Almazov National Medical Research Research Articles

One-stage correction of epispadia to Macedo in a girl 3 years old

Introduction. Epispadias is an extremely rare disease. Epispadias in women is 5–6 times less common than in men. The incidence of female epispadias ranges from 1 in 160,000 to 480,000 live births. Epispadias can be diagnosed by a thorough examination of the genitals. Treatment of epispadias is only surgical, which is quite difficult and requires experience. The literature that discusses female epispadias is very limited. In this article, we would like to report on the surgical treatment of isolated epispadias in a 3-year-old girl at the Almazov National Medical Research Center.Clinical case. A 3-year-old girl presented with the main complaint of urinary incontinence since birth, during the day and at night. Urinary incontinence was not caused by physical activity and was not aggravated by eating/drinking. Physical examination showed that the external genital organs of the patient have underdeveloped labia minora, abnormal urethra, bifurcated clitoris. Laboratory results were within the normal range. Voiding cystourethrography revealed urine leakage during the filling phase. The bladder wall was normal, there was no vesicoureteral reflux. The patient underwent simultaneous surgical interventions, consisting in plastic surgery of the urethra and reconstruction of the bladder neck, plastic surgery of the clitoris and labia minora. There were no intraoperative and postoperative complications. After 1 week and 6 months of observation, the patient achieved urinary retention, the surgical wound healed primarily.Discussion. Epispadias is a rare condition that can vary in severity from mild to severe. Severely, splitting of the entire urethra occurs, involving the bladder neck, causing permanent urinary incontinence in the patient. Cases of epispadias are quite difficult to diagnose. The doctor should dilute the labia majora and carefully conduct a physical examination. The objective goals of the surgical treatment of epispadias are to achieve urinary retention, restore the anatomy, function and cosmetic appearance of the urethra and genitals. One-stage reconstruction is a modern surgical technique used to treat isolated epispadias in women.Conclusion. Epispadias in women is a rare congenital anomaly that is often not detected in childhood. For isolated epispadias in a girl, one-stage surgical correction is currently preferred.

THE EXPERIENCE OF PEDIATRIC HEART TRANSPLANTATION ON NORTH-WEST OF RUSSIAN FEDERATION

Aim:to estimate early and long-term outcomes in recipients under 18 years old who have been heart transplanted in Almazov National Medical Research Centre.Materials and methods. From April 2011 to September 2017 we performed 5 heart transplantations (HTx) in recipients under 18 years old (female) old) from adults donors. The median of age were 15 years (range 10–16 years), LVEF prior HTx – 17% (10–33%). Causes of heart failure were dilated cardiomyopathy (n = 2), non-compacted myocardium (n = 1), arrhythmogenic ventricular dysplasia (n = 1) and Ebstein’s anomaly (n = 1). They spent in HT waiting list 76 days (12–684 days). One patient underwent biventricular assist device Berlin Heart EXCOR implantation (days on support – 250) as a «bridge» to transplant. Due to coronary angiography (CAG) results 1 patient underwent HTx and CABG simultaneously. All recipients treated by triple-drug therapy (steroids, calcineurin inhibitors, mycophenolate mofetil), induction (thymoglobulin – n = 4, basiliximab – n = 1). We evaluated retrospectively laboratory-instrumental investigations and frequency of complications after HTx.Results.The median of survival after HT was 35,93 months (4,4–73,7 months), all of them are alive. Patients spent in ICU 12 days (4–18 days), but one – 18 days due to posterior reversible encephalopathy syndrome (PRES), tacrolimus was switched to cyclosporine. They required inotropic support during 3 days (3–8 days). In 1 yr after HT TTE results got to normal values, the same as VO2peak signifi cantly improved. According to EMB (n = 48) results there were no clinical signs of rejection, acute cellular rejection (R2) was diagnosed in 12,5% cases. In long-term follow-up there was no signifi cant post transplant complications and comorbidities.Conclusion.Pediatric heart transplantation is an effective treatment of terminal CHF. There was no signifi cant clinical rejection under combined immunosuppressive regimens. All patients recovered and went back to normal life. Physical capacity improved in all recipients.