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Úvod: Pyeloplastiku pro vrozenou hydronefrózu (obstrukci pyelo-ureterální junkce, PUJ) je u dětí možno provést otevřeně, laparoskopicky nebo roboticky. Cílem sdělení je prezentace pilotního souboru dětských pacientů operovaných roboticky. Metody: Retrospektivní analýza souboru dětských pacientů od 3 do 18 let věku, kteří podstoupili primární pyeloplastiku od zahájení robotického programu FN Motol od prosince 2018 do června 2021. Exkludováni byli pacienti již operovaní pro obstrukci PUJ v minulosti. Indikační kritéria, klinické symptomy, operační čas, délka hospitalizace a komplikace byly vyhodnoceny na základě zdravotnické dokumentace. Výsledky: V uvedeném období jsme operovali 28 dětí ve věku 3 až 17 let (medián 8 let), exkludováni byli dva pacienti. Z 26 analyzovaných dětí bylo před operací 6 (23 %) bez symptomů, 17 (65 %) udávalo bolesti, dvě (8 %) prodělaly před operací akutní pyelonefritidu a jedno (4 %) mělo nefrogenní hypertenzi. Nejčastější příčinou obstrukce byla aberantní céva u 22 (85 %) pacientů. Čas od incize k zašití ran byl 106 až 201 minut (medián 142 minut). Pacienti byli hospitalizováni 3 až 5 dní po operaci (medián 4 dny) a sledováni 1 až 30 měsíců (medián 6 měsíců). 24 z 26 pacientů mělo na pooperačním ultrazvuku regresi dilatace pánvičky a byli bez symptomů obstrukce PUJ. 7 pacientů (27 %) mělo pooperační komplikaci vyžadující intervenci v celkové anestezii (3b dle Clavien-Dindo klasifikace), z toho dva museli být reoperováni. Závěr: Robotická pyeloplastika je alternativou laparoskopické a otevřené operace. Jde o bezpečnou a úspěšnou (92 %) metodu. Vyšší podíl komplikací (27 %) v našem souboru přičítáme faktu, že jde o nový přístup, se kterým je třeba získat více zkušeností.

Introduction: Pyeloplasty for congenital hydronephrosis (pyeloureteral junction obstruction, PUJO) can be performed as an open, laparoscopic or robotic procedure in children. The aim of this study was to analyze a pilot cohort of pediatric patients operated robotically. Methods: A retrospective analysis of the cohort of patients 3 to 18 years of age who underwent primary robotic pyeloplasty between December 2018 (which is when the robotic program was launched at the University Hospital Motol) and June 2021. Patients already operated on for PUJO in the past were excluded. The indication criteria, clinical symptoms, operation time, hospitalization length and complications were evaluated from the medical records. Results: During this period, we operated on 28 children aged 3 to 17 years (median 8); 2 patients were excluded. Of the 26 children analyzed, 6 (23%) were asymptomatic before surgery, 17 (65%) reported pain, 2 (8%) had acute pyelonephritis before the surgery, and 1 (4%) suffered from nephrogenic hypertension. The most common etiology of PUJO was an aberrant vessel in 22 (85%) patients. The time from incision to skin closure was 106 to 201 minutes (median 142 minutes). Patients were hospitalized for 3 to 5 days (median 4) after the surgery and followed up for 1 to 30 months (median 6). Twenty-four of the 26 patients showed regression of the pelvic dilatation according to the postoperative ultrasound scan and no symptoms of PUJO. Seven (27%) had postoperative complications requiring a procedure under general anesthesia (all Clavien-Dindo 3b); of these, 2 were re-operated. Conclusion: Robotic pyeloplasty is an alternative to laparoscopic and open surgery. It is a safe and successful (92%) method. We believe that the higher rate of complications (27%) in our cohort is due to the fact that this is a new technique and more experience is needed.

Klíčová slova
pyeloplastika,
MeSH
dítě MeSH
laparoskopie metody MeSH
ledvinná pánvička * chirurgie MeSH
lidé MeSH
pilotní projekty MeSH
pooperační komplikace MeSH
retrospektivní studie MeSH
roboticky asistované výkony * metody škodlivé účinky MeSH
urologické chirurgické výkony * metody MeSH
Check Tag
dítě MeSH
lidé MeSH

Článek

Sexual function in adult females after feminizing genitoplasty for congenital adrenal hyperplasia

