Analysis of problems, complications, avoidance and management with transanal pull-through for Hirschsprung disease

Abstract Background Transanal endorectal pull-through (TEPT) is considered the most preferable treatment method for Hirschsprung disease (HSCR) since it is less invasive and has fewer morbidities than transabdominal pull-through. Here, functional outcomes in short-segment HSCR patients after TEPT were assessed and associated with the prognostic factors. Methods Krickenbeck classification was used to assess the functional outcomes in patients with HSCR after TEPT surgery at our institution from 2012 to 2020. Results Fifty patients were involved in this study. Voluntary bowel movement (VBM) was achieved in 82% of subjects. Nine (18%) subjects had soiling grade 1, while two (4%) and two (4%) patients suffered constipation that was manageable with diet and laxative agents, respectively. Patients who underwent TEPT at ≥ 4 years old tended to have soiling more than patients who underwent TEPT at < 4 years old (OR = 16.47 [95% CI 0.9–301.61]; p = 0.06), whereas patients with post-operative complications had 10.5-fold higher risk for constipation than patients without post-operative complications (p = 0.037; 95% CI 1.15–95.92). Multivariate analysis showed male sex was significantly associated with VBM (OR = 9.25 [95% CI 1.34–63.77]; p = 0.024), while post-operative complications were strongly correlated with constipation (OR = 10 [95% CI 1.09–91.44]; p = 0.04). Conclusions The functional outcomes of HSCR patients after TEPT in our institution are considered relatively good. Moreover, the VBM, soiling, and constipation risk after TEPT might be affected by sex, age at TEPT performed, and post-operative complications, respectively, while the age at TEPT performed might not be associated with functional outcomes. Further multicenter studies with a larger sample size are necessary to clarify and confirm our findings.

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Perioperative and long-term functional outcomes of neonatal versus delayed primary endorectal pull-through for children with Hirschsprung disease: A Pediatric Colorectal and Pelvic Learning Consortium Study

Journal of Pediatric Surgery ◽

10.1016/j.jpedsurg.2021.04.024 ◽

2021 ◽

Author(s):

Zachary J. Kastenberg ◽

Mark A. Taylor ◽

Megan M. Durham ◽

Casey M. Calkins ◽

Rebecca M. Rentea ◽

...

Keyword(s):

Functional Outcomes ◽

Hirschsprung Disease ◽

Pull Through

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Altered Cytoskeleton in Smooth Muscle of Aganglionic Bowel

Archives of Pathology & Laboratory Medicine ◽

10.5858/2002-126-0692-acismo ◽

2002 ◽

Vol 126 (6) ◽

pp. 692-696

Author(s):

Laszlo Nemeth ◽

Udo Rolle ◽

Prem Puri

Keyword(s):

Smooth Muscle ◽

Smooth Muscle Cells ◽

Laser Scanning ◽

Hirschsprung Disease ◽

Muscle Cells ◽

Cytoskeletal Proteins ◽

Laser Scanning Microscopy ◽

Confocal Laser ◽

Normal Bowel ◽

Pull Through

Abstract Context.—Intestinal motility is under the control of smooth muscle cells, enteric plexus, and hormonal factors. In Hirschsprung disease (HD), the aganglionic colon remains spastic or tonically enhanced and unable to relax. The smooth muscle cell's cytoskeleton consists of proteins or structures whose primary function is to link or connect protein filaments to each other or to the anchoring sites. Dystrophin is a subsarcolemmal protein with a double adhesion property, one between the membrane elements and the contractile filaments of the cytoskeleton and the other between the cytoskeletal proteins and the extracellular matrix. Desmin and vinculin are functionally related proteins that are present in the membrane-associated dense bodies in the sarcolemma of the smooth muscle cells. Objective.—To examine the distribution of the cytoskeletal proteins in the smooth muscle of the aganglionic bowel. Design.—Bowel specimens from ganglionic and aganglionic sections of the colon were collected at the time of pull-through surgery from 8 patients with HD. Colon specimens collected from 4 patients at the time of bladder augmentation acted as controls. Anti-dystrophin, anti-desmin, and anti-vinculin antibodies were used for fluorescein immunostaining using confocal laser scanning microscopy. Results.—Moderate to strong dystrophin immunoreactivity was observed at the periphery of smooth muscle fibers in normal bowel and ganglionic bowel from patients with HD, whereas dystrophin immunoreactivity was either absent or weak in the smooth muscle of aganglionic colon. Moderate to strong cytoplasmic immunostaining for vinculin and desmin was seen in the smooth muscle of normal bowel and ganglionic bowel from patients with HD, whereas vinculin and desmin staining in the aganglionic colon was absent or weak. Conclusion.—This study demonstrates that the cytoskeletal proteins are abundant in the smooth muscle of normal bowel, but are absent or markedly reduced in the aganglionic bowel of HD. As cytoskeletal proteins are required for the coordinated contraction of muscle cells, their absence may be responsible for the motility dysfunction in the aganglionic segment.

