrolled in a QoL survey to compare with controls matched for sex and age and selected randomly from the general population using sampling set in a ratio of four controls to one case of HD. Results In total, 53 operated patients were enrolled. Mean age of the patients was 16 ± 8 years, with 68% boys. Rectosigmoid aganglionosis was the most seen form of HD in 38 (72%) cases. Open Soave was performed in 40 (75.5%) cases, and minimally invasive surgery Soave (MIS Soave) in 13 (24.5%) cases. At investigation, prevalence of fecal incontinence and constipation were 22.6 and 13.2%, respectively. Regarding QoL survey, 45 patients and 180 controls were enrolled, excluding 8 patients with neurologic impairment. Thirty-seven (82.2%) patients were classified as having a good QoL (score ≥ 9 points); whereas six had a fair QoL (5–8 points) and two had a poor QoL (< 5 points). QoL score in the cases and the controls were 10.2 ± 2.5 and 11.9 ± 0.4 points, respectively. Long aganglionosis form of HD was significantly associated with a low QoL (score < 8 points), adjusted odds ratio = 9, 95% confidence interval [1.3; 64.1] (p < 0.05). In subscales analyses, the prevalence of each dimension including fecal continence, school absenteeism, unhappiness or anxiety, food restriction, and peer rejection was significantly higher in operated patients than in controls (p <0.001). Conclusion Although the QoL of patients operated on for HD in general was with good outcomes, fecal incontinence and constipation still are problematic issues and challenges in a high percentage of patients. Therefore, a long-term and multidisciplinary follow-up is essentially required for these patients.

scholarly journals Early and long-term complications following transanal pull through Soave technique in infants with Hirschsprung's diseaseric infants with Hirschsprung's disease

2019 ◽  
Author(s):  
Manoochehr Ghorbanpour ◽  
Mohammad Ali Seyfrabie ◽  
Babak Yousefi
Keyword(s):  
Intestinal Obstruction ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Statistical Tests ◽  
Case Series ◽  
P Value ◽  
Short Term ◽  
Case Series Study ◽  
The Mean

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.

Long-term outcomes of Hirschsprung's disease

2012 ◽  
Vol 21 (4) ◽  
pp. 336-343 ◽  
Author(s):  
Risto J. Rintala ◽  
Mikko P. Pakarinen
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Long Term Outcomes

Topical dihydrotestosterone to treat micropenis secondary to partial androgen insensitivity syndrome (PAIS) before, during, and after puberty – a case series

2016 ◽  
Vol 29 (2) ◽  
Author(s):  
David Becker ◽  
Lisa M. Wain ◽  
Yih Harng Chong ◽  
Sonal J. Gosai ◽  
Nina K. Henderson ◽  
...  
Keyword(s):  
Adverse Effects ◽  
Case Series ◽  
Androgen Insensitivity Syndrome ◽  
High Dose ◽  
Long Term Outcomes ◽  
Penile Length ◽  
Androgen Insensitivity ◽  
First Case ◽  
Partial Androgen Insensitivity Syndrome

AbstractX-linked partial androgen insensitivity syndrome (PAIS) causes under-virilization at all stages of development. In two thirds of males, this results in micropenis. Dihydrotestosterone (DHT) is a potent androgen that is critical for male genital development, which when applied topically, has been shown to increase penile length with micropenis of varying etiologies. We present the first case series using topical DHT gel to treat micropenis in 46,XY males with PAIS, before, during, and after puberty.Three related 46,XY males with confirmed p.L712F androgen receptor mutations exhibited varying degrees of micropenis post-surgical correction. They were of pre-pubertal, peri-pubertal and adult ages, respectively. Following baseline clinical and laboratory assessments all completed a 4-month course of daily DHT gel 2.5% (androstanolone) topically to penis (0.3 mg/kg body weight), with monitoring for adverse effects. Primary outcome was change in stretched penile length (SPL) following treatment.Mixed results were obtained following topical DHT therapy. In the pre- and peri- pubertal patients, SPL changed from 2.5 cm to 3.5 cm (+40%), and 3.5 cm to 5.7 cm (+63%), respectively. In the adult patient with 1 year of prior high-dose weekly testosterone therapy, no additional change in SPL was seen. No adverse effects of topical DHT were reported or observed throughout the 4 months of treatment.Topical DHT treatment appears to be a safe and well-tolerated method of virilising micropenis both prior to and during puberty in children with PAIS. Questions remain about long-term outcomes into adulthood, and efficacy in adults with prior lengthy exposure to high-dose testosterone.

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