Literature DB >> 23426836

A case of recurrent labial adhesions in a 15-month-old child with asymptomatic non-classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Dominika Janus1, Malgorzata Wojcik, Ewa Malunowicz, Jerzy B Starzyk.   

Abstract

Labial adhesions (synechia vulvae) are a relatively common disorder of the external genitalia in prepubertal girls. They usually occur between 3 months and 6 years of age, with a peak between 13 and 23 months of life. In the majority of cases, labial adhesions are asymptomatic and noticed by the parents or a physician during a routine physical examination. Frequently, they cause recurrent urinary tract infections. This report presents the case of a 15-month-old girl with recurrent labial adhesions and urinary tract infections. She was followed-up by a urologist from the age of 3 months, treated topically (estriol cream 1 mg/g and emollients) and surgically. No signs of androgenization were seen apart from advanced bone age. Urinary steroid profile and molecular analysis confirmed the diagnosis of non-classic congenital adrenal hyperplasia (NCCAH) due to 21-hydroxylase deficiency. In the described case, a coincidence of NCCAH and labial adhesion cannot be excluded particularly in view of the resolving of urinary tract infections at the same time as resolving of labial adhesions. However, it seems that in cases of recurrent labial adhesion/synechia that require repeated surgical interventions in view of ineffectiveness of conservative treatment - as it was observed in the presented case - one should consider searching for NCCAH. This may allow not only for the causal treatment of labial adhesions but, above all, NCCAH and, in consequence, the prevention of intensification of virilization, hirsutism, menstrual cycle, and fertility disturbances that are observed starting from puberty.

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Year:  2012        PMID: 23426836     DOI: 10.1515/jpem-2012-0190

Source DB:  PubMed          Journal:  J Pediatr Endocrinol Metab        ISSN: 0334-018X            Impact factor:   1.634


  2 in total

Review 1.  Non-Classical Congenital Adrenal Hyperplasia in Childhood.

Authors:  Selim Kurtoğlu; Nihal Hatipoğlu
Journal:  J Clin Res Pediatr Endocrinol       Date:  2016-06-29

2.  Labial Fusion Recurrence in a Prepubertal Girl: A Case Report and Review of Literature.

Authors:  Ipsita Mohapatra; Subha R Samantaray
Journal:  Cureus       Date:  2022-06-18
  2 in total

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