Maladie de Hirschsprung chez l'adulte

Faucheron, Jean‐Luc; Poncet, Gilles; Voirin, David; Moreno, W.; Stathopoulos, Lefteris

doi:10.1016/s0246-0424(09)43191-1

Cited by 9 publications

(13 citation statements)

References 14 publications

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“…Its diagnosis, usually neonatal, is confirmed in adults by manometry showing the absence of rectoanal inhibitory reflex. Deep biopsies are performed in case of diagnostic doubt and may show marked hyperplasia of the nerve threads [1, 5].…”

Section: Discussionmentioning

confidence: 99%

“…Unrecognized HD until adulthood represents 2% of patients with chronic, rebellious constipation and affects men more often (four men for every woman). The relatively good tolerance of the disease is mainly due to a short aganglionic zone, less than 10 cm long, and an upstream colon keeping enough propulsive force to counter distal obstruction [1]. In retrospect, HD could have been suspected on the basis of our patient’s history found during the interview (megadolichocolon, manual evacuation of stools, and evacuating enemas since childhood).…”

Section: Discussionmentioning

confidence: 99%

“…Once the diagnosis is well established in adults, the intervention is almost systematically indicated [1]. The evolution leads not only to worsening constipation by continuing low-noise distention of the upstream colon, as well as undernutrition and alteration of the general state, but also to sudden complications such as intestinal perforation, severe respiratory failure due to major abdominal distention [6], or necrotizing enterocolitis with sometimes fatal septic shock [7–9].…”

Section: Discussionmentioning

confidence: 99%

“…The diagnosis of HD in adulthood is uncommon and is reported to correspond to 2% of adult patients with chronic, rebellious constipation. The symptomatology of these patients is refractory constipation and abdominal pain of the colic type [1, 2].…”

Section: Introductionmentioning

confidence: 99%

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Hirschsprung disease in an adult with intestinal malrotation and volvulus: an exceptional association

Lupon

Labbé²,

Sondji³

2019

J Med Case Reports

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Background Hirschsprung disease is a neonatal discovery in almost all cases, and the association of Hirschsprung disease in adults with symptomatic intestinal malrotation is unusual. This combination delays diagnosis and can lead to mistake in surgical strategy. Case presentation A 43-year-old patient with a history of colectomy for colonic inertia and megadolichocolon was admitted to the Carcassonne Hospital emergency room for a volvulus of small bowel obstruction in a chronic intestinal obstruction context with episodes of acute, variable-looking occlusive syndromes. Intestinal malrotation was discovered during surgical small bowel detorsion. The acute occlusion syndrome recurred after the procedure. In view of the unfavorable evolution, an emptying of the dilated small bowel and a discharge ileostomy upstream of the rectum were performed. In the face of postoperative improvement, rectal manometry and deep full parietal rectal biopsies made it possible to highlight the diagnosis of Hirschsprung disease. The patient thus had functional acute occlusive syndromes and chronic occlusion due to Hirschsprung disease of attenuated form and acute organic occlusive syndromes related to her incomplete common mesentery. Conclusions This rare association, which may be responsible for delayed diagnostic and therapeutic wandering, highlights the importance of performing manometry and deep full parietal biopsies before a colectomy for colonic inertia, as well as the possibility of suggesting a common Hirschsprung disease and/or mesentery in an adult with multiple occlusive syndromes of variable appearance.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Hirschsprung disease in an adult with intestinal malrotation and volvulus: an exceptional association

Lupon

Labbé²,

Sondji³

2019

J Med Case Reports

View full text Add to dashboard Cite

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“…Compared with the Soave and Swenson procedures, it is superior in terms of impotence rate, anastomotic dehis- cence and reservoir function to minimize soiling (8). Myomectomy has the advantage of being technically easier and presenting a low morbidity, but it has worse functional outcomes and can only be used in ultra-HD (7,8).…”

Section: Discussionmentioning

confidence: 99%

Hirschsprung disease with debut in adult age as acute intestinal obstruction: case report

et al. 2016

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Hirschsprung's disease is characterized by absence of ganglion cells in submucosal and myenteric plexus of distal bowel. Most cases become manifest during the neonatal period, but in rare instances, this disease is initially diagnosed in adult age. It usually presents as severe constipation with colonic dilatation proximal to the aganglionic segment. The treatment is surgical, removing the aganglionic segment and restoring continuity of digestive tract. The disease rarely presents as an acute intestinal obstruction. We report a case not previously diagnosed, which presented as a massive colonic dilatation with a maximum diameter of 44 cm, with imminent risk of drilling that forced to perform an emergency surgery. We include a review of existing literature.

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Complication of Hirschsprung’s disease immediately after pregnancy: A rare case report

Sergi

Serra

Cucciarrè

et al. 2021

International Journal of Surgery Case Reports

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Highlights Hirschprung’s disease (HD) is a rare congenital colonic disorder. Volvulus is a rare complication of HD, described in children and adults and occasionally in infants. Diagnosis of Hirschprung’s disease was accepted. The sigmoid volvulus was HD complication appeared at the term of pregnancy. Duhamel’s procedure revealed as a safe technique to use in HD adult.

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Maladie de Hirschsprung chez l'adulte

Cited by 9 publications

References 14 publications

Hirschsprung disease in an adult with intestinal malrotation and volvulus: an exceptional association

Hirschsprung disease in an adult with intestinal malrotation and volvulus: an exceptional association

Hirschsprung disease with debut in adult age as acute intestinal obstruction: case report

Complication of Hirschsprung’s disease immediately after pregnancy: A rare case report

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