Hirschsprung’s Disease and Intestinal Neuronal Dysplasias

AbstractThis study describes functional and health-related quality of life (HRQoL) outcomes in patients with Hirschsprung’s disease (HSCR) with associated learning disability or neurodevelopmental delay (LD), completing a core outcome set for HSCR. This was a cross-sectional study from a tertiary pediatric surgery center. Patients treated between 1977 and 2013 were prospectively contacted to complete an outcomes survey. Children under 12 and older patients with LD were assisted to complete these by a proxy. Bowel and urologic function were assessed (Rintala’s BFS and modified DanPSS) along with HRQoL (PedsQL/GIQLI/SF-36). Thirty-two patients with LD were compared to 186 patients with normal cognition. Patients with LD had 76% survival over the follow-up period, compared to 99% in the remainder of the cohort. Poor functional outcomes were common in the patients with LD, considerably higher than cognitively normal patients: with weekly issues withholding stool, soiling and fecal accidents in over half of patients surveyed (44–60%), and urinary incontinence in 46%. Use of permanent stoma was significantly higher (22% vs. 4%; p = 0.001). HRQoL was worse in domains of physical functioning in adults and children but not for social or emotional domains in adults. Subgroup analysis of patients with Down syndrome suggested similar functional results but better QoL. Multivariate analysis demonstrated a dramatically higher incidence of poor continence outcomes in patients with LD (adjusted OR 9.6 [4.0–23]).Conclusions: We provide LD-specific outcomes showing inferior function but similar HRQoL to other patients with HSCR, this is much needed in the counselling of families of these children. What is Known:• Hirschsprung’s disease is commonly associated with syndromes or other anomalies with resultant cognitive impairments.• The outcomes for these patients specifically have been poorly described in the literature. What is New:• Objective functional and quality of life surveys demonstrate significant differences from patients without cognitive impairment.• Patients with learning disability Patients with associated LD were almost ten times more likely to have an associated poor functional outcome, with very little impact on proxy-reported quality of life.

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Long-term outcomes in children with Hirschsprung’s disease and transition zone bowel pull-through: impact of surgical techniques and role for conservative approach

Pediatric Surgery International ◽

10.1007/s00383-021-04974-3 ◽

2021 ◽

Author(s):

Gowri Shankar ◽

J. G. Deepak ◽

Vinay Jadhav ◽

K. Venkatesh ◽

Usha Kini ◽

...

Keyword(s):

Transition Zone ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Surgical Techniques ◽

Conservative Approach ◽

Long Term Outcomes ◽

Pull Through

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Multiple 'omics'-analysis reveals the role of prostaglandin E2 in Hirschsprung's disease

Free Radical Biology and Medicine ◽

10.1016/j.freeradbiomed.2020.12.456 ◽

2021 ◽

Vol 164 ◽

pp. 390-398

Author(s):

Weibing Tang ◽

Minjian Chen ◽

Xuejiang Guo ◽

Kun Zhou ◽

Zechao Wen ◽

...

Keyword(s):

Prostaglandin E2 ◽

Hirschsprung's Disease ◽

Hirschsprung’S Disease ◽

Omics Analysis

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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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