scholarly journals Congenital hyperinsulinism associated with Hirschsprung’s disease—a report of an extremely rare case

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Takeshi Shono ◽  
Kumiko Shono ◽  
Yoshiko Hashimoto ◽  
Shohei Taguchi ◽  
Masanori Masuda ◽  
...  
Keyword(s):  
Rare Disease ◽  
Pancreatic Beta Cells ◽  
Rare Case ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Bowel Movement ◽  
Severe Hypoglycemia ◽  
Hyperinsulinemic Hypoglycemia ◽  
High Dose ◽  
Congenital Hyperinsulinism

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.

A rare case of Hirschsprung's disease in a young Nigerian man

2018 ◽  
Vol 6 (1) ◽  
pp. 21
Author(s):  
Mohammed Kabir Saleh ◽  
Austine Abebe Osawe ◽  
Sule Idris Kazaure ◽  
Abdulrahman Abba Sheshe
Keyword(s):  
Rare Case ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease

scholarly journals COMPLICATION OF HIRSCHSPRUNG’S DISEASE IMMEDIATELY AFTER PREGNANCY: A RARE CASE REPORT

2021 ◽  
pp. 105893
Author(s):  
William Sergi ◽  
Francesco Serra ◽  
Giovanni Cucciarrè ◽  
Nicola De Ruvo ◽  
Roberta Gelmini
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Rare Case Report

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

2021 ◽  
Author(s):  
Sharhanin Bahrudin ◽  
Abdul Malek Moahamd ◽  
Azmi Mohd Nor ◽  
Faisal Elagili
Keyword(s):  
Abdominal Pain ◽  
Success Rate ◽  
Rare Disease ◽  
Adolescent Girl ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Sigmoid Volvulus ◽  
Uneventful Recovery ◽  
Idiopathic Megacolon ◽  
Segmental Colectomy

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.

scholarly journals One stage transanal full thickness pull-through operation for rectosigmoid Hirschsprung's disease

2016 ◽  
Vol 9 (3) ◽  
pp. 135
Author(s):  
A. K. M. Zahid Hossain ◽  
Gazi Zahirul Hasan ◽  
Susankar Kumar Mandal ◽  
Md. Nooruzzaman ◽  
A. Shahinoor ◽  
...  
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Bowel Movement ◽  
Long Term Results ◽  
Full Thickness ◽  
Urinary Function ◽  
One Stage ◽  
Pull Through

<p>The surgical management of Hirschsprung's disease (HD) has changed from the original staged operations to the latest introduced minimally invasive one stage techniques. One stage transanal full thickness Swenson-like procedure is a new concept of single stage procedure for HD. We reviewed the early outcome of one stage transanal Swenson-like pull-through operation for rectosigmoid HD. By using the transanal concept we choose to apply Swenson's principle in transanal dissection for the primary treatment of HD and describe technical aspects and impact on fecal and urinary function. We reviewed our series of HD patient who underwent one-stage transanal full thickness, Swenson-like rectosigmoid dissection, assessing for postoperative stricture or stenosis, anastomotic leak, enterocolitis, obstruction and long-term results for bowel and urinary function. Of 15 patients all had the transanal resection, the age of the patients ranged from 6 months to 5 years. The average length of resection was 20 ± 5 cm. Mean follow-up was 24 months. 10 patients were at least three years old at follow-up and were assessed for urinary and fecal continence. All had the voluntary bowel movement and urinary continence. Three patients had episodes of postoperative enterocolitis and two patients developed stenosis at the anastomotic site. Postoperative frequent bowel movement was present in all patients and continued for 2-8 weeks. In conclusion, our data support the fact that a modification of Swenson's original transabdominal dissection concept using the recently describe transanal approach is an excellent technique for HD and produces excellent long-term outcome for fecal and urinary function.</p><p> </p>

A rare case of multiple skip segment Hirschsprung’s disease in the ileum and colon

