Adult Hirschsprung’s disease

Martinez, Joseph P.

doi:10.1017/cem.2014.57

Cited by 9 publications

(4 citation statements)

References 3 publications

(3 reference statements)

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“…Hirschsprung's disease is a widely recognized condition characterized by congenital absence of intestinal ganglia in the plexus. On the other hand, a few adults who present with the clinical symptoms of chronic constipation, pseudoobstruction, and acute abdomen are diagnosed with an adult onset of AIHG [8,9]. A nationwide survey over 10 years in Japan showed that only 8 (7.1%) of 112 patients with isolated hypoganglionosis were diagnosed with AIHG [3].…”

Section: Discussionmentioning

confidence: 99%

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

Tominaga

Nagayama

Takamatsu

et al. 2019

Clin J Gastroenterol

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Acquired isolated hypoganglionosis is a rare intestinal neurological disease, which presents in adulthood with the clinical symptoms of chronic constipation. A 39-year-old man underwent laparoscopic low anterior resection and covering ileostomy for locally advanced-rectal cancer. A 6-month course of postoperative adjuvant chemotherapy was completed, followed by closure of the ileostoma. After the closure, he developed severe colitis which required 1-month of hospitalization. Mucosal erosions and pseudo-membrane formation were evident on colonoscopy and severe mucosal damage characterized by infiltration of inflammatory cells and crypt degeneration were pathologically confirmed. Even after the remission of the colitis, he suffered from severe constipation and distention. At 4 years after the stoma closure, he decided to undergo laparoscopic total colectomy. Histopathologically, the nerve fibers and ganglion cells became gradually scarcer from the non-dilated to dilated regions. Immunohistochemical staining examination confirmed that the ganglion cells gradually decreased and became degenerated from the normal to dilated region, thereby arriving at the final diagnosis of isolated hypoganglionosis. The patient recovered without any complications and there has been no evidence of any relapse of the symptoms. We present a case of acquired isolated hypoganglionosis-related megacolon, which required laparoscopic total colectomy, due to severe enterocolitis following stoma closure.

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Section: Discussionmentioning

confidence: 99%

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

Tominaga

Nagayama

Takamatsu

et al. 2019

Clin J Gastroenterol

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“…[25][26][27][28][29] HSCR beyond childhood and notably in adulthood is considered rare. [30][31][32] In their systematic review and metaanalysis of HSCR in adults, Doodnath and Puri 31 reported that disease was limited to the rectum and rectosigmoid in 79.8% and 12.5% of patients respectively. Most had symptoms during the neonatal period followed by lifelong constipation, abdominal distention, and pain that was poorly responsive to laxatives or enemas.…”

Section: Clinical Presentationmentioning

confidence: 99%

Diagnosis of Hirschsprung Disease

2019

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Diagnosis or exclusion of Hirschsprung disease (HSCR) is a frequent exercise in any pediatric hospital. Although HSCR may present at different ages and with varied clinical findings, the most common presentation is a neonate with severe constipation or signs of intestinal obstruction. A variety of diagnostic tests including contrast enema and anorectal manometry may be used as diagnostic screens, but diagnosis ultimately rests upon histopathological evaluation of a rectal biopsy. For the experienced pathologist, conventional hematoxylin-and-eosin-stained sections often suffice to exclude HSCR or establish the diagnosis. However, ancillary diagnostic tests such as acetylcholinesterase histochemistry or calretinin immunohistochemistry are complementary and extremely helpful in some cases. In this Perspectives article, we review the clinical and pathological features of HSCR, highlight those that are found in most patients, and discuss how to address particularly challenging aspects of the diagnostic workup.

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“…In fact, further considering the patient’s medical history in this case, he also had decades of constipation and had received multiple conservative treatments. Surgery is the definitive treatment method for adult HD [ 22 , 23 ].…”

Section: Discussionmentioning

confidence: 99%

Hirschsprung\'s Disease-Related Giant Sigmoid Volvulus Complicated by Refractory Hypertension in an Elderly Man

Sun

et al. 2018

Am J Case Rep

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Patient: Male, 82Final Diagnosis: Hirschsprung’s disease-related sigmoid volvulus complicated with refractory hypertensionSymptoms: Constipation • moderate abdominal pain and progressive abdominal distension • hypertensionMedication: Antihypertension medicationClinical Procedure: CT scan • Hartmann’s procedureSpecialty: General SurgeryObjective:Rare diseaseBackground:Sigmoid volvulus (SV) is a life-threatening condition occasionally seen in adults. Adult Hirschsprung’s disease (HD)-related SV is rarely complicated by difficult-to-control hypertension. In this report we present the case of an elderly man with a rare constellation of HD, SV, and refractory hypertension.Case Report:An 82-year-old man had long-term constipation, moderate abdominal pain, and progressive abdominal distension. A CT scan revealed the typical “coffee bean sign”. Blood pressure was abnormal high. Subsequently, the patient’s condition deteriorated. Therefore, he underwent a Hartmann’s procedure. A giant and redundant sigmoid colon (length more than 60 cm, maximal diameter about 15 cm) was demonstrated to be the cause of SV during the process of surgery. Moreover, abdominal compartment syndrome caused by SV resulted in his high and refractory blood pressure (BP). Postoperative pathological results revealed HD in his sigmoid colon.Conclusions:SV is rarely combined with conditions like refractory hypertension or HD among the elderly. Clinical features of SV typically present with long-term constipation, severe abdominal pain, and progressive abdominal distension. The “coffee bean sign” could be observed in imaging examinations. It is important to note that the management of SV is to relieve the obstruction and prevent recurrence, no matter which therapy is used in elderly patients with Hirschsprung’s disease.

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Adult Hirschsprung’s disease

Cited by 9 publications

References 3 publications

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

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

Diagnosis of Hirschsprung Disease

Hirschsprung\'s Disease-Related Giant Sigmoid Volvulus Complicated by Refractory Hypertension in an Elderly Man

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