Outcomes of one-stage feminizing genitoplasty in children with congenital adrenal hyperplasia and severe virilization.

PURPOSE: To present our surgical experience and outcomes in congenital adrenal hyperplasia (CAH) patients with severe virilization using a combined technique of total urogenital mobilization (TUM) and a modified pull-through vaginoplasty to perform a safe and effective one-stage feminizing genital reconstruction for these children. METHODS: Fourteen CAH patients with severe virilization, defined by a Prader IV and V rating of the external genitalia, underwent TUM followed by a limited vaginal pull-through procedure from June 2016 to December 2020. Postoperative anatomical and cosmetic outcomes, and urinary continence, were evaluated. RESULTS: Out of the 14 cases in this study, 8 were classified as prader IV and 6 as Prader V. The median age at surgery was 11 months (range 6-36 months), and the mean urethral length was 1.4 cm (range 1.2-1.8 cm). The median follow-up period was 4 years. Our cosmetic outcomes were good in 11 (78.5%), satisfactory in 2, and poor in one case. All patients achieved age-appropriate toilet training without urinary incontinence. CONCLUSION: Adopting our surgical approach of TUM with modified pull-through vaginoplasty has simplified feminizing surgical reconstruction in CAH cases with severe genital atypia and a very high vaginal confluence with short urethral length, yielding adequate introitus with good anatomical and cosmetic appearance and adequate urinary continence outcomes.

High Abdominal Perfusion Pressure Using Umbilical Cord Flap in the Management of Gastroschisis.

Background: Gastroschisis management remains a controversy. Most surgeons prefer reduction and fascial closure. Others advise staged reduction to avoid a sudden rise in intra-abdominal pressure (IAP). This study aims to evaluate the feasibility of using the umbilical cord as a flap (without skin on the top) for tension-free repair of gastroschisis. Methods: In a prospective study of neonates with gastroschisis repaired between January 2018 to October 2020 in Tanta University Hospital, we used the umbilical cord as a flap after the evacuation of all its blood vessels and suturing the edges of the cord with the skin edges of the defect. They were guided by monitoring abdominal perfusion pressure (APP), peak inspiratory pressure (PIP), central venous pressure (CVP), and urine output during 24 and 48 h postoperatively. The umbilical cord flap is used for tension-free closure of gastroschisis if PIP > 24 mmHg, IAP > 20 cmH2O (15 mmHg), APP <50 mmHg, and CVP > 15cmH2O. Results: In 20 cases that had gastroschisis with a median age of 24 h, we applied the umbilical cord flap in all cases and then purse string (Prolene Zero) with daily tightening till complete closure in seven cases, secondary suturing after 10 days in four cases, and leaving skin creeping until complete closure in nine cases. During the trials of closure, the range of APP was 49-52 mmHg. The range of IAP (IVP) was 15-20 cmH2O (11-15 mmHg), the range of PIP was 22-25 cmH2O, the range of CVP was 13-15 cmH2O, and the range of urine output was 1-1.5 ml/kg/h. Conclusion: The umbilical cord flap is an easy, feasible, and cheap method for tension-free closure of gastroschisis with limiting the PIP ≤ 24 mmHg, IAP ≤ 20 cmH2O (15 mmHg), APP > 50 mmHg, and CVP ≤ 15cmH2O.

Coccygectomy for Chronic Refractory Coccygodynia in Pediatric and Adolescent Patients.

AIMS: Coccygodynia (also known as coccydynia and coccyalgia) described for the first time by Simpson in 1859. It is a disabling pain in the coccyx usually provoked by sitting or changing position from a sitting to a standing position. Treatment started with adjustment of ergonomics as specialized cushion for sitting, application of local heat, and oral analgesics. The aim of this study was to assess the results of total or partial coccygectomy for patients suffering from chronic refractory coccygodynia and not improving on conservative treatment in pediatrics and adolescents. METHODS: The study included 22 patients, 8 of them with chronic refractory coccygodynia not responding to conservative management underwent partial or total coccygectomy using Key's surgical exposure after good preoperative preparation. RESULTS: This study included 5 females (62.5%) and 3 males (37.5%), their ages ranged from 7 to 15 years. The results were excellent in 75% of patients and good in the remaining cases. The mean length of hospital stay was 1.43 ± 0.98 days. No significant intraoperative or postoperative complications were reported. The parents were satisfied in almost all cases in the long term follow up. CONCLUSIONS: Coccygodynia is a disabling problem, especially in pediatric patients. Coccygectomy is a feasible management option and has a favorable outcome when performed for an appropriate indication.