The prevalence of LA in the current study was 1.7%, aligning with the existing literature [1, 5]. Since most of the Indian population lives in villages, it was reflected in the study too, where close to 70% of patients belonged to the rural background. The patients whose presentation was delayed may be due to this reason. The health facilities are limited in the villages [6, 7]. The transport facilities are also inadequate. It leads to delayed diagnosis and travel problems compound the situation, thus delaying the evaluation and management at an expert center.
The attendants had a common complaint of the absent vaginal opening. The attendants were very apprehensive of the possibility of vaginal absence. The possibility of an absent vagina may lead to a feeling of distortions of body image and psychological distress. It may lead to anxiety and depression [8]. The pediatric surgical facilities are limited to less than 50% of districts of India [9]. Besides, the training centers are limited [10]; hence, many clinicians do not have exposure to pediatric surgical clinical situations. It can be gauged that some patients underwent unnecessary USG abdomen to look for internal genitalia based on suspicion of vaginal atresia. The other clinical problems may be UTI, abnormal urinary stream, recurrent vulvar or vaginal infections, and post-void dripping. Rarely, hydronephrosis secondary to urinary retention has also been reported [2, 4, 11].
LA is not a congenital anomaly, and its exact cause is not clear [12]. It has been suggested that chronic inflammation from infection, poor perineal hygiene, or trauma can erode the epithelium of the labia minora. In association with minimal estrogen in prepubertal females, it may facilitate adhesion of the labia minora [11, 13]. However, in one study, serum estradiol levels of 59 prepubertal females with LA and 60 prepubertal females showed no statistically significant difference in the estrogen levels [1]. In the current series, poor perineal hygiene was noted in 65 (43.33%) patients. It is not clear whether UTI was secondary to LA or its precursor.
Our management strategy differs from what is mentioned in the literature. Most of the literature is from the USA or European countries [2, 3, 5, 12, 14]. The preferred choice of management is either observation or estrogen/steroid cream and follow-up in the OPD. As mentioned earlier about factors, such as monetary or transportation issues, the attendants do not wish to visit the OPD unless deemed extremely necessary. Hence, the options of application of an estrogen or steroid cream with follow-up become a tad difficult. Besides, it is not necessary that the attendants will come on the next scheduled visit. They may keep on applying the cream till then. There are side effects of estrogen cream mentioned, such as hyperpigmentation of the labia and breast development. Besides, there can be a possibility of vaginal bleeding and precocious puberty [2, 5]. Moreover, the success rate of applying estrogen or betamethasone cream is close to 70% and 80%, respectively [2], although some recent reports have better responses [13].
Manual separation is an office procedure. When explained, all of the attendants chose manual separation. The idea of observation was rejected as they wanted immediate treatment due to the apprehension, as discussed earlier. The majority of patients fared well with the application of an antibiotic ointment after manual release, and we did not need estrogen cream after manual release as recommended [13]. It has been found to be more effective than estrogen cream alone [15].
The incidence of recurrence has wide variations from 7 to 55% [13]. The treatment modality in these series is not clear. Our recurrence incidence was 6.6%, which is less than the minimum range reported. It is probably due to the procedure of manual release. The patients who had recurrence did not follow the advice properly, and the perineal hygiene was not good. It may be the reason for the recurrence. However, after re-release, no further recurrence was noted. An uncommon cause of recurrence, such as congenital adrenal hyperplasia, may be looked at if there are multiple recurrences [16]. The limitation of this study can be its retrospective nature; however, we believe that the detailed analysis of data may compensate for this deficit.
