Total Small Bowel and Colon Aganglionosis

Fj, Walther; Kootstra, G

doi:10.1055/s-2008-1063137

Cited by 2 publications

(2 citation statements)

References 2 publications

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“…Walther et al described a patient with TCSA who presented with dilated small bowel and visible peristalsis on X-ray despite biopsy-proven absence of ganglion cells distal to the stomach. Intraoperatively, distal small bowel peristalsis was observed; however, that patient died on DOL 80 of septicemia [18].…”

Section: Discussionmentioning

confidence: 99%

Delayed appearance of mature ganglia in an infant with an atypical presentation of total colonic and small bowel aganglionosis: a case report

et al. 2019

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Background Total colonic and small bowel aganglionosis (TCSA) occurs in less than 1% of all Hirschsprung’s disease patients. Currently, the mainstay of treatment is surgery. However, in patients with TCSA, functional outcomes are often poor. A characteristic transition zone in TCSA can be difficult to identify which may complicate surgery and may often require multiple operations. Case presentation We present the case of a male infant who was diagnosed with biopsy-proven total colonic aganglionosis with extensive small bowel involvement as a neonate. The patient was diverted at one month of age based on leveling biopsies at 10 cm from the Ligament of Treitz. At 7 months of age, during stoma revision for a prolapsed stoma, intra-operative peristalsis was observed in nearly the entire length of the previously aganglionic bowel, and subsequent biopsies demonstrated the appearance of mature ganglion cells in a previously aganglionic segment. Conclusions TCSA remains a major challenge for pediatric surgeons. Our case introduces new controversy to our understanding of aganglionosis. Our observations warrant further research into the possibility of post-natal ganglion maturation and encourage surgeons to consider a more conservative surgical approach.

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Section: Discussionmentioning

confidence: 99%

Delayed appearance of mature ganglia in an infant with an atypical presentation of total colonic and small bowel aganglionosis: a case report

et al. 2019

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“…Abdominal protrusion is most often conspicuous, but may be absent. Passage of meconium is lacking or at least delayed (Zuelzer-Wilson's disease: 2,6-8, 16, 19-21, 23; absence ofthe enteric nervous system: 1-5,13, 14,17,18,[22][23][24]. Rarely, an acute appendicitis is the first sign of the underlying disease (2).…”

Section: Discussionmentioning

confidence: 99%

Zuelzer-Wilson's Syndrome and Absence of the Enteric Nervous System

Rudin¹,

Jenny²,

Fliegel³

et al. 1986

Eur J Pediatr Surg

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47 children have been treated in Basel for aganglionosis (Hirschsprung's disease) during the last 25 years. Six children presented severe vomiting and an ileus as leading symptoms instead of chronic constipation, the classical leading symptom of Hirschsprung's disease. Clinical, radiological and intraoperative findings were virtually identical in these 6 patients. However, enzyme histochemical and immunocytochemical investigations disclosed an aganglionosis of the entire colon (Zuelzer-Wilson's disease) in 3 patients, absence of the enteric nervous system in the small and large intestine in 2 patients, and a combination of both in 1 patient. In contrast to children suffering from aganglionosis of the entire colon, the chance of survival for patients with an absence of the enteric nervous system is extremely small. It is therefore necessary in presence of severe vomiting and an ileus to take intraoperative biopsies from the large and the small intestine. The precise diagnosis can be made only by using enzyme histochemical and immunocytochemical techniques.

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Total Small Bowel and Colon Aganglionosis

Cited by 2 publications

References 2 publications

Delayed appearance of mature ganglia in an infant with an atypical presentation of total colonic and small bowel aganglionosis: a case report

Delayed appearance of mature ganglia in an infant with an atypical presentation of total colonic and small bowel aganglionosis: a case report

Zuelzer-Wilson's Syndrome and Absence of the Enteric Nervous System

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