2013 ◽  
Vol 30 (3) ◽  
pp. 349-351 ◽  
Author(s):  
Elif Emel Erten ◽  
Yusuf Hakan Çavuşoğlu ◽  
Nilüfer Arda ◽  
Ayşe Karaman ◽  
Çağatay Evrim Afşarlar ◽  
...  
Keyword(s):  
Rare Case ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease

Rare association of Beckwith-Wiedemann syndrome with Hirschsprung’s disease in an infant with hypoglycemia

2020 ◽  
Vol 13 (4) ◽  
pp. e235121 ◽  
Author(s):  
Nikhil Shah ◽  
Anuradha Khadilkar ◽  
Vaman Khadilkar ◽  
Sagar Lad
Keyword(s):  
High Glucose ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Rectal Biopsy ◽  
Abdominal Distension ◽  
Intravenous Fluids ◽  
Congenital Hyperinsulinism ◽  
Rare Association ◽  
Hirschprung's Disease ◽  
Hyperinsulinemic Hypoglycaemia

Hypoglycaemic due to congenital hyperinsulinism in Beckwith-Wiedemann syndrome is commonly seen. It is usually transient and is managed by enteral feeds, high glucose-containing intravenous fluids and medications like diazoxide. We describe a case of an infant with genetically proven Beckwith-Wiedemann syndrome with prolonged hyperinsulinemic hypoglycaemia. Despite treatment with high glucose-containing intravenous fluids, diazoxide and octreotide, her hypoglycaemia persisted. In addition to this, she also developed features of intestinal obstruction, which further complicated the management of hypoglycaemia. She underwent a rectal biopsy for this, which was highly suggestive of Hirschprung’s disease. Following surgery, her abdominal distension and feed intolerance were settled and sugar control was improved. We present a rare association of Hirschsprung’s disease with Beckwith-Wiedemann syndrome. To the best of our knowledge, this association has not been previously reported and this added to the difficulty in managing hyperinsulinemic hypoglycaemia in our patient.

scholarly journals A Rare Case Report on Hirschsprung’s Disease

2021 ◽  
pp. 132-136
Author(s):  
Pratibha Wankhede ◽  
Achal Gulghane ◽  
Mayur Wanjari
Keyword(s):  
Abdominal Pain ◽  
Blood Test ◽  
Rare Case ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Failure To Thrive ◽  
Clinical Findings ◽  
Appropriate Treatment ◽  
Rare Case Report ◽  
Rbc Count

Introduction: Hirschsprung’s disease is a condition that affects the large intestine and causes problems passing stool. It was first identified by “Ruysch” in 1961 and popularized by Hirschsprung’s in 1886. A missing nerve cell in the baby's colon muscles causes the condition, which is present at birth. Clinical Findings: Difficulty in passing stool, Abdominal pain, fever, (Temperature 100-degree f) Vomiting, Failure to thrive, poor feeding. Diagnostic Evaluation: CBC Infestation, Blood test, Hb -10.4 gm%, Total RBC Count -4.37 million /cu mm, RDW-13.3%, HCT-30.5%, Total WBC Cont-4000 /cu mm, Monocytes- 13%, Granulocytes-65%, Lymphocytes-30%, AST (SGOT)-28 U/L. Therapeutic Intervention: Blood Transfusion, Inj. Aminoven 200 mg BD, Cefotaxime 400 mg BD IV, Inj. Amikacin 130 mg OD, Inj. Metrogyl 90 mg BD, Inj. Pantocid 10 mg OD. Outcome: After Treatment the child show improvement in his passing stool frequently and relived Vomiting, fever, and increases feeding pattern. Conclusion: My patient was admitted to pediatric ward no 22 at AVBRH with a known case of Hirschsprung's Disease, and he had complained of difficulty in passing stool, at birth after getting appropriate treatment and surgery his condition was improving.

Diagnosis of Hirschsprung's Disease: Accuracy of Barium Enema Findings

1997 ◽  
Vol 36 (4) ◽  
pp. 631
Author(s):  
Sue Yun Yu ◽  
Gye Yeon Lim ◽  
Ji Yeong Yun ◽  
Seong Tae Hahn ◽  
Hak Hee Kim ◽  
...  
Keyword(s):  
Barium Enema ◽  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease
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