Diagnosis and surgical treatment of isolated hypoganglionosis

Zhang, Hongyi; Feng, Jiexiong; Huang, Lei; Wei, Guo; Wei, Mingfa; Weng, Yi-Zheng

doi:10.1007/s12519-008-0053-3

Cited by 30 publications

(13 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Etiology is represented by impaired craniocaudal migration of neuroblasts during the first 3 months of gestation [ 10 , 11 ]. The transition of the distal aganglionic segment to the proximal normal bowel may be characterized by a segment of hypoganglionosis with maintained but reduced number of ganglia [ 11 , 12 ]. A number of ganglia less than 10 mm is generally considered diagnostic for hypoganglionosis [ 13 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Adult-Onset Rectosigmoid Hypoganglionosis

et al. 2018

View full text Add to dashboard Cite

Patient: Male, 20Final Diagnosis: Rectosigmoid hypoganglionosisSymptoms: Severe abdominal pain • obstipation • vomiting • shortness of breath • palpitationsMedication: —Clinical Procedure: Hartmann’s procedureSpecialty: SurgeryObjective:Congenital defects/diseasesBackground:Intestinal hypoganglionosis is very rare and accounts for 3% to 5% of all classified congenital intestinal innervation disorders. Isolated hypoganglionosis of the colon is a particularly rare form of the disease, and differential diagnosis includes association with Hirschsprung’s disease and chronic intestinal pseudo-obstruction (CIPO) related to visceral myopathies. Most cases are diagnosed at an early age or in childhood with only a few cases reported in adults.Case Report:We report a case of isolated hypoganglionosis of the rectum and sigmoid presenting as an emergency with acute intestinal obstruction in a 20-year-old male patient. A history of chronic constipation was reported since childhood, but this condition had never been investigated. A preoperative CT scan showed a megasigmoid and megarectum. A Hartmann’s procedure was performed. The patient made a slow recovery and was discharged on the 12th postoperative day in good condition. Histology showed features consistent with isolated hypoganglionosis, and a full thickness rectal biopsies taken 2 months later confirmed the diagnosis.Conclusions:Isolated hypoganglionosis in an adult is very rare, and a high index of suspicion is warranted in young patients with a history of chronic constipation to avoid delayed presentation as an emergency.

show abstract

Section: Discussionmentioning

confidence: 99%

A Rare Case of Adult-Onset Rectosigmoid Hypoganglionosis

et al. 2018

View full text Add to dashboard Cite

show abstract

“…IH can be differentiated by reduced number and size of myenteric ganglia, a low or absent AchE activity in the lamina propria, and hypertrophy of the muscularis mucosa and circular muscle. In HD, there is an absence of nerve cells in both submucosal and intramuscular layers of the distal colon 3,5 . Our patient had sparse dysplastic nerve cells in the myenteric plexus of the distal narrowed part of the removed specimen with normal ganglia at both proximal and distal margins.…”

Section: Discussionmentioning

confidence: 99%

“…IND is characterized by hyperganglionosis, giant ganglia and ectopic ganglia. In isolated hypoganglionosis (IH), the number of ganglia per a centimeter of bowel's longitudinal axis is decreased and the distance between ganglia, as well as the plexus area, is increased 4,5 . There is very low or absent activity of acetyl cholinesterase (AchE) in the mucosa.…”

Section: Anahtar Kelimeler: Yetișkin; Kolonda Yalancı Tıkanma; Hischsmentioning

confidence: 99%

Isolated adult hypoganglionosis resulting in toxic megacolon: A Case Report

Kahramanca¹,

Özgehan²,

Celep³

et al. 2014

Kafkas J Med Sci

View full text Add to dashboard Cite

show abstract

“…The diagnosis of IH is histological and requires the demonstration of the following histological characteristics in three to five full-thickness biopsies of the intestinal wall: very low or absent activity of acetylcholinesterase activity in the mucosa; significant reduction of nerve cells in the myenteric and submucous plexus; and hypertrophy of the muscularis mucosa and muscle layers [7,8]. Since conservative therapies cannot usually alleviate symptoms effectively, the treatment of choice for IH involves resection of the affected bowel segment [8,10]. …”

Section: Discussionmentioning

confidence: 99%

Hypoganglionosis in pregnancy: a case report

Figueiredo¹,

Martins²,

Palma³

et al. 2012

J Med Case Reports

View full text Add to dashboard Cite

IntroductionWe report a very rare case of isolated hypoganglionosis first diagnosed during early pregnancy, which should be discussed from an obstetric and a gastroenterological point of view.Case presentationA pregnant 18-year-old Caucasian woman presented at twelve weeks of gestation with lower abdominal pain, mild constipation and a large abdominal mass. Abdominal and pelvic magnetic resonance imaging demonstrated a megarectum and megasigmoid, and our patient was managed with medical therapy during her pregnancy, which occurred without major incidents. At the onset of labor, a fecaloma obstructing the pelvic outlet was detected, which required manual disimpaction. However, during the procedure a sudden continuous fetal bradycardia was detected. An emergency Cesarean section was performed but the fetus suffered hypoxic ischemic encephalopathy. One year after the delivery, our patient underwent a sigmoid resection. A histopathological analysis revealed a reduction of nerve cells in the myenteric and submucous plexus, suggesting hypoganglionosis.ConclusionAlthough there are some reports of pregnancies complicated by megacolon, they are too few and too old to delineate guidelines for clinical orientation. In our article, we discuss several issues regarding the management of these rare intestinal innervation disorders during pregnancy that we believe will enhance their obstetric and gastroenterological management during pregnancy.

show abstract

Diagnosis and surgical treatment of isolated hypoganglionosis

Cited by 30 publications

References 17 publications

A Rare Case of Adult-Onset Rectosigmoid Hypoganglionosis

A Rare Case of Adult-Onset Rectosigmoid Hypoganglionosis

Isolated adult hypoganglionosis resulting in toxic megacolon: A Case Report

Hypoganglionosis in pregnancy: a case report

Contact Info

Product

Resources

About