I found this on a med students page. They even mention the new type of surgery and the uncertain outcome of new surgerie. http://home.coqui.net/titolugo/medical.htm#CAH CONGENITAL ADRENAL HYPERPLASIA: A Surgeon's Role by: Marideli López, Rafael S. Rodríguez, Frajam Farzam, Juan Pérez, MSIII, UCC School of Medicine Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder characterized by a deficiency in any of the five enzymatic steps involved in the synthesis of cortisol. About 90-95% of cases reported involve 21-hydroxylase as the deficient enzyme, followed by 11-B-hydroxylase dehydrogenase, 17-A-hydroxylase, and cholesterol desmolase. Due to lack of these enzymes there is an underproduction of cortisol that causes an overproduction of ACTH by negative feedback mechanism. Affected children with CAH have been exposed to excessive levels of androgens during the prenatal period producing phenotypic variations that range from mild clitoral enlargement, hirsutism, scrotalization of the labial folds, vaginal atresia and complete masculinization in females to prostatic hypertrophy, pubic and axillary hair, increased penis size, and cryptorchidism in the male. If this condition is suspected in the prenatal period through amniocentesis, dexamethasone therapy is administered to the mother. After birth conditions refractory to medical treatment needs surgical intervention. Clitoral recesion, practiced in the 1950's is no longer recommended because of the importance of the clitoris for orgasm and normal sexual function, therefore all attempts to preserve all or part of the clitoris with its nerve supply should be made. Surgical management considered ideal consists of a clitoral recession at three to six months of age followed by flap vaginoplasty at two to four years of age. The reason for delaying vaginoplasty is to allow sufficient growth of the vaginal and perineal tissue, making injury to the external sphincter muscle less likely. Recent experience indicates that early one-stage reconstruction produces more favorable cosmetic and functional outcomes, with less incidence of postoperative vaginal stenosis. It must be emphasized, that long term follow-up of patients following one stage procedure is needed to asses its true efficacy, since vaginal pull through is a technically demanding operation that poses a high risk of injury to the urethra. In conclusion, management of children with CAH must involve a multi disciplinary approach involving pediatric endocrinology, neonatology and surgery. The ideal surgical management for CAH patients taking into account the different variations and the rarity of these anomalies will depend mostly on the long-term follow-up of cases to asses the functional outcome of the surgical procedure performed. References 1- New MI: Genetic Disorders of Adrenal Hormone Synthesis. Horm Res 37:22-33, 1992 2- Donahue PK, Gustafson M: Early One Stage Surgical Reconstruction of Extremely High Vagina in Patients with Congenital Adrenal Hyperplasia. J Pediatr Surg 29(2): 352-358, 1994 3- Coran AG, Polley JR: Surgical Management of Ambiguous Genitalia in the Infant and Child. J Pediatr Surg 26(7): 812-820, 1991 4- Bhatia V, Shukla R, Mishra SK, et al: Adrenal Tumor Complicating Untreated 21-hydroxylase Deficiency in a 5.5 year old boy: Arch Pediatr Adoles Med 147: 1321-1323, 1993 5- Srikanth MS, West BR, Is***ani M, et al: Benign Testicular Tumors in Children with Congenital Adrenal Hyperplasia. J Pediatr Surg 27(5): 634-641, 1992 6- Hurtig AL, Radhakrishnan J, Reyes, HM, et al: Psychological Evaluation of Treated Females with Virilizing Congenital Adrenal Hyperplasia. J Pediatr Surg 18(6): 887-893, 1983 7- Vanderkamp JJ, Slijper ME, Brandenburg JJ, et al: Evaluation of Young Women with Congenital Adrenal Hyperplasia. Horm Res 37: 45-49, 1992 8- Siebemann RE: Disorder of Steroid Hormone Biosynthesis. Progress Pediatr Surg 16: 171-183, 1983