Journal of pediatric urology. 2022 ; 18 (3) : 282-286. [pub] 20220214

J Pediatr Urol
ISSN 1873-4898
Medvik
Zdroj

OBJECTIVE: To investigate sexual function and subjective attitudes towards surgery in adult women after feminizing genitoplasty (FG) for congenital adrenal hyperplasia (CAH). MATERIALS AND METHODS: We retrospectively reviewed the medical files of patients who underwent FG from 1996 to 2018. Of those, patients older than 16 years of age were asked to answer non-standardized questions aiming at the awareness of genital surgery and standardized questionnaire evaluating sexual function (GRISS). The anonymized answers were compared to a control group of 50 healthy females. Student's t-test, Pearson's χ2 test, Fisher's exact test and Spearman's rank correlation coefficient were performed. A P-value less than 0.05 was considered significant. RESULTS: Out of 106 patients who underwent FG, 64 were included, 55 successfully contacted and 32, aged 17-40 years (median 25.5), answered the questionnaires (58% response rate). In all, 11 patients underwent historical clitorectomy in early childhood by a pediatric gynecologist before 1996 and pull-through vaginoplasty at puberty. A total of 21 patients underwent modern one- or two-staged FG. There were statistically significant differences between the CAH group and controls as to the proportion living in committed partnerships (28% and 84%, respectively; p = 0.0000), having sexual intercourse (41% and 92%, respectively; p = 0.0000) and having their own children (13% and 36%, respectively; p = 0.0369). The median overall GRISS score was 4 (range 1-7) in the CAH group compared to 1 (range 1-9) in the control group, with infrequency and vaginismus representing the least favorable subscales (median 5). In all, 81% of the women operated on for CAH would recommend such an operation to any other individual with CAH and 66% of them suggested the surgery should be performed in early childhood. DISCUSSION: By this study we have contributed to the debate over suggestions that FG may cause adverse effects on sexual life of the patients in adulthood, beside already published effects on their mental well-being and LUTS. Despite possible selection bias due to only 58% response rate the comparison with control group shows only a moderate sexual dysfunction and more significant impact on long-term partnership that might be caused by other factors related to CAH beside FG itself. CONCLUSIONS: Partnership, offspring, sexual frequency and function were impaired in adult females with CAH after FG. However, the median GRISS score was below the pathological level of 5.

MeSH
dítě MeSH
dospělí MeSH
gynekologické chirurgické výkony MeSH
kongenitální adrenální hyperplazie * chirurgie MeSH
lidé MeSH
mladiství MeSH
předškolní dítě MeSH
retrospektivní studie MeSH
sexuální chování MeSH
urogenitální chirurgické výkony MeSH
Check Tag
dítě MeSH
dospělí MeSH
lidé MeSH
mladiství MeSH
mužské pohlaví MeSH
předškolní dítě MeSH
ženské pohlaví MeSH
Publikační typ
časopisecké články MeSH

Článek

Lower Urinary Tract Symptoms in Adult Females after Feminizing Genitoplasty for Congenital Adrenal Hyperplasia

The Journal of urology. 2021 ; 205 (5) : 1483-1489. [pub] 20201221

J Urol
ISSN 1527-3792
Medvik
Zdroj

PURPOSE: We investigated quality of life, long-term lower urinary tract symptoms, sexual function and subjective attitudes toward surgery in adult women after feminizing genitoplasty for congenital adrenal hyperplasia. MATERIALS AND METHODS: We retrospectively reviewed the medical files of all patients with congenital adrenal hyperplasia who underwent feminizing genitoplasty from 1996 to 2018 in our tertiary center. Of those, patients older than 16 years of age were asked to answer 1 nonvalidated and 3 standardized and validated questionnaires evaluating their current mental well-being (WHO-5 Well-Being Index), lower urinary tract symptoms (ICIQ-FLUTS) and sexual function (GRISS). The anonymized answers of this cross-sectional study were compared to a control group of 50 healthy females. Student's t-test, Pearson's χ2 test, Fisher's exact test and Spearman's rank correlation coefficient were performed. A p-value less than 0.05 was considered significant. RESULTS: Out of 106 patients who underwent feminizing genitoplasty, 64 patients were included and 32 patients, aged 17 to 40 years (median 25.5 years), answered the questionnaires (50% response rate). The difference between congenital adrenal hyperplasia and control group mental well-being was not statistically significant (WHO-5 median score 60 and 64, respectively; p=0.82). We found no significant difference in the lower urinary tract symptoms subscales of filling, voiding or incontinence, nor in the overall lower urinary tract symptoms score (ICIQ-FLUTS overall median score 3.5 and 3, respectively; p=0.43). CONCLUSIONS: We found in our group no abnormal mental well-being or prevalence of long-term symptoms of lower urinary tract dysfunction in adult female patients with congenital adrenal hyperplasia following feminizing genitoplasty.