Image of the Month: Decision-Making in Surgery for Late Onset Hirschsprung Disease

Apte, Anisha; McKenna, Elise; Levitt, Marc A.

doi:10.1055/s-0040-1721049

Cited by 4 publications

(3 citation statements)

References 5 publications

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“…Published by Universitas Diponegoro. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/) Available online at: https://ejournal2.undip.ac.id/index.php/dimj 34 Symptoms in older children are more subtle, it usually comes with various and more complex complications, like Hirschsprung-associated enterocolitis, chronic constipation, failure to thrive, or more 5 . However, short and long-term complications remain a considerably serious problem.…”

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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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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“…With total colonic HD a diverting ileostomy is the initial choice of treatment. [40][41][42] Another component of the pre-surgical management is appropriate treatment of HAEC. It includes rectal irrigations and antibiotics although surgical evaluation should be considered if symptoms are severe or do not improve with medical management.…”

Section: Pre-surg Ic Al Manag Ementmentioning

confidence: 99%

“…These types of patients most likely will need a two‐stage approach with an initial ostomy diversion and a second‐stage pull‐through. With total colonic HD a diverting ileostomy is the initial choice of treatment 40–42 …”

Section: Pre‐surgical Managementmentioning

confidence: 99%

Hirschsprung's disease. Management

Morera,

Nurko

2024

Aliment Pharmacol Ther

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SummaryBackgroundHirschsprung's disease (HD) is a rare congenital disease that is characterised by the absence of ganglion cells in the myenteric plexus starting in the distal bowel. This results in distal functional obstruction and may lead to complications like enterocolitis. The treatment is surgical and requires the resection of the aganglionic segment, and the pull‐through of normal intestine into the anal opening. However, even after successful surgery, patients may continue to have symptoms.AimDiscuss current surgical techniques and management strategies for patients with postoperative symptoms after surgical correction of Hirschsprung's disease.MethodsA review of the literature was done through PubMed, with a focus on clinical management and approach.ResultsWe describe the clinical problems that can occur after surgical correction. These include obstructive symptoms, enterocolitis, or faecal incontinence. A systematic approach for the evaluation of these patients includes the exclusion of anatomic, inflammatory, behavioural or motility related factors. Depending on the severity of the symptoms, the evaluation includes examination under anaesthesia, the performance of contrast studies, endoscopic studies, measurement of anal sphincter function and colonic motility studies. The treatment is focused towards addressing the different pathophysiological mechanisms, and may include medical management, botulinum toxin to the anal sphincter or rarely redo‐operation.ConclusionsPatients with Hirschsprung's disease need to have surgical correction, and their postoperative long‐term management is complex given a variety of associated problems that can occur after surgery. A systematic evaluation is necessary to provide appropriate therapy.

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