Feminising surgery in male pseudohermaphrodites.

In the framework of the international project between the paediatric surgery clinics of Kaunas Medical University and Copenhagen University Hospital, 34 patients raised as females were examined and treated. Eighteen patients were affected by androgen insensitivity syndrome, and 16 patients by mixed gonadal dysgenesis. All patients had a Y chromosome, although external genitalia was either typically female or had mixed features of both sexes. The particulars of diagnostics and treatment are discussed, focusing on surgical complications.

Congenital diaphragmatic hernia: a survey of practice in Scandinavia.

There is no consensus on the treatment of congenital diaphragmatic hernia (CDH), and practice seems to vary between centres. The main purpose of the present study was to survey current practice in Scandinavia. Thirteen paediatric surgical centres serving a population of about 22 million were invited, and all participated. One questionnaire was completed at each centre. The questionnaire evaluated management following prenatal diagnosis, intensive care strategies, operative treatment, and long-term follow-up. Survival data (1995-1998) were available from 12 of 13 centres. Following prenatal diagnosis of CDH, vaginal delivery and maternal steroids were used at eight and six centres, respectively. All centres used high-frequency oscillation ventilation (HFOV), nitric oxide (NO), and surfactant comparatively often. Five centres had extracorporeal membrane oxygenation (ECMO) facilities, and four centres transferred ECMO candidates. The majority of centres (7/9) always tried HFOV before ECMO was instituted. Surgery was performed when the neonate was clinically stable (11/13) and when no signs of pulmonary hypertension were detected by echo-Doppler (6/13). The repair was performed by laparotomy at all centres and most commonly with nonabsorbable sutures (8/13). Thoracic drain was used routinely at seven centres. Long-term follow-up at a paediatric surgical centre was uncommon (3/13). Only three centres treated more than five CDH patients per year. Comparing survival in centres treating more than five with those treating five or fewer CDH patients per year, there was a tendency towards better survival in the higher-volume centres (72.4%) than in the centres with lower volume (58.7%), p =0.065.

[Adrenogenital syndrome: feminizing genital reconstruction]. / Adrenogenitalinis sindromas: feminizuojanti genitaliju plastika, pooperaciniai rezultatai.

Adrenogenital syndrome, or so called congenital adrenal hyperplasia, is caused by a congenital insufficiency of the enzyme 21-hydroxylase, which is responsible for converting cortisol into cholesterol. Because of virilizing effect of androgens overproduction girls develop clitoral hypertrophy and persistent urogenital sinus (common channel for urethra and vagina). Surgical treatment is recommended in order to repair those developmental faults. The aim of this study was to employ the contemporary surgical techniques and to evaluate the postoperative results. Forty-seven patients affected by adrenogenital syndrome were investigated and treated at two institutions: Departments of Pediatric Surgery of Copenhagen University Hospital and Kaunas Medical University Hospital. Forty-three patients have been operated and underwent genitoplasty. Surgical method was chosen individually depending on the height of the urogenital sinus. In a case of low sinus a simple cut-back procedure was performed. In a case of high sinus the more complex procedure such as total urogenital mobilization or vaginal pull through would be involved. All patients underwent vaginal dilatations for 6-12 months postoperatively. Twenty-eight patients underwent clitoroplasty while the glans and the neurovascular bundle were preserved and clitoral skin used for plasty of the labia minora. Postoperatively the patients were observed for 0.5-5 years, the close results showed to be good. There were 4 cases of vaginal stenosis and 2 cases of urethrovaginal fistula (all successfully repaired later). Early one staged genitoplasty and postoperative vaginal dilatations for the period of 6-12 months is recommended.

Congenital diaphragmatic hernia in Scandinavia from 1995 to 1998: Predictors of mortality.

BACKGROUND/PURPOSE: There is a lack of large contemporary studies on the management of congenital diaphragmatic hernia (CDH), and the prediction of mortality remains difficult. The aim of this study was to investigate the influence of perinatal factors on mortality rate in a contemporary multicenter study. METHODS: The authors conducted a retrospective multicenter cohort study. Twelve of 13 Scandinavian pediatric surgical centers participated in the study. During a 4-year period (1995 through 1998) 195 children with CDH were included. The main endpoints were hospital mortality rate and total mortality rate (before 2001). Bivariate and multivariate survival analyses were performed using Kaplan-Meier plots, Log-rank test, and Cox regression. RESULTS: Overall hospital mortality rate was 30%. Among 168 neonates with symptoms within 24 hours (early presenters) 35% died before discharge. All 61 deaths occurred in 157 neonates with symptoms within the first 2 hours of life. Among early presenters, 27% had prenatal ultrasound diagnosis, 26% were delivered by cesarean section, and 21% had associated major malformations. Bivariate analysis of early presenters showed increased risk of death in neonates with prenatal diagnosis, associated anomalies, right-sided diaphragmatic hernia (RCDH), low 1-minute and 5-minute Apgar scores, low birth weight, short gestational age, and cesarean delivery. Neonates with prenatal diagnosis were characterized by significantly lower Apgar scores, lower birth weight, and increased frequency of associated anomalies than those diagnosed after birth. Multivariate analysis found that prenatal diagnosis (P =.004), 1-minute Apgar (P =.001), and RCDH (P =.042) were independent predictors of total mortality rate. CONCLUSIONS: In a series of 195 CDH patients, all 61 deaths occurred in the 157 neonates presenting with symptoms within the first 2 hours of life. Prenatal diagnosis, 1-minute Apgar score, and RCDH were significant independent predictors of total mortality.