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Comparison of the Results of Transanal Endorectal Pull-through and Martin-modified Duhamel Operations in Hirschsprung Disease

Journal of Ankara University Faculty of Medicine ◽

10.4274/atfm.galenos.2019.02418 ◽

2020 ◽

Vol 72 (3) ◽

pp. 349-355

Author(s):

Yasemin Dere Günal ◽

Mustafa Kemal Aslan ◽

Ayşe Karaman ◽

İbrahim Karaman ◽

Derya Erdoğan ◽

...

Keyword(s):

Hirschsprung Disease ◽

Pull Through ◽

Comparison Of The Results

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Results of the Surgical Treatment in Children with Hirschprung Disease: A 12-Year Experience

Turkish Association of Pediatric Surgeons ◽

10.5222/jtaps.2021.49092 ◽

2021 ◽

Author(s):

Ceren Cesur ◽

Ayşenur Celayir

Keyword(s):

Surgical Treatment ◽

Complication Rate ◽

Operative Procedure ◽

Surgical Techniques ◽

Hirschsprung Disease ◽

Rectal Biopsy ◽

X Rays ◽

Surgical Methods ◽

Pull Through ◽

Subacute Intestinal Obstruction

Objective: In the treatment of Hirschsprung’s disease, many surgical techniques have been developed and modified including laparotomy, resection of the affected colon segment, and pull-through of the functional bowel segment to the anus. Conventional surgical methods have many early and late postoperative complications, albeit at a reduced rate today. In this study, it was aimed to compare the treatment results of children with Hirschsprung Disease who were operated with single or two-stage techniques in our department. Method: Hospital records of the patients operated for Hirschsprung disease were retrospectively evaluated. Demografic characteristics, findings of physical and rectal examinations, X-rays findings, rectal biopsy results, surgical treatment methods and outcomes were analyzed. Results: Fifty-two children including 41-male (78.8%) and 11-female (21.2%) patients with age ranging from 1-day to 5-year and mean age of 6.8-day in neonates and 6.3-month in others were enrolled in the study. All patients presented with acute or subacute intestinal obstruction. Levels were observed on supine abdominal radiographs in all; transition zone and retention of opaque material were observed on opaque enema or retention radiographs.In patients whose rectal biopsies revealed aganglionic megacolon two staged procedures were performed including initial colostomy followed by definitive procedure of Duhamel with stapler in 30, and Soave in 12, and Swenson in 1 patient. Nine patients were operated with one-stage endorectal pull-through technique. The complication rate in 43 patients with Hirschsprung disease managed by two staged operative procedure was (78.8%), and it was 11.1% in 9 cases that underwent transanal endorectal pullthrough procedure. It was learned that effective anal dilatation was not performed in 22-patients who had postoperative enterocolitis attacks. Conclusion: In addition to the low complication rate, the morbidity rates in the transanal endorectal pull-through method, which achieved successful results with only one procedure, were very low compared to multiple-stage operations.

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Postoperative enterocolitis assessment using two different cut-off values in the HAEC score in Hirschsprung patients undergoing Duhamel and Soave pull-through

BMC Pediatrics ◽

10.1186/s12887-020-02360-x ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Gunadi ◽

Afnandito Valeno Risky Sukarelawanto ◽

Azmi Ritana ◽

Naisya Balela ◽

Wayan Julita Krisnanti Putri ◽

...

