Langerhans cell histiocytosis in children: A retrospective case series of 126 cases.

BACKGROUND/OBJECTIVES: Langerhans cell histiocytosis (LCH), a rare neoplasm of hematopoietic myeloid precursor cells, is clinically characterized by spontaneously resolving lesions to a progressive life-threatening multisystem disorder. Diagnosing LCH in children is challenging as it mimics other skin disorders. This study describes the varied clinical presentation and disease course in children less than 18 years diagnosed with LCH. METHODS: We performed a retrospective observational study of all cases diagnosed with LCH presenting to a children's hospital in the last 26 years. Data on history, cutaneous and systemic examination, and laboratory evaluation performed, were recorded. RESULTS: A total of 126 children diagnosed with LCH were included in the study. There were 68% cases limited only to skin, and 32% children with multisystem involvement at the initial presentation. Scaly papules were the most common morphologic finding in skin. The skeletal system was the second most common organ system to be affected. Failure to thrive was a common symptom. Progression of skin to systemic involvement was seen in 27.9%. In 76.7%, skin lesions cleared over a period of 2 to 4 years. Complete remission was seen in 56.9% of children over a period of 3 to 7 years, while 8.1% children died of complicationsand 31.8% were lost to follow-up. CONCLUSIONS: Long-term follow-up in this study has shown cutaneous LCH without systemic involvement has a good prognosis. Skin involvement,along with failure to thrive, was the most common clinical presentation in our study. The skeletal system was the second most common organ system involved.

Fraser syndrome with partial anomalous pulmonary venous connection.

Fraser syndrome is characterized by cryptophthalmos, cutaneous syndactyly, malformations of the larynx and genitourinary tract, craniofacial dysmorphism, orofacial clefting, mental retardation, and musculoskeletal anomalies. We report a case of a two day old neonate who presented with features suggestive of the diagnosis of Fraser syndrome. This child also had partial anomalous pulmonary venous connection and congenital hypo-thyroidism.

Diagnostic Dilemma in a 15-Year-Old Boy with Recurrent Pneumococcal Meningitis.

Recurrent bacterial meningitis is very rare phenomenon in paediatric age group. Finding the aetiology of recurrent meningitis often poses diagnostic challenge to clinicians. Among the several aetiologies, structural deficiencies at the base of skull, congenital or acquired, are readily correctable ones and hence it's imperative to find out the location of the gap in order to surgically repair it. We report the diagnostic dilemma faced while managing a 15-year-old boy with recurrent pneumococcal meningitis. Aetiology could only be found after exclusion of immunodeficiency and performing a series of imaging studies. CT cisternogram clinched the diagnosis and patient was cured successfully. A single imaging modality, be it CT/MRI scan, although proven to be better than others according to literature, might not be sufficient while finding the cause of recurrent bacterial meningitis in an immunocompetent host.