ANALYSIS OF LATE DIAGNOSIS AND TREATMENT OF HIRSCHSPRUNG'S DISEASE IN CHILDREN OVER 5 YEARS OLD

2021 ◽  
pp. 56-61
Author(s):  
O. A. Govorukhina ◽  
◽  
N. S. Prokopenya ◽  
S. A. Sukharev ◽  
O. A. Pataleta ◽  
...  
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Surgical Techniques ◽  
Functional Results ◽  
Colon Resection ◽  
Intestinal Wall ◽  
Late Diagnosis ◽  
Defect Correction ◽  
Surgical Interventions ◽  
Erroneous Diagnosis

Frequent complications of Hirschsprung's disease and other disorders of intestinal motility are problems of late or erroneous diagnosis, and sometimes to a greater extent than complications of surgical techniques when performing surgical interventions. However, there are still cases of late diagnosis of the disease, which inevitably leads to technical difficulties during surgical treatment and an increase in the size of the colon resection. Secondary changes in the intestinal wall worsen the functional results of defect correction.

scholarly journals THE VALUE OF INTRAOPERATIVE EXPRESS BIOPSY IN DETERMINING THE LEVEL OF COLON RESECTION IN THE SURGICAL TREATMENT OF HIRSCHSPRUNG'S DISEASE

2021 ◽  
pp. 42-46
Author(s):  
O.A. Govorukhina ◽  
Keyword(s):  
Surgical Treatment ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Bowel Resection ◽  
Radical Surgery ◽  
Transitional Zone ◽  
Colon Resection ◽  
Intestinal Wall ◽  
Aganglionic Segment

Introduction. Hirschsprung's disease is a congenital malformation characterized by the absence of intramural ganglia in the intestinal wall. The goals of the surgical treatment of HD are to remove the aganglionic segment of the intestine and restore the patency of the gastrointestinal tract, bringing down the normally innervated intestine to the anus while maintaining the function of the sphincters. An incorrect determination of the length of the affected segment of the colon can lead to non-radical surgical treatment with a persistent aganglionic zone. The use of an intraoperative express biopsy to determine the resection area during radical surgery will help to solve this problem. Purpose: determination of the level of agangliosis of the colon in the surgical treatment of Hirschsprung's disease. Methods: From 2010 to 2019, 49 patients aged 3 weeks to 16 years underwent an intraoperative express biopsy to determine the area of bowel resection. The use of intraoperative express biopsy made it possible to objectively and reliably determine the area of bowel resection, to reduce the number of complications associated with errors in resection of the aganglionic segment. Conclusion. When performing radical surgery for Hirschsprung's disease, an intraoperative express biopsy is required to determine the level of coloanal anastomosis in order to avoid errors in determining the aganglionic region and the transitional zone of the intestine.

scholarly journals Outcomes in Hirschsprung’s disease with coexisting learning disability

2021 ◽  
Author(s):  
Joseph R. Davidson ◽  
Kristiina Kyrklund ◽  
Simon Eaton ◽  
Mikko P. Pakarinen ◽  
David Thompson ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Learning Disability ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Core Outcome Set ◽  
Functional Results ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Related Quality

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.

Megacólon Agangliônico Congênito: relato de caso de diagnóstico tardio de Doença de Hirschsprung / Congenital Aganglionic Megacolon: case report of a late diagnosis of Hirschsprung's Disease

2021 ◽  
Vol 7 (7) ◽  
pp. 73996-73999
Author(s):  
Helen Brambila Jorge Pareja ◽  
Pedro Augusto de Assis Goes ◽  
Eraldo de Azevedo Coelho Junior ◽  
Beny Goulart Dias De Castro ◽  
Alessandro Luiz Gonçalves ◽  
...  
Keyword(s):  
Case Report ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Late Diagnosis

scholarly journals HIRSCHSPRUNG’S DISEASE

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. 

