What is the clinical significance and management approach for ovarian inclusion cysts in female patients with Hirschsprung's disease?

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No Established Clinical Correlation Between Hirschsprung Disease and Ovarian Inclusion Cysts

There is no documented clinical association or correlation between Hirschsprung disease and ovarian inclusion cysts in the medical literature. These are distinct, unrelated pathological entities that occur through completely different mechanisms and affect different organ systems 1, 2, 3.

Understanding Each Condition Separately

Hirschsprung Disease

  • Hirschsprung disease is a congenital intestinal disorder caused by failed migration of ganglion cells during gestation, resulting in functional colonic obstruction 1, 4.
  • The disease affects 1 in 5,000 live births and typically presents in the neonatal period with delayed meconium passage beyond 24 hours, abdominal distension, and vomiting 1.
  • Approximately 94% of patients are diagnosed before age five, though rare adult presentations occur with chronic constipation 5.
  • The condition is associated with genetic syndromes including trisomy 21, Mowat-Wilson syndrome, and Shah-Waardenburg syndrome, but not with gynecologic pathology 1.

Peritoneal Inclusion Cysts (Ovarian-Related)

  • Peritoneal inclusion cysts are benign cystic masses that form adjacent to or surrounding a functioning ovary, taking the shape of underlying pelvic structures 6.
  • These cysts are classified as O-RADS 2 (almost certainly benign, <1% malignancy risk) when they demonstrate typical features 6.
  • On ultrasound, they follow the contour of adjacent pelvic organs without exerting mass effect and typically contain septations, with the ovary either at the margin or suspended within the lesion 6.
  • Management by a gynecologist is recommended for typical peritoneal inclusion cysts of any size 6.

Clinical Management Approach

If Both Conditions Coincidentally Present

  • Manage each condition independently according to established protocols for that specific disease 1, 4, 6.
  • For Hirschsprung disease: Surgical removal of aganglionic bowel with intestinal reconstruction (Swenson, Soave, or Duhamel procedures) 1.
  • For peritoneal inclusion cysts: Gynecologic consultation with imaging surveillance or surgical management based on O-RADS classification 6.

Diagnostic Pitfall to Avoid

  • Do not assume any pathophysiologic connection between abdominal distension from Hirschsprung disease and incidentally discovered pelvic cystic masses 1, 3.
  • Cystic pelvic masses must be distinguished from ovarian versus nonovarian origin by identifying normal ovaries separately at imaging 3.
  • In female patients with Hirschsprung disease undergoing abdominal imaging, any pelvic cystic findings should be evaluated using standard gynecologic imaging protocols with transvaginal ultrasound and O-RADS classification 6, 7.

References

Research

Hirschsprung disease.

Nature reviews. Disease primers, 2023

Research

Nonovarian cystic lesions of the pelvis.

Radiographics : a review publication of the Radiological Society of North America, Inc, 2010

Research

Hirschsprung's disease: diagnosis and management.

American family physician, 2006

Research

Hirschsprung's Disease: A Rare Adult Diagnosis.

Clinical practice and cases in emergency medicine, 2020

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Guideline

Ovarian Cyst Management Guideline

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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