No abstract

Background: Congenital adrenal hyperplasia in females leads to virilization of external genitalia and persistent urogenital sinus. There are controversies regarding the timing and outcomes of surgery. Deferring surgeries beyond childhood is difficult to implement in conservative societies, and may result in stigmatization and distress to individuals with disorders of sexual differentiation and their families. Methods: Thirty girls with virilization due to congenital adrenal hyperplasia were admitted for single-stage feminizing genitoplasty, between 2011 and 2014. We prospectively studied the concerns and input of the families represented by the mothers. After comprehensive counselling, the mothers completed a questionnaire to clarify their priorities and concerns related to surgery. Results: Surgeries were performed at a mean age of 22 months. Most cases ranged between Prader's degrees III and IV. Egyptian families believe that early surgical reconstruction is in the best interest of their girls. They are marginally more concerned about functional outcomes and future child bearing than external appearance and cosmetic outcomes. Conclusions: Social difficulties noticeably add challenges to the management plan within conservative societies. Early genital reconstructive surgery, when reasonably indicated, needs to remain a viable option. Comprehensive psychosocial support within a multidisciplinary approach is needed to defer feminizing genitoplasty in selected cases to adolescence.

Wide bilateral cleft lip deformity reconstruction represents a special difficulty as it affects the lip, nose, and maxillary segments making single-stage reconstruction sometimes unobtainable. Many surgical and nonsurgical techniques have been prescribed to facilitate the definitive repair. Although some of these techniques proved to be useful, they have their inherent limitations and add another treatment step with all its possible complications and costs. The authors present a new method to address muscle layer repair in 1-stage procedure. It entails using fascial graft obtained from the temporalis muscle fascia or fascia lata, to reconstruct orbicularis oris lip muscle. Seven patients of wide bilateral cleft lip deformity (mean 17 mm) with a mean age of 4.4 months were subjected to single-stage lip reconstruction. After measuring the defect between both lateral muscle segments in front of the premaxilla intraoperatively ensuring that direct muscle repair could not be obtained, a fascial graft was harvested and sutured to both muscle edges. The authors found that, regardless the defect size or premaxilla protrusion, all wide clefts could be reconstructed satisfactorily in 1 stage procedure. No serious postoperative complications have been encountered in the lip or donor areas. Early follow-up reporting of the patients revealed stable repair. However more follow-up is still needed to assess late sequelae. In conclusion, fascial graft muscle repair of wide bilateral cleft lip deformity enables early 1-stage lip reconstruction without tension. The added donor morbidity is minimal and well tolerated.

Introduction: Hirschsprung's disease (HD) should be considered in children with neonatal-onset constipation. Clinical differentiation between HD and idiopathic constipation (IC) is difficult in late presenting infants. Consequently, paediatric surgical centres receive numerous referrals for rectal biopsies, requiring admissions and GA, particularly if suction biopsy is unavailable, and in older children. Methods: Forty-two cases referred for rectal biopsy, were studied for clinical features, single contrast enema, as compared to rectal biopsy findings, to determine the statistical reliability towards achieving a diagnosis. Results: The mean age at presentation was 106 days in HD patients, and 172 days in IC. Significant neonatal clinical features were present in 54%. Delayed passage of meconium was present in 86% of HD, compared to 14% of IC (p = 0.001). Rectal examination found a tight segment in 90% of HD, and a distended anorectum in 64% of IC (p = 0.005). The sensitivity of contrast enema was 86%,