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Purpose. Presentation of clinical cases of rare combination of omphalocele with pylorostenosis in the postoperative period and additional liver lobe. Materials and methods. In National Medical Research Center for Childrens Health of health surgical ward of newborns and infants for the 2019 us operated 2 children who performed surgery involving intra-operative decision making about further surgical tactics. Results. the results of surgical treatment of newborns with omphalocele combined with hypertrophic pylorostenosis and extra liver lobe are presented. Conclusions. In patients with malformations of the anterior abdominal wall in the postoperative period, when regurgitation syndrome appears, it is necessary to make a differential diagnosis between the functional and organic causes of obstruction. When confirming the organic nature of the obstruction, surgical intervention is indicated. It is necessary to be able to timely and objectively assess the risks and expediency of the approach when choosing surgical tactics in each individual case.

Background and aims Prevalence of bladder exstrophy (BE) is 1 per 30–50 thousand newborns and it is observed in boys 2–6 times more often than in girls. This pathology demands reconstructive surgery at early age. We are presenting the unique clinical case of bladder exstrophy at the girl with the Apert syndrome (its prevalence is 1 per 100–160 thousand newborns) confirmed with molecular genetic testing. Research is focused on identification of appropriate surgical intervention taking into account anomaly form and anatomic features and child general medical condition. Methods Case history: the child from intact pregnancy and delivery. Multiple congenital anomalies (MCAs) haven’t been revealed prenatally. Girl was born on 27.05.2018. At birth the diagnosis of BE and multiple dysembryogenic stigmas was made. The girl was transferred to the NMRCCH in an urgent order at first 24 hours of life. On presentation: BE, total epispadia, divergent labia minora and majora, pubic bones diastasis (up to 4.6 cm). There was a defect on anterior abdominal wall wherethrough bladder mucous membrane was prolapsing. Anus was normal. External sex organs were abnormal, child gender couldn’t be identified adequately. The uterus was prolapsing due to anxiety at first 24 hours of life. Acrocephaly, frontal bone persistence, fingers and toes complete syndactyly, uranostaphyloschisis and choanal atresia came under notice. Results We have performed primary repair of bladder with local flaps on 5th day. Later we performed rehabilitation measures. Due to molecular genetic testing we revealed heterozygous mutation c.755C>G (p.Ser252Trp) in FGFR2 gene (Apert syndrome, autosomal dominant disorder). Long-term outcomes were estimated at six months age. The child is tube fed, puts on weight and develops. Urinary system infections took place twice due to vesicoureteral reflux. Note that bladder voiding and storage functions are adequate. Bladder volume is 60 ml and the interval between urinations is about 1 hour. Following step is to perform neurosurgical treatment. Orthopedic surgery should be performed in older age. Conclusion Bladder exstrophy treatment in newborns even comorbid with other MCAs is optimal and shall be carried out in referral centres. The choice and validation of surgery method for BE treatment demand personalized approach. Multidisciplinary approach in management of these patients is important in the presence of other MCAs.