Journal of pediatric surgery · 2026
Skip segment Hirschsprung Disease: Unicentric experience with four cases and systematic literature review.
СтатьяСистематический обзорКлинический случайНазвание и аннотация: Перевод в очереди
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Bertolino Alessia, Mottadelli Giulia, Pagliara Camilla, Erculiani Marta, Pini Prato Alessio
Аннотация
INTRODUCTION: Skip lesion aganglionosis is characterized by intestinal aganglionosis interrupted by portions of normally ganglionated bowel that is located in between segments of aganglionosis. Even though this is a well-known entity, only scattered cases have been reported so far. The aim of this manuscript is to present our series of patients and perform a systematic literature review, to offer a critical overview of possible management.
PATIENTS AND METHODS: We hereby present a case series of 4 patients diagnosed during the last five years in our Centre in accordance to SCARE guidelines. A systematic review according to PRISMA guidelines was performed to better address this extremely rare surgical dilemma.
RESULTS: Our case-series included only patients with ultralong Hirschsprung, being 3 Total Colonic Aganglionosis and 1 Extended Aganglionosis. Detailed description of each case is reported. Systematic Literature Review included ended up with a total of 50 patients mostly suffering from ultralong Hirschsprung and whose diagnostic pathway proved to be heterogenous with diagnostic delays and misdiagnoses.
DISCUSSION: Based on this case series and on literature data, skip lesion aganglionosis should be referred to highly experienced referral centers and deserve a dedicated diagnostic and therapeutic management. The motility of ganglionated bowel distal to skip lesions proved to be impaired in our experience thus suggesting avoiding the use of this non-functioning bowel to restore continuity. The Authors propose a detailed algorithm to reduce misdiagnoses and avoid dangerous delays.
PATIENTS AND METHODS: We hereby present a case series of 4 patients diagnosed during the last five years in our Centre in accordance to SCARE guidelines. A systematic review according to PRISMA guidelines was performed to better address this extremely rare surgical dilemma.
RESULTS: Our case-series included only patients with ultralong Hirschsprung, being 3 Total Colonic Aganglionosis and 1 Extended Aganglionosis. Detailed description of each case is reported. Systematic Literature Review included ended up with a total of 50 patients mostly suffering from ultralong Hirschsprung and whose diagnostic pathway proved to be heterogenous with diagnostic delays and misdiagnoses.
DISCUSSION: Based on this case series and on literature data, skip lesion aganglionosis should be referred to highly experienced referral centers and deserve a dedicated diagnostic and therapeutic management. The motility of ganglionated bowel distal to skip lesions proved to be impaired in our experience thus suggesting avoiding the use of this non-functioning bowel to restore continuity. The Authors propose a detailed algorithm to reduce misdiagnoses and avoid dangerous delays.