Open Journal of Obstetrics and Gynecology, 2012, 2, 382-384 OJOG http://dx.doi.org/10.4236/ojog.2012.24078 Published Online November 2012 (http://www.SciRP.org/journal/ojog/) A review of conditions altering the permanent appearance of the vulva Ian S. C. Jones1,2 1Women’s and Newborn Services, Royal Brisbane and Women’s Hospital, Herston, Australia 2University of Queensland, Brisbane, Australia Email: [email protected] Received 16 August 2012; revised 14 September 2012; accepted 23 September 2012 ABSTRACT and performing an appropriate, thorough and sensitive examination can not be over emphasised. An appropriate This article is aimed at providing information on genital examination may require examination under gen- variations in the clinical appearance of the vulva. The eral anaesthesia. appearance of the vulva can be altered by reversible Of equal importance and before considering abnormal or permanent conditions both of which may result in conditions normal variants need to be recognised. The minor or major changes. Reversible conditions in- range of normal appearance for the vulva and lower va- clude those associated with infections or acute trauma which results in distortion of the vulva. Some perma- gina is large. Such changes affect the mons pubis, labia, nent changes are caused by life threatening conditions clitoris, vestibule and hymen. The size of these structures which are present at birth whereas others develop varies with age, ethnicity and parity. A degree of asym- more slowly or as the result of a deliberate act either metry is common and usually of no significance but traditional female surgery or surgery performed by a asymmetry of the labia majora has been a presenting sign registered medical practitioner. To the inexperienced in a case of neurofibromatosis [1] hence each case needs practitioner changes from the normal vulvar ap- to be carefully assessed. pearance can be confusing. The aim of this article is to highlight and categorise changes that can affect the 2. CATEGORISATION appearance of the vulva. Whatever the presentation Conditions that may cause reversible changes to the ap- the importance of obtaining a detailed history and pearance of the vulvar include those associated with in- performing an appropriate, sensitive and thorough fections, local reaction to drugs and chemicals and examination can not be over emphasised. trauma, all of which may distort the vulva until resolved naturally or by treatment. Although changes to the ap- Keywords: Vulvar Appearance; Congenital; Traumatic; pearance of the vulva occur as a result of tumours and Cultural; Iatrogenic ulcers treatment usually corrects such changes and so this group of conditions has been omitted from further 1. INTRODUCTION study. It is recognised however that sometimes perma- nent scarring may result following these conditions. Variations in the clinical appearance of the vulvar can Causes of permanent changes to the vulva can be cause embarrassment for the woman and her physician. categorised as congenital, traumatic, cultural and iatro- This article is aimed at providing information on varia- genic. In turn each of these categories can be further tions in the clinical appearance of the vulva. It is impor- subdivided (Table 1). tant to be sensitive to the feelings of the woman (or child) and her family when discussing variations in anatomy 3. CONGENITAL irrespective of the cause of such change. Allowing the woman to indicate her own preference for describing The complex congenital abnormalities of bladder ex- how she sees her external genitalia is recommended. The strophy and cloacal exstrophy have a major affect on the need to enter the debate as to why any deliberate change, appearance of the vulva. Bladder exstrophy involves an either minor or major has been made to the appearance absence of the lower central anterior abdominal wall of the vulva should be resisted. with the inside of the bladder and the urethra exposed Whenever a female of any age presents with a vulvar onto the surface of the lower abdomen. There is diastasis condition the importance of obtaining a detailed history of the symphysis pubis, the clitoris is separated into two OPEN ACCESS I. S. C. Jones / Open Journal of Obstetrics and Gynecology 2 (2012) 382-384 383 Table 1. Categories and subcategories for causes of permanent hematocolpus, hematometra, retrograde menstruation variations in appearance. and acute urinary retention. Examination shows a bulge in the vestibule. Treatment is by surgical division of the Congenital Traumatic Cultural Iatrogenic thickened hymen thereby releasing the retained men- Exstrophy of Traditional Sexual strual blood and relieving the introital distension. bladder or Obstetric female reassignment cloacal The appearance of the vulva where a female fetus is surgery surgery exstrophy exposed to raise androgen levels depends on when dur- ing intrauterine life this occurs. Early exposure results in Reconstruction Non Double vulva of congenital complete external virilisation [5] with lesser effects re- obstetric abnormalities sulting from lower androgen levels. Classifications