Hirschsprung’s Disease in Adults Revealed by an Occlusive Syndrome

Soussan, Haitam; Jabi, Rachid; Ouryemchi, Mouad; Haddadi, Zakaria; Bouziane, Mohammed

doi:10.7759/cureus.18484

Cited by 6 publications

(5 citation statements)

References 14 publications

(26 reference statements)

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“…Abdominal CT scan showed dilated colon and intestine. Surgical treatment was done, and histological findings showed aganglionic part of sigmoid, thus confirming the diagnosis of Hirschprung’s disease [ 14 ].…”

Section: Discussionmentioning

confidence: 91%

Adult Hirschsprung’s disease presenting as chronic constipation: a case report

Rahardjo

Nurzaman²,

Natalia

et al. 2023

J Med Case Reports

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Background Hirschsprung’s disease is a congenital disorder identified by the absence of ganglion cells at the Meissner’s plexus of the submucosa and Auerbach’s plexus of the muscularis. This disease can be found in approximately 1 in 5000 live births. It is a congenital disorder that is rarely diagnosed in adults, where 95% of cases are diagnosed in infants aged under 1 year old. Here we present a rare case of adult Hirschsprung’s disease to enrich the body of knowledge in diagnosing adult patients with chronic refractory constipation symptoms. Case report An 18-year-old Indonesian woman came to the general surgery department of Unggul Karsa Medika Teaching Hospital with a defecating problem (constipation) since childhood. There was no history of her passage of meconium. A contrast enema study showed that the sigmoid colon was dilated and the rectum was narrowed, with rectosigmoid index < 1. With these findings, it was suspected that the patient may have ultra-short segment Hirschsprung’s disease. The patient was then referred to the digestive surgery department of referral hospital for surgical treatment. Conclusion In adult patients presenting with history of constipation since childhood, it is necessary to consider the possibility of Hirschsprung’s disease that was not diagnosed in early childhood. Hirschsprung’s disease in adults is usually a short or ultra-short aganglionic segment because it shows relatively mild symptoms. Surgical removal of the aganglionic segment of the gut is the definitive treatment for Hirschsprung’s disease.

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Section: Discussionmentioning

confidence: 91%

Adult Hirschsprung’s disease presenting as chronic constipation: a case report

Rahardjo

Nurzaman²,

Natalia

et al. 2023

J Med Case Reports

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“…Early diagnosis is crucial in preventing later complications. Late-onset hirschsprung's disease (in children of 2 years age or over) usually comes with a different presentation than in the newborn period 2,4 .…”

Section: Discussionmentioning

confidence: 99%

Malnutrition and Developmental Delay Presenting as Complications of Late Onset Hirschprung’s disease : A Case Report

Pujiati,

Raharjo,

Ovaditya

2023

Diponegoro. Int. Med. J.

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Background: Hirschsprung’s Disease is a congenital disorder characterized by the absence of ganglionic cells in the colon, causing loss of peristaltic movement in the bowel. Most cases are diagnosed before the age of one and marked by the delayed passage of meconium, vomiting, and distended abdomen. Here, we present a case of a 2-year-old boy with an atypical presentation of malnutrition and developmental delay as a complication of Hirschsprung’s Disease. Case Presentation: A 2-year-old boy who presented with abdominal distention and chronic constipation was referred to our hospital. Past personal history revealed that he frequently suffered from the symptoms but was previously diagnosed with regular constipation and only consumed symptomatic medication. On admission, his vital signs were all normal, but his appearance was pale and weak. Growth and developmental screening showed poor growth status and developmental delay in all four domains. From the physical examination, gross abdominal distention was found and the rectal examination demonstrated a tight anal sphincter, empty rectal vault, and a green-colored stool with no forceful expulsion. Radiologic and histopathological findings suggested Hirschsprung’s disease. Conclusion: Chronic abdominal distention followed by malnutrition and developmental delay can present as a complication of hirschsprung’s diseasein older children. Due to these subtle signs and symptoms, a thorough examination and proper diagnostic algorithm are

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“…En la fisiopatología de la EH hay una inervación anormal del intestino distal que comienza a nivel del esfínter interno, incluyéndolo, y se extiende en sentido proximal, en tramos variables (8) . Estas anomalías anatómicas de la inervación alteran la motilidad coordinadora del colon y el acto de la defecación, produciendo diversos grados de obstrucción funcional intestinal; así como obstrucción intestinal estructural no intrínseca por la formación de fecalomas (9) . Asimismo, la desnutrición y la falta del crecimiento pueden sumarse a la presentación en adolescentes con EH de segmento corto, diagnosticada más adelante en la vida (10) .…”

Section: Discussionunclassified

Obstrucción intestinal por fecaloma gigante en adolescente con enfermedad de Hirschsprung

Fernández-Ríos,

Aparicio-Curazi,

Altamirano-Farfan

et al. 2023

An Fac med

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Hirschsprung’s disease (HD) is uncommon, and most of the cases occur in neonates. It is rarely diagnosed during adolescence, and even fewer cases are documented. We present the case of a 16-year-old male who had 15 days of constipation, anorexia, postprandial vomiting and abdominal distension. Imaging examinations showed a massive fecaloma obstructing the colon, and histopathological examinations showed HD. Given the failure of conservative evacuation measures, an extended left hemicolectomy and colostomy were performed, plus appendectomy for bowel obstruction. The patient was discharged with antibiotic therapy. This case highlights the importance of considering HD in adolescents with chronic constipation and abdominal masses. Although patients may experience postoperative problems, surgery is the most effective treatment for HD.

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Hirschsprung’s Disease in Adults Revealed by an Occlusive Syndrome

Cited by 6 publications

References 14 publications

Adult Hirschsprung’s disease presenting as chronic constipation: a case report

Adult Hirschsprung’s disease presenting as chronic constipation: a case report

Malnutrition and Developmental Delay Presenting as Complications of Late Onset Hirschprung’s disease : A Case Report

Obstrucción intestinal por fecaloma gigante en adolescente con enfermedad de Hirschsprung

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