Idiopathic Megacolon in an Adolescent Girl: a Case Report on a Rare Disease

Author(s):  
Sharhanin Bahrudin ◽  
Abdul Malek Moahamd ◽  
Azmi Mohd Nor ◽  
Faisal Elagili

Abstract Introduction: Idiopathic Megacolon is a rare condition where there is persistent dilatation of colon in the absence of identifiable cause. Symptoms start as early as in childhood or in adulthood. Colectomy have shown to have high success rate in patient with idiopathic megacolon. We reported a case of an adolescent girl with idiopathic megacolon that was successfully treated with colectomy.Presentation of case: A 15 years old girl presented with a complaint of abdominal pain associated with gradual abdominal distension for 1 week duration, not passing flatus and had multiple episodes of vomiting. She was diagnosed to have sigmoid volvulus and underwent endoscopy decompression, however symptoms recurred. Segmental colectomy was performed and she had an uneventful recovery. Discussion: Patient presented with acute intestinal obstruction attributed to sigmoid volvulus with a history of constipation. A diagnostic dilemma between Hirschsprung’s disease with other causes of megacolon occurred as these diseases have similar presenting symptoms. A confirm histopathology of presence of ganglionic cells within the plexus exclude the diagnosis of Hirschsprung’s disease hence the diagnosis of Idiopathic Megacolon was made. Segmental colectomy give good result in relieving patient symptoms of abdominal pain and constipation and one of the recommended surgical options in treating Idiopathic Megacolon.Conclusion: Idiopathic megacolon is a rare disease and for a long time has been a disease of exclusion. Rectal biopsy is important to exclude the diagnosis of Hirschsprung’s Disease. There are wide variety of surgical treatment available in treating Idiopathic megacolon. Segmental Colectomy have shown good success rate.

1979 ◽  
Vol 9 (3) ◽  
pp. 234-240 ◽  
Author(s):  
Naomi Iwai ◽  
Shuhei Ogita ◽  
Makoto Kida ◽  
Bunzo Nishioka ◽  
Yoshihiro Fujita ◽  
...  

BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Abay Wondimu Gosaye ◽  
Temesgen Setato Nane ◽  
Tihitena Mammo Negussie

Abstract Background Sigmoid volvulus is an uncommon problem in children and adolescents, and is rarely considered a diagnosis in this group. A high index of suspicion is necessary to reach a diagnosis and avoid morbidity and mortality. Sigmoid volvulus is a rare complication of Hirschsprung’s disease, which has been reported in neonates, children, and adults. Here we report a case of sigmoid volvulus accompanied by undiagnosed Hirschsprung's disease. Case presentation A 9 years old boy who presented with sudden onset of colicky abdominal pain of 4 h duration associated with gross abdominal distension and 2 episodes of non-bilious vomiting. A plain abdominal radiographs showed single hugely dilated bowel loops in the left lower quadrant with single air fluid level. Sigmoid volvulus was considered and rectal tube deflation was done and it was successful. Full thickness rectal biopsy was done and it was consistent with aganglionic megacolon. A primary trans-anal Soave endo-rectal pull through was done 3 weeks later, after biopsy result arrived, which yielded loss of symptoms and regular bowel movement. Conclusions Sigmoid volvulus should be considered in the differential for children presenting with acute onset of abdominal obstruction. It should be known that when its’s diagnosed in children, it is often associated with Hirschsprung's disease. Therefore, a proper diagnostic and treatment algorithm should be followed in order not to miss associated HD and to give optimum care to such patients.


This chapter on paediatric surgery covers procedures performed from fetus to 16 years of age. The wide range is succinctly covered with appropriate detail for both those looking to apply for medicine, and those in medical school. The chapter reviews oncological conditions, most importantly, leukaemia. It also studies common conditions such as abdominal pain, hernia, undescended testicles, and appendicitis. Surgical management of gastrointestinal illnesses such as pyloric stenosis, intussusception, and Hirschsprung’s disease is covered along with urological conditions such as hypospadias. It also discusses important neonatal presentations of necrotizing enterocolitis and gastrointestinal atresia, to name a few.


2016 ◽  
pp. bcr2016214693 ◽  
Author(s):  
Abhishek Ranjan ◽  
Vishesh Jain ◽  
Shilpa Sharma ◽  
Devendra Kumar Gupta

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Takeshi Shono ◽  
Kumiko Shono ◽  
Yoshiko Hashimoto ◽  
Shohei Taguchi ◽  
Masanori Masuda ◽  
...  

Abstract Background Congenital hyperinsulinism (CH) is a rare disease, characterized by severe hypoglycemia induced by inappropriate insulin secretion from pancreatic beta-cells in neonate and infant. Hirschsprung’s disease (HD) is also a rare disease in which infants show severe bowel movement disorder. We herein report an extremely rare case of combined CH and HD. Case presentation The patient was a full-term male infant who showed poor feeding, vomiting, and hypotonia with lethargy on the day of birth. He was transferred to tertiary hospital after a laboratory analysis revealed hyperinsulinemic hypoglycemia. The patient showed remarkable abdominal distension without meconium defecation. An abdominal X-ray showed marked dilatation of the large bowel. He was diagnosed with CH (nesidioblastosis) associated with suspected HD. He was initially treated with an intravenous infusion of high-dose glucose with the intermittent injection of glucagon. This was successfully followed by treatment with diazoxide and octreotide (a somatostatin analog). At 8 months of age, HD was confirmed by the acetylcholinesterase staining of a rectal mucosal biopsy specimen, and a transanal pull-through operation was performed to treat HD. At 14 months of age, subtotal pancreatectomy was performed for the treatment of focal CH located in the pancreatic body. His postoperative course over the past 12 years has been uneventful without any neurologic or bowel movement disorders. Conclusions Although it is extremely rare for CH to be associated with HD, associated HD should be considered when a patient with CH presents severe constipation.


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