Keyword(s):

Risk Factors ◽

Educational Level ◽

Medical Records ◽

Hirschsprung Disease ◽

Scoring Method ◽

Multicenter Studies ◽

Mother’S Age ◽

Pull Through ◽

Definitive Surgery ◽

Larger Sample

Abstract Background Hirschsprung-associated enterocolitis (HAEC) is the most severe and potentially lethal complication of Hirschsprung disease (HSCR) which might occur following definitive surgery. Our objectives were: 1) to compare the incidence of HAEC after Duhamel and Soave procedures using different cut-off values of the HAEC scoring method; and 2) to associate them with the risk factors, including sex, aganglionosis type, mothers’ age at childbirth, gestational age, and mothers’ educational level. Methods Medical records of patients with HSCR who underwent Soave and Duhamel procedures in our institution, Indonesia (January 2012 – December 2016) were reviewed retrospectively. Two cut-off values of the HAEC scoring system (i.e., ≥10 and ≥ 4) were utilized. Results Eighty-three patients with HSCR were recruited in this study (Soave: 37 males and 7 females vs. Duhamel: 28 males and 11 females; p = 0.18). The incidence of HAEC after surgery was 14/83 (16.9%) and 38/83 (45.8%) for cut-off values of ≥10 and ≥ 4, respectively (p = 0.00012), and tended to have an association with sex (p = 0.09). Although it was not statistically significant (p = 0.07), the frequency of HAEC after Soave procedure tended to be higher in patients with their mother’s age of ≤35 years at childbirth than those with their mother’s age of > 35 years (OR = 7.9; 95% CI = 0.9–72.1). Multivariate analysis indicated none of the risk factors were associated with the frequency of HAEC after definitive surgery. Conclusions The lower cut-off value of ≥4 might increase the possibility to diagnose HAEC, particularly the mild cases. The incidence of HAEC after definitive surgery was not associated with any risk factors in our cohort patients. Further multicenter studies with a larger sample size are necessary to confirm our findings.

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Quality of life of patients with Hirschsprung disease after Duhamel and Soave pull-through procedures: A mixed-methods sequential explanatory cohort study

Annals of Medicine and Surgery ◽

10.1016/j.amsu.2020.05.043 ◽

2020 ◽

Vol 56 ◽

pp. 34-37

Author(s):

Michelle Raj Saysoo ◽

Fatwa Sari Tetra Dewi ◽

Gunadi

Keyword(s):

Quality Of Life ◽

Cohort Study ◽

Mixed Methods ◽

Hirschsprung Disease ◽

Sequential Explanatory ◽

Pull Through

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Comparison of Hirschsprung Disease Characteristics between Those with a History of Postoperative Enterocolitis and Those without: Results from the Pediatric Colorectal and Pelvic Learning Consortium

European Journal of Pediatric Surgery ◽

10.1055/s-0040-1716876 ◽

2020 ◽

Author(s):

Mark A. Taylor ◽

Brian T. Bucher ◽

Ron W. Reeder ◽

Jeffrey R. Avansino ◽

Megan Durham ◽

...

Keyword(s):

Risk Factors ◽

Transition Zone ◽

Private Insurance ◽

Univariate Analysis ◽

Hirschsprung Disease ◽

Clinical Information ◽

Retrospective Case ◽

Disease Characteristics ◽

History Of ◽

Pull Through

Abstract Introduction The current understanding of Hirschsprung-associated enterocolitis (HAEC) is based mainly on single-center, retrospective studies. The aims of this study are to determine risk factors for postoperative HAEC using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) database. Materials and Methods We performed a multicenter, retrospective, case–control study of children with Hirschsprung disease (HD) who had undergone a pull-through procedure and were evaluated at a PCPLC member site between February 2017 and March 2020. The cohort with a history of postoperative HAEC was compared with that without postoperative episodes of HAEC to determine relevant associations with postoperative HAEC. Results One-hundred forty of 299 (46.8%) patients enrolled had a history of postoperative HAEC. Patients with a rectosigmoid transition zone had a lower association with postoperative HAEC as compared with those with a more proximal transition zone (odds ratio [OR]: 0.46, 95% confidence interval [CI]: 0.26, 0.84, p < 0.01). Private insurance was protective against postoperative HAEC on univariate analysis (OR: 0.62, 95% CI: 0.38, 0.99, p = 0.047), but not on multivariate analysis (OR: 0.62, 95% CI: 0.37, 1.04, p = 0.07). Preoperative HAEC was not associated with the development of postoperative HAEC. Conclusion Patients with a rectosigmoid transition zone have less postoperative HAEC compared with patients with a more proximal transition zone. Multi-institutional collection of clinical information in patients with HD may allow for the identification of additional risk factors for HAEC and afford the opportunity to improve care.

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