Background: The one-stage pull-through procedures for Hirschsprung's disease (HD) have become popular because it is well accepted by surgeons and mothers with no visible scar and a short hospital stay. It represents the latest development in the concept of a minimally invasive surgery for HD. We introduce a new method of transanal one-stage pull-through for Hirschsprung's disease, different from the transanal Soave procedure.
Methods: One hundred and thirty-four patients aged 9 days to 5 years underwent a transanal one-stage pull-through procedure. The diagnosis was definite by barium enema or rectal biopsies preoperatively. The patients were anesthetized and placed in the lithotomy position. A urinary catheter was optional. Giving anorectal dilatations for half a minute, a pull-through of the rectum above the peritoneal reflection and into the intussusception was performed. Fine silk suturing was performed circumferentially at the level of that point which was used for traction for the distal end. Another circumferential suture was performed parallel 0.5 cm distance above the original one and used for traction for the proximal intestines. The full-thickness rectal wall was truncated between the above two circumferential sutures with cautery. The proximal intestines were pulled down and the mesenteric vessels were dissected with ligation until normal intestines were accessed; the presence of ganglion cells was determined by intraoperative rapid frozen section. The distal end was dissected anteriorly 2.5- 3.5 cm above the dentate line. The posterior rectal wall was split longitudinally and dissected to a point 0.5- 1.0 cm above the dentate line. The segment of the lesion was resected. The length of bowel resected ranged from 12 to 50 cm (median 16.5 cm). An oblique anastomosis was made.
Results: The mean operating time was 70 minutes. Postoperative rectal dilation was not required. The patient tolerated feeding on the first postoperative day. Eighty-eight patients were followed-up. All these patients had 2- 3 bowel movements per day at postoperative month 1. They were discharged within 4.5 days after the operation. Six patients presented with complications. All recovered by reoperation or conservative treatments.
Conclusion: The modification of the transanal Swenson pull-through procedure for Hirschsprung's Disease is an easy adaptation to a well-established technique and has a reasonable result.
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Hum Mol Genet
January 2025
Department of Pediatric Surgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, No. 1095 Jiefang Avenue, Qiaokou District, Wuhan, Hubei 430030, China.
Hirschsprung's disease (HSCR) is a congenital enteric neuropathic disorder characterized by high heritability (>80%) and polygenic inheritance (>20 genes). The previous genome-wide association studies (GWAS) identified several common variants associated with HSCR and demonstrated increased predictive performance for HSCR risk in Europeans using a genetic risk score, there remains a notable gap in knowledge regarding Chinese populations. We conducted whole exome sequencing in a HSCR case cohort in Chinese.
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December 2024
Comprehensive Colorectal Center, Department of Surgery, Children's Mercy Kansas City, Kansas City, USA.
Long-segment Hirschsprung disease (HSCR) presents significant challenges in surgical management, often requiring extensive bowel mobilization and creative techniques to achieve tension-free anastomosis. Colonic derotation offers a viable solution for preserving bowel length and maintaining the ileocecal valve, which is crucial for postoperative bowel function. The procedure involves extensive colonic mobilization and strategic vascular divisions of the right and middle colic vessels while preserving the ileocolic and marginal arteries, followed by a 180° counterclockwise rotation of the colon around the ileocolic vascular axis.
View Article and Find Full Text PDFNeurogastroenterol Motil
January 2025
Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Introduction: Colonic manometry (CM) is a diagnostic procedure used to evaluate pediatric patients with refractory constipation, fecal incontinence, Hirschsprung disease, and pediatric intestinal pseudo-obstruction. Pan-colonic high-amplitude propagated contractions (HAPCs), measured by CM, reflect an intact neuromuscular function of the colon. Current guidelines recommend starting CM with fasting recording for 1-2 h, but no prior evaluation has determined the diagnostic yield of the fasting phase.
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January 2025
The Department of Paediatric Surgery, The Royal Hospital for Children Glasgow, Glasgow, United Kingdom.
A primary pull-through for Hirschsprung's disease (HD) requires confirmation of normal ganglionic bowel by intraoperative biopsies to determine the level of resection. Despite this, aganglionic bowel that is not fully resected (so-called "transition zone pull-throughs") is reported in 15%-19% of patients. We hypothesize that this may result from insufficient biopsies sent for intraoperative diagnosis.
View Article and Find Full Text PDFBiomedicines
December 2024
Diagnostic and Interventional Neuroradiology Unit, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
The glycosylphosphatidylinositol (GPI) is a glycol-lipid that anchors several proteins to the cell surface. The GPI-anchor pathway is crucial for the correct function of proteins involved in cell function, and it is fundamental in early neurogenesis and neural development. The PIG gene family is a group of genes involved in this pathway with six genes identified so far, and defects in these genes are associated with a rare inborn metabolic disorder manifesting with a spectrum of clinical phenotypes in newborns and children.
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