Late diagnosis of Hirschsprung’s disease

2016 ◽  
Vol 3 (4) ◽  
pp. 220
Author(s):  
Suleiman Lawal ◽  
PhilipO Ibinaiye ◽  
SefiyaA Olarinoye-Akorede ◽  
JosephB Igashi ◽  
AhmedH Umdagas
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Late Diagnosis

Surgical interventions for traumatic lesions of the brachial plexus: a retrospective study of 134 cases

2005 ◽  
Vol 103 (4) ◽  
pp. 614-621 ◽  
Author(s):  
Julia A. Kandenwein ◽  
Thomas Kretschmer ◽  
Martin Engelhardt ◽  
Hans-Peter Richter ◽  
Gregor Antoniadis
Keyword(s):  
Brachial Plexus ◽  
Surgical Techniques ◽  
Functional Results ◽  
Content Type ◽  
Surgical Interventions ◽  
Louisiana State University ◽  
Surgical Measures ◽  
Grade 3 ◽  
Nerve Surgery ◽  
Fine Print

Object. Surgical therapy for traumatic brachial plexus lesions is still a great challenge in the field of peripheral nerve surgery. The aim of this study was to present the results of different surgical interventions in patients with this lesion type. Methods. One hundred thirty-four patients with traumatic brachial plexus lesions underwent surgery between January 1991 and September 1999. In more than 50% of the patients, injury was caused by a motorbike accident. Patients underwent surgery a mean of 6.3 months posttrauma. The following surgical techniques were applied: neurolysis for nerve lesions in continuity (27 cases), grafting for lesions in discontinuity (149 cases), and neurotization for root avulsions (67 cases). Sixty-five patients were evaluated for at least 30 months (mean follow up 42.1 months) after surgery. Function was graded using the Louisiana State University Health Sciences Center classification system. Only 2% of the patients had Grade 3 or better function preoperatively, increasing to 52% postoperatively. The effect of surgical measures on the functional results for different muscles were compared (supra- or infraspinatus, deltoid, biceps, and triceps muscles); the best results were obtained for biceps muscle function (57% of patients with Medical Research Council Grades M3–M5 function). Graft reconstruction yielded a better outcome than neurotization. Surgery within 5 months posttrauma clearly resulted in improved recovery of motor function compared with later interventions. Sural nerve grafts (monofascicular nerves) showed better results. Conclusions. The results of neurosurgical interventions for brachial plexus lesions are satisfactory, especially when the operation is performed between 3 and 6 months after trauma.

Minimal Invasive Surgery and Therapy (MIS and MIT) in Hirschsprung's Patients

2008 ◽  
pp. 882-889
Author(s):  
Nilmini Wickramasinghe ◽  
Giuseppe Turchetti ◽  
Barbara Labella ◽  
Andrea Moglia ◽  
Arianna Menciassi ◽  
...  
Keyword(s):  
Minimally Invasive ◽  
Invasive Surgery ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Surgical Techniques ◽  
Diagnostic Procedures ◽  
Minimal Invasive ◽  
Surgical Tools ◽  
Economic Perspectives ◽  
Laparoscopic Tools

The generally accepted treatment for Hirschsprung’s disease is through surgical intervention. Currently, the recognized techniques include the Swenson technique, which is less frequently used as the operation is delicate and can give rise to complications; the Duhamel technique, which is arguably the simplest approach and consistently provides good results; and the Soave technique, which also provides good results but is often more complex than the Duhamel approach. However, surgical and diagnostic procedures of the future will evolve from embracing current technologies that enable minimally invasive approaches to extremely targeted, localized, and high-precision endoluminal techniques. This requires entirely new types of surgical tools capable of entering the human body through natural orifices (by insertion, ingestion, or inhalation), very small incisions (injections), or even through skin absorption and maybe configuring themselves in complex kinetic structures at the specific site of intervention. Moreover, such approaches necessitate modification of classic surgical techniques. While the advantages of minimally invasive surgery and minimally invasive therapy (MIS and MIT) are widely acknowledged, this chapter serves to highlight the advantages of such procedures in pediatric medicine, both at the diagnostic and intervention levels and the consequent implications to classic surgical techniques. The particular focus of the chapter pertains to the specific advantages the following techniques can bring into pediatric diagnostic and surgical techniques in the case of Hirschsprung’s disease: (1) endoluminal miniaturized tools for gastrointestinal endoscopy, (2) gastrointestinal capsules for digestion (e.g., M2A capsule), and (3) laparoscopic tools for surgery. Both technological and economic perspectives are discussed.

scholarly journals Effects of potato-derived protease inhibitors on perianal dermatitis after colon resection for long-segment Hirschsprung’s disease

2012 ◽  
Vol 8 (2) ◽  
pp. 173-176 ◽  
Author(s):  
Steffen Berger ◽  
Janine Rufener ◽  
Peter Klimek ◽  
Zacharias Zachariou ◽  
Colette Boillat
Keyword(s):  
Protease Inhibitors ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Colon Resection ◽  
Perianal Dermatitis
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