Li WC, Li ML, Ding JW, Wang L, Wang SR, Wang YY, Xiao LF, Sun T. Incontinentia pigmenti with intracranial arachnoid cyst: A case report. World J Clin Cases 2022; 10(23): 8352-8359 [PMID: 36159532 DOI: 10.12998/wjcc.v10.i23.8352]
Corresponding Author of This Article
Tao Sun, Doctor, Chief Doctor, Department of Neurosurgery, General Hospital of Ningxia Medical University, No. 804 Shengli South Street, Xingqing District, Yinchuan 750000, Ningxia Hui Autonomous Region, China. suntao_nxmu@163.com
Research Domain of This Article
Clinical Neurology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Aug 16, 2022; 10(23): 8352-8359 Published online Aug 16, 2022. doi: 10.12998/wjcc.v10.i23.8352
Incontinentia pigmenti with intracranial arachnoid cyst: A case report
Wen-Chao Li, Man-Li Li, Jiang-Wei Ding, Lei Wang, Shu-Ren Wang, Yang-Yang Wang, Li-Fei Xiao, Tao Sun
Wen-Chao Li, Jiang-Wei Ding, Lei Wang, Yang-Yang Wang, Li-Fei Xiao, Tao Sun, Department of Neurosurgery, General Hospital of Ningxia Medical University, Yinchuan 750000, Ningxia Hui Autonomous Region, China
Wen-Chao Li, Shu-Ren Wang, Department of Neurosurgery, The First Affiliated Hospital of Xinxiang Medical University, Xinxiang 453000, Henan Province, China
Man-Li Li, Department of Physiology, Sanquan College of Xinxiang Medical University, Xinxiang 453000, Henan Province, China
Author contributions: Li WC and Wang SR were the patient’s neurosurgeons, reviewed the literature and contributed to manuscript drafting; Ding JW and Li ML contributed to manuscript drafting; Xiao LF, Wang YY and Wang L analyzed and interpreted the imaging findings; Sun Tao was responsible for revision of the manuscript for important intellectual content; all authors have read and approved the final manuscript.
Supported bythe National Science Fund Subsidized Project, No. 81971085.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016). The manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Tao Sun, Doctor, Chief Doctor, Department of Neurosurgery, General Hospital of Ningxia Medical University, No. 804 Shengli South Street, Xingqing District, Yinchuan 750000, Ningxia Hui Autonomous Region, China. suntao_nxmu@163.com
Received: March 13, 2022 Peer-review started: March 13, 2022 First decision: June 16, 2022 Revised: June 25, 2022 Accepted: July 5, 2022 Article in press: July 5, 2022 Published online: August 16, 2022 Processing time: 140 Days and 23.6 Hours
Abstract
BACKGROUND
Incontinentia pigmenti (IP) is a rare X-linked dominant genetic disorder that can be fatal in male infants. It is a disease that affects many systems of the human body. In addition to characteristic skin changes, patients may also have pathological features of the eyes, teeth, and central nervous system. Therefore, the lesions in these systems may be the first symptoms for which patients seek treatment. To date, no cases of IP complicated by intracranial arachnoid cyst (IAC) have been reported. This paper aims to report a case of IP with IAC in order to share the diagnosis and treatment experience of this rare case with other clinicians.
CASE SUMMARY
An 11-year-old female patient suffered intermittent limb convulsions for five months and was sent to hospital. In the initial stage, the patient was considered to have primary epilepsy. Further investigation of the patient's medical history, physical examination and imaging examination led to the diagnosis of IP combined with intracranial space-occupying lesions, and secondary epilepsy. The patient was treated with craniotomy, and postoperative pathology revealed an IAC. The patient recovered well after craniotomy and had no obvious surgery-related complications. During the follow-up period, the patient did not have recurrent epilepsy symptoms.
CONCLUSION
IP is a multi-system disease that presents with typical skin lesions at birth, but the long-term prognosis of this disease depends on the involvement of systems other than the skin, especially nervous system and ocular lesions.
Core Tip: We report a rare case of incontinentia pigmenti (IP) with an intracranial arachnoid cyst (IAC). The initial diagnosis in this patient was considered to be primary epilepsy. Following admission, her medical history, physical examination and imaging examination led to the diagnosis of IP complicated by intracranial space-occupying lesions and was treated by craniotomy. Postoperative pathology revealed an IAC. During follow-up, the patient recovered well and did not experience recurrence of epilepsy. By recording the diagnosis and treatment history of this patient in detail, and reviewing relevant literature, discussing the characteristics of patients with IP and IAC will help to improve diagnosis and treatment, and improve the overall quality of life of such patients.
