TÇCD 2011 29. National Pediatric Surgery Congress and 27. National Congress of The Egyptian Pediatric Surgical Association

Background:

In recent years, laparoscopy has gained acceptance as a useful method in management of intersex states. The indications for laparoscopy in such conditions are still evolving.

 

Materials and Methods:

            Laparoscopic operations were performed for 97 patients with disorders of sexual differentiation (DSD) conditions. Their ages ranged between 3 weeks and 46 years (Mean = 5.8).  Cases of gonadal dysgenesis (GD) included 42 patients; twelve of them were true hermaphrodites (TH). Patients with female pseudohermaphroditism (FPH) included 26 cases while those with male pseudohermaphroditism (MSH) were 29. Two trocars were used for diagnostic procedures and a third was added if further surgical management was needed.

 

Results:

              Simple diagnostic laparoscopy was performed for 43 cases; 25 of these were FPH reared as males having congenital adrenal hyperplasia (CAH) with severely masculinized external genitalia .They reversed their sex after surgery. All cases of gonadal dysgenesis required laparoscopy for their accurate diagnosis as well as for their further management.

Hysterectomy was performed for 22 patients: 8 cases had gonadal dysgenesis (MGD); 7 cases were TH reared as males, 5 cases of dysgenetic male pseudohermaphroditism, a case of persistent Mullerian duct syndrome and an adult male with lately diagnosed CAH.

Forty-eight gonadectomies were performed for 35 patients: streak gonads (26), ovaries (7), dysgenetic testes (7) and ovotestes (8). Bilateral gonadectomy for gonadoblastoma was performed for three patients: a pure gonadal dysgenesis, an androgen insensitivity syndrome and a patient with 5-alpha reductase deficiency.

Laparoscopic orchiopexy was performed for 19 patients. Staged Fowler Stephen’s technique was adopted in 13 of these cases. Laparoscopic repair of associated hernial sacs was performed in 5 cases.

 

Conclusion:

          Although the initial diagnosis can be reached in most cases of DSD by the clinical presentation, cytogenetic, biochemical and hormonal studies; yet in certain cases, laparoscopy provides a minimally invasive technique for their accurate diagnosis especially for cases with abnormal gonadal differentiation. It also serves as an efficient method for completion of their surgical reconstruction and removing structures contradictory to the assigned gender.

It is associated with low morbidity allowing a short postoperative recovery with minimal scars, an aspect which is important for patients with ambiguous genitalia who seek for reaffirmation of their body image and self- esteem.