Fiona M. Lewis Abnormalities of the vulva in infants may be the result of developmental defects, and these are usually present at birth (see Chapter 1). Acquired anatomical changes occur later in childhood, and hymenal abnormalities may not present until puberty. Common problems such as labial adhesions will often resolve without treatment, whereas others may need more complex surgery. This is a common problem in young girls. The prevalence of labial adhesions is estimated at up to 5% girls [1], and most occur before the age of 2 years. It is generally thought that the low oestrogen state at this age coupled with minor inflammation and irritation leads to adhesion of the labia. Often there are few symptoms, but there may be soreness from an irritant dermatitis. Urinary symptoms including recurrent urinary tract infections, dribbling, and frequency may be the presenting feature. The adhesions occur in varying degrees from the clitoris to the posterior fourchette (Figure 51.1). Partial adhesion is more common posteriorly. The major differential diagnosis is lichen sclerosus (LS) as labial adhesions can occur in LS, but other features of the disease must be present to make this diagnosis, and they are more common in older children. In younger children, congenital abnormalities can occur, but a useful distinguishing sign is a line of demarcation between the clitoral hood and the labia minora seen with simple labial adhesions [2]. If urine pools posteriorly behind the fusion, this can lead to incontinence where the pooled urine leaks onto the skin after micturition. As the skin is then constantly wet, irritant dermatitis is a common problem. Recurrent urinary tract infections must be treated as these can potentially lead to long‐term renal disease. They occur in over 50% of children and are more common in those with complete and thick adhesions [3]. If asymptomatic, the parents can be reassured and the problem simply observed. In those who are symptomatic, topical oestrogens applied once or twice a day for 2–6 weeks are very helpful. Topical 0.05% betamethasone has also been used, and in one study, the results with this preparation and topical oestrogens were similar, with over 80% responding well [4]. Betamethasone may lead to a more rapid resolution with fewer side effects [5]. Surgery is reserved for those who fail to respond to medical treatment and who continue to be symptomatic. There is the potential for scarring, and if surgery is undertaken as a last resort, topical oestrogens must be applied to prevent recurrence, as this is common post‐operatively. The vast majority resolve with simple treatment, but recurrence is common and treatment may need to be repeated. This entity was first described in 1996 [6] as oedematous protrusions anterior to the anus. In the series reported, they are more frequent in female infants. Fourteen of the 15 children in the original series were girls [6].
51
Anatomical Abnormalities in Children
Labial adhesions (labial agglutination)
Epidemiology
Pathophysiology
Clinical features
Differential diagnosis
Complications
Treatment
Prognosis
Infantile pyramidal perineal protrusion
Epidemiology
Pathophysiology