Incontinentia pigmenti with intracranial arachnoid cyst: A case report

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 surgeryrelated 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.

The supraorbital keyhole approach with eyebrow incisions for treating lesions in the anterior fossa and sellar region

Background Keyhole surgery has developed since the 1990s as a less invasive therapeutic strategy for intracranial lesions, initially for the treatment of intracranial aneurysms. The purpose of this study was to describe and evaluate the results of surgical treatment of lesions in the anterior fossa and sellar region via a supraorbital keyhole approach using eyebrow incisions. Methods Between April 1994 and July 2003, 54 patients with lesions in the anterior fossa and sellar region were operated on via the supraorbital keyhole approach. The surgical results were studied retrospectively and compared with that of patients with lesions at the same locations but treated via a conventional subfrontal approach.Results No significant difference in curative effect was found between the conventional subfrontal approach and the supraorbital keyhole approach. However, the supraorbital approach required a much smaller skin incision, causing less surgical trauma, while achieving excellent surgical exposure and good recovery. Conclusion The supraorbital keyhole approach using an eyebrow incision is safe, effective, and both suitable and convenient for treating lesions in the anterior fossa and sellar region, with almost no adverse consequences on the facial features of patients.

Clinical features and treatment outcomes of human immunodeficiency virus-associated cryptococcal meningitis:a 2-year retrospective analysis

Background:Cryptococcal meningitis(CM)is one of the most common opportunistic infections caused by Cryptococcus neoformans in human immunodeficiency virus(HIV)-infected patients,and is complicated with significant morbidity and mortality.This study retrospectively analyzed the clinical features,characteristics,treatment,and outcomes of first-diagnosed HIV-associated CM after 2-years of follow-up.Methods:Data from all patients(n=101)of HIV-associated CM hospitalized in Shanghai Public Health Clinical Center from September 2013 to December 2016 were collected and analyzed using logistic regression to identify clinical and microbiological factors associated with mortality.Results:Of the 101 patients,86/99(86.9%)of patients had CD4 count<50 cells/mm^3,57/101(56.4%)were diagnosed at≥14 days from the onset to diagnosis,42/99(42.4%)had normal cerebrospinal fluid(CSF)cell counts and biochemical examination,30/101(29.7%)had concomitant Pneumocystis(carinii)jiroveci pneumonia(PCP)on admission and 37/92(40.2%)were complicated with cryptococcal pneumonia,50/74(67.6%)had abnormalities shown on intracranial imaging,amongst whom 24/50(48.0%)had more than one lesion.The median time to negative CSF Indian ink staining was 8.50 months(interquartile range,3.25-12.00 months).Patients who initiated antiretroviral therapy(ART)before admission had a shorter time to negative CSF Indian ink compared with ART-naïve patients(7 vs.12 months,χ^2=15.53,P<0.001).All-cause mortality at 2 weeks,8 weeks,and 2 years was 10.1%(10/99),18.9%(18/95),and 20.7%(19/92),respectively.Coinfection with PCP on admission(adjusted odds ratio[AOR],3.933;95%confidence interval[CI],1.166-13.269,P=0.027)and altered mental status(AOR,9.574;95%CI,2.548-35.974,P=0.001)were associated with higher mortality at 8 weeks.Conclusion:This study described the clinical features and outcomes of first diagnosed HIV-associated CM with 2-year follow-up data.Altered mental status and coinfection with PCP predicted mortality in HIV-associated CM.