A case of severe megacolon due to acquired isolated hypoganglionosis after low anterior resection for lower rectal cancer

Tominaga, Tetsuro; Nagayama, Satoshi; Takamatsu, Manabu; Miyanari, Shun; Nagasaki, Toshiya; Yamaguchi, Takahiro; Akiyoshi, Takashi; Konishi, Tsuyoshi; Fujimoto, Yoshiya; Fukunaga, Yosuke; Ueno, Masashi

doi:10.1007/s12328-019-01079-2

Cited by 4 publications

(6 citation statements)

References 33 publications

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“…He initially did well after surgery but at 4 years after the stoma closure developed severe post-operative constipation. 19 His reoperation laparoscopic total colectomy histopathologically showed ganglion cells gradually decreasing and degenerating from the normal to dilated region. 19 The etiology was suspected to have been secondary to chemotherapy, radiation, ischemia, and/or an episode of severe colitis shortly after the postoperative period with subsequent resolution after medical treatment.…”

Section: Discussionmentioning

confidence: 99%

“…19 His reoperation laparoscopic total colectomy histopathologically showed ganglion cells gradually decreasing and degenerating from the normal to dilated region. 19 The etiology was suspected to have been secondary to chemotherapy, radiation, ischemia, and/or an episode of severe colitis shortly after the postoperative period with subsequent resolution after medical treatment. 19 Interestingly, there are many reports of children and adults with acquired immune-mediated aganglionosis and HD-like clinical presentation.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…19 The etiology was suspected to have been secondary to chemotherapy, radiation, ischemia, and/or an episode of severe colitis shortly after the postoperative period with subsequent resolution after medical treatment. 19 Interestingly, there are many reports of children and adults with acquired immune-mediated aganglionosis and HD-like clinical presentation. Notably, a 48-year-old female from China with no prior surgical history, and neither a personal past medical history of bowel disease nor related family history presented with symptoms of discontinuation of bowel movements for 2 months.…”

Section: Discussionmentioning

confidence: 99%

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Reoperation for Hirschsprung Disease: Two cases of Vanishing Ganglion Cells and Review of the Literature

et al. 2022

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Hirschsprung disease (HD) is characterized by circumferential aganglionosis of the rectum with variable proximal bowel involvement. The underlying pathogenesis is due to failure of caudal migration of neural crest cells during pre-natal development, causing functional bowel obstruction. Definitive therapy is surgical resection; however, a subset of patients will require reoperation. An important cause of reoperation is the rare but distinct entity described as the ganglion cell “vanishing” phenomenon. In this phenomenon, affected patients have normal circumferential ganglion cells present at the proximal margin during primary resection. They undergo a variable asymptomatic period post-primary resection but ultimately develop recurrent symptoms. Upon reoperation, ganglion cells seemingly vanish and are no longer present in the previously functioning and ganglionated bowel proximal to the initial anastomotic site. To further characterize and investigate this poorly understood pathology, here we present 2 cases of HD patients who required reoperation. Our small series implicates that an immune component may contribute as patient 2 had a brisk neurotrophic eosinophilic infiltrate only present in the reoperation specimen. However, this was not observed in patient 1. Other possible etiologies include post-operative ischemia/hypoxia, visceral neuropathy, or signaling abnormalities within the residual ganglion cells themselves.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Reoperation for Hirschsprung Disease: Two cases of Vanishing Ganglion Cells and Review of the Literature

et al. 2022

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“…The first therapeutic option is conservative treatment, as most patients can be managed non-surgically [67]. However, drug treatment is ineffective in controlling symptoms in 50-70% of cases, and it can be difficult to tolerate, especially in the context in which it needs to be administered throughout life to avoid recurrence of symptoms [67,[71][72][73]. This aspect becomes even more obvious in the case of the association with neuro-psychiatric disorders, where the patient's compliance and cooperation is formally deficient.…”

Section: Therapeutic Managementmentioning

confidence: 99%

Idiopathic Megacolon—Short Review

et al. 2021

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Introduction: Idiopathic megacolon (IM) is a rare condition with a more or less known etiology, which involves management challenges, especially therapeutic, and both gastroenterology and surgery services. With insufficiently drawn out protocols, but with occasionally formidable complications, the condition management can be difficult for any general surgery team, either as a failure of drug therapy (in the context of a known case, initially managed by a gastroenterologist) or as a surgical emergency (in which the diagnostic surprise leads additional difficulties to the tactical decision), when the speed imposed by the severity of the case can lead to inadequate strategies, with possibly critical consequences. Method: With such a motivation, and having available experience limited by the small number of cases (described by all medical teams concerned with this pathology), the revision of the literature with the update of management landmarks from the surgical perspective of the pathology appears as justified by this article. Results: If the diagnosis of megacolon is made relatively easily by imaging the colorectal dilation (which is associated with initial and/or consecutive clinical aspects), the establishing of the diagnosis of idiopathic megacolon is based in practice almost exclusively on a principle of exclusion, and after evaluating the absence of some known causes that can lead to the occurrence of these anatomic and clinical changes, mimetically, clinically, and paraclinically, with IM (intramural aganglionosis, distal obstructions, intoxications, etc.). If the etiopathogenic theories, based on an increase in the performance of the arsenal of investigations of the disease, have registered a continuous improvement and an increase of objectivity, unfortunately, the curative surgical treatment options still revolve around the same resection techniques. Moreover, the possibility of developing a form of etiopathogenic treatment seems as remote as ever.

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Chronic intestinal pseudo-obstruction due to adult-onset acquired isolated hypoganglionosis with muscular atrophy in the small intestine: a case report and review of literature

Tayama,

Furuta,

Morito

et al. 2024

Clin J Gastroenterol

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A case of severe megacolon due to acquired isolated hypoganglionosis after low anterior resection for lower rectal cancer

Cited by 4 publications

References 33 publications

Reoperation for Hirschsprung Disease: Two cases of Vanishing Ganglion Cells and Review of the Literature

Reoperation for Hirschsprung Disease: Two cases of Vanishing Ganglion Cells and Review of the Literature

Idiopathic Megacolon—Short Review

Chronic intestinal pseudo-obstruction due to adult-onset acquired isolated hypoganglionosis with muscular atrophy in the small intestine: a case report and review of literature

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