of Reconstruction ambiguous external genitalia are based on anatomical Clitoromegaly following radical and aetiological mechanisms. Patients with pure gonadal cancer surgery dysgenesis have a small phallus, poorly developed labia Labial majora and fusion of the labioscrotal folds. With true hypertrophy hermaphrodites the appearance of the external genitalia Imperforate varies considerably but most are raised as males because hymen of the size of the phallus. Female pseudohermaphrodites Intersex have an enlarged clitoris, variable degrees of labial fu- Inguinal sion and the urethral opening may not be distinct from herniation the vagina [6]. Whatever ambiguity is found consultation Vascular with experts in this area of medicine is recommended. Vascular tumours include benign hemangiomas and halves (bifid clitoris) and the labia minora displaced lat- angiokeratomas and malignant epithelioid hemangioen- erally [2]. dothelioma and angiosarcoma. Tumors of lymphatic ori- Cloacal exstrophy has bladder exstrophy and large in- gin such as lymphangiomas are also described. Heman- testine presenting through the anterior abdominal wall giomas usually occur in infancy and childhood and are defect, anal atresia, anomalous genitalia and an ompha- usually small. Varicose veins of the vulva are more often locele. found in women with varicose veins in the legs. Vulval Surgical repair of either bladder or cloacal exstrophy varicosities occur in four percent of women [7] and are results in considerable scaring of the anterior abdominal common during pregnancy. In severe cases surgical wall and an improved but not entirely normal appearance management may be required. of the vulvar. An indirect inguinal hernia may distort the vulvar in The finding of a double vulvar is extremely rare and is the upper labia majora due to persistence of the equiva- lent of the male processus vaginalis. A mesothelial cyst associated with duplication of other portions of the re- anywhere along the line of the round ligament as it productive tract, urethra, bladder and colon but with passes through the inguinal canal to its insertion in the normal ovaries and kidneys [3]. The presence of clite- upper labia majora is called a cyst in the canal of Nuck. romegaly suggests excess male hormone either endoge- Such cysts and hydroceles can if large enough cause a nous or exogenous however the possibility of life threat- distortion in the labia majora. ening congenital adrenal hyperplasia either the acute type seen in the newborn or the non-classic type seen in later 4. TRAUMATIC life, must be seriously considered. The defective conver- sion of 17-hydroxyprogesterone to 11-deoxycortisol is Obstetric trauma can cause minor or severe injury to any the cause of congenital adrenal hyperplasia. In the new- part of the vulvar. Severe injury results in tears of differ- born there is ambiguity of the female genitalia whereas at ent shapes and size to labia, clitoris, perineum, urethra, older age acne, hirsutism (including cliteromegaly, ru- anal sphincter and anus. Blood loss can be life threaten- gated and partly fused labia majora and a common uro- ing. Surgical repair of these injuries can distort the ap- genital sinus) and irregular menstruation are presenting pearance of the vulvar. Not repairing transverse tears in symptoms and signs [4]. the labia minora can result in excessively mobile skin Labial hypertrophy or marked asymmetry of the labia which can cause pain or discomfort. minora can be a normal variant that may cause discom- Non obstetric trauma may result in haematoma forma- fort during coitus, walking or sitting down. Being able to tion, lacerations, tears or a combination of the three. show clinical photographs of such variation assists the Mechanisms of injury include falling astride a firm ob- woman to understand this issue. ject, consensual coitus, sexual assault, physical assault, An imperforate hymen may lead to cryptomenorrhoea, burns and waxing. Genital piercings and shaving can also Copyright © 2012 SciRes. OPEN ACCESS 384 I. S. C. Jones / Open Journal of Obstetrics and Gynecology 2 (2012) 382-384 cause injury and infection. 7. DISCUSSION 5. CULTURAL At times physicians are required to examine women with genital piercings, genital tattoos and female circumcision. Within cultures of those who practice traditional female The need to enter the debate as to why any deliberate surgery phrases like cutting, ritual female surgery and change, either minor or major has been made to the ap- female circumcision are more acceptable than female pearance of the vulvar should be resisted. Knowledge of genital mutilation (FMG) which is the term used in both normal and altered vulvar anatomy assists the prac- Western societies. The basis for the World Health Or- titioner to manage difference and provide the best care ganisation classification of FMG is the amount of vulvar for each patient. tissue removed [8]. The more tissue removed increases This article highlights and categorises physical changes the degree of distortion of the appearance of the vulva. In to the anatomy of the vulvar.