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Incontinentia pigmenti with intracranial arachnoid cyst: A case report

Wen-Chao Li1,2, Man-Li Li3, Jiang-Wei Ding1

  • 1Department of Neurosurgery, General Hospital of Ningxia Medical University, Yinchuan 750000, Ningxia Hui Autonomous Region, China.

World Journal of Clinical Cases
|September 26, 2022
PubMed

Insights

Incontinentia pigmenti (IP) is a rare genetic disorder affecting multiple systems. This case highlights a rare complication of IP with an intracranial arachnoid cyst (IAC), emphasizing the need for comprehensive diagnosis.

Area of Science:

  • Genetics
  • Neurology
  • Dermatology

Background:

  • Incontinentia pigmenti (IP) is a rare X-linked dominant disorder affecting multiple organ systems, including skin, eyes, teeth, and the central nervous system.
  • While IP commonly presents with characteristic skin lesions, neurological complications can significantly impact patient prognosis.
  • Intracranial arachnoid cysts (IACs) are rare, and their association with IP has not been previously reported.

Observation:

  • An 11-year-old female patient presented with a five-month history of intermittent limb convulsions, initially diagnosed as primary epilepsy.
  • Comprehensive evaluation, including medical history, physical examination, and imaging, revealed IP with intracranial space-occupying lesions leading to secondary epilepsy.
  • Surgical intervention via craniotomy was performed, with postoperative pathology confirming an intracranial arachnoid cyst (IAC).

Findings:

  • The patient experienced a favorable outcome after craniotomy, with no significant surgery-related complications.
  • Post-surgery, the patient remained free of recurrent epilepsy symptoms during the follow-up period.
  • This case represents the first reported instance of Incontinentia pigmenti complicated by an intracranial arachnoid cyst.

Implications:

  • This case underscores the importance of considering diverse neurological complications in patients with Incontinentia pigmenti.
  • Early diagnosis and management of associated intracranial pathologies like IACs are crucial for improving patient outcomes.
  • The findings contribute to a better understanding of the multi-systemic nature of IP and its potential neurological manifestations.
Abstract

Keywords:
Case reportCraniotomyIncontinentia pigmentiIntracranial space-occupying lesionsX-linked

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