Aim A standardized assessment for the optimal repair of hypospadias remains elusive. The aim of this study was to assess a postoperative cosmetic outcome of hypospadias repair using a validated questionnaire, Hypospadias Objective Scoring Evaluation (HOSE). Methods During the period between January 2016 and May 2019, 40 patients who underwent hypospadias repair were identified and they agreed to a follow-up using the HOSE. Distal hypospadias repairs underwent a cross-sectional assessment of the cosmetic outcome. Cosmetic assessment was performed by an independent physician using the HOSE scoring system. Results The native meatus was coronal in 10 (25%), subcoronal in eight (20%), and distal penile in 22 (55%) patients. Mean followup was 35.90 months (SD ±29.58) postoperatively (range 12-162 months). Complications occurred in one (2.5%) patient. Out of 40 uncomplicated repairs, 39 (97.5%) were satisfactory. A vertical slit-like meatus located at the distal glans was created in 33 (82.5%) boys, and at the proximal glans in seven (17.5%). The urinary stream was single and straight in 39 and spray in one patient. A straight erection was observed in 39 (97.5%) boys. The median HOSE score was 16 (range 12-16). One patient had a small, single coronal fistula. The technique used included tubularised incised plate urethroplasty. Conclusion The HOSE score is simple, easy, non-invasive and non-expensive tool for objective assessment of long-term outcomes of hypospadias repair.

Abstract Rationale: Pentalogy of Cantrell (POC) is an extremely rare syndrome with an estimated incidence of 1:65,000 to 200,000 live births. Its complete form includes a midline epigastric abdominal wall defect, defects affecting the lower sternum, anterior diaphragm, diaphragmatic pericardium, and various intracardiac defects. Patient concerns: We report a case of complete POC affecting only the first-born of a set of premature dizygotic twins. Diagnosis: A giant omphalocele with an eviscerated liver and bowel on prenatal, obstetric ultrasonography at 24 gestational weeks was observed. At birth, physical examination confirmed a massive (10 × 8 cm) epigastric omphalocele in which a significant part of the liver was seen. A postnatal echocardiogram revealed the presence of an ostium secundum atrial septal defect, perimembranous ventricular septal defect, and moderate pulmonary stenosis. X-ray showed an abnormal intrathoracic positioned stomach, which was confirmed with a plain x-ray of the upper intestinal tract with hydrosoluble contrast. Computed tomography (CT) scan revealed the sternum's absence and a close connection between the pericardial sac and the stomach wall. Interventions: The patient underwent surgical intervention at 18 days of age. Outcomes: Despite adequate and appropriate postoperative treatment, the baby rapidly deteriorated and died 72 hours after surgery. Lessons: POC is a complex, high-mortality syndrome whose management requires a multidisciplinary approach and meticulous planning. Despite all efforts, POC carries a poor prognosis, particularly in patients affected by its complete form.

Abstract Rationale: Pheochromocytoma (PHEO) is a rare neuroendocrine tumor arising from chromaffin cells of the adrenal medulla. Most pediatric PHEOs are functional tumors, and clinical manifestations are related to catecholamine hypersecretion and/or tumor mass effects. Patient concerns: We report here a case of a 10-year-old boy with a highly functional adrenal PHEO detected after the evaluation of a generalized tonic-clonic seizure in the patient. His vital signs at admission were: blood pressure up to 220/135 mm Hg; pulse, 112 beats/min; temperature, 37.4°C; respiratory rate, 22 breaths/min. Diagnosis: A 24-hour urine collection for catecholamines test showed a marked increase in Vanillylmandelic acid levels (338.9 μmol/L). An abdominal magnetic resonance imaging revealed a well-defined left adrenal gland mass measuring ∼5 cm in its largest dimension. Interventions: The mass was surgically removed, and histopathological examination revealed PHEO with low malignant potential (Adrenal Gland Scaled Score/PASS/ < 4). Outcomes: The patient was discharged on the 10th postoperative day in good condition. At 24-month follow-up, the patient was doing well without complications such as tumor recurrence, elevated blood pressure, and seizure. Lessons: PHEO should be considered in the differential diagnosis of children with seizures presenting in the emergency department. A multidisciplinary approach to the evaluation and treatment of PHEO is also crucial for a successful outcome.

Clinic of Pediatric surgery, Clinical Center University in Sarajevo, Patriotske lige 81, 71 000 Sarajevo, Bosnia and Herzegovina Clinic of Radiology, Clinical Center University in Sarajevo, Bolnička 25, 71000 Sarajevo, Bosnia and Herzegovina Clinic for Gastroenterology, Clinical Center University in Sarajevo, Bolnička 25, 71000 Sarajevo, Bosnia and Herzegovina Clinic of Pediatrics, Clinical Center University in Sarajevo, Patriotske lige 81, 71 000 Sarajevo, Bosnia and Herzegovina Clinic for Anaesthesiology and Reanimation, Clinical Center University in Sarajevo, Bolnička 25, 71 000 Sarajevo, Bosnia and Herzegovina Pediatric Intensive Care Department, Pediatric Clinic, Clinical Center University in Sarajevo, Patriotske lige 81, 71 000 Sarajevo, Bosnia and Herzegovina

A 10 years old boy, was sent from regional hospital. His symptoms started the day before he was hospitalized and represented as gastrointestinal bleeding, lower abdominal pain and four times vomiting, without fever. Ultrasound and X-ray of the abdomen were normal. Blood fi ndings showed: RBC 3,19, hemoglobin 0,95, hematocrit 0,27. During a physical examination abdomen was palpatory soft, with no presence of the pain. Digital rectal examination showed blood. A scintigraphy pathologic scan showed a focal lesion of the right hemi abdomen consistent with the Meckel’s diverticulum.Patient was treated byLaparoscopic-Assisted Transumbilical Extracorporeal Resection of the Meckel’s Diverticulum. Case Report

A preterm female infant with a birthweight 1770 g was born via spontaneous vaginal delivery at 34 weeks’ gestation to a 21-yearold mother who received an adequate prenatal care. Routine prenatal ultrasound at 30 weeks revealed a proximal dilatation of the gut and polyhydramnios. At delivery, initial physical exam revealed a soft ‘scaphoid’ abdomen. Apgar score was 7 and 8 at 1st and 5th minute, respectively. A nasogastric tube (NGT) was placed for gastric decompression and bilious drain was observed. Post-natal erect X-ray of the abdomen showed dilated loops in the upper abdomen with the paucity of gas in the pelvis and confirmed proximal bowel obstruction (Fig. 1). After 24 h of stabilisation and care in the neonatal intensive care unit, the patient was taken to the operating room for surgical repair of her presumed jejunal atresia. Surgical exploration revealed a markedly distended atretic jejunum with a ‘type 3B’ intestinal atresia (apple-peel jejunal atresia) and a significant loss of intestinal length. The proximal end of the jejunal atresia was located 15 cm below the ligament of Treitz. On the distal end of ileal atresia, there were two multiple ileal atresias, each 7 cm of length. Approximately, 55 cm of the small bowel distal from the ileal atresias was found to be patent (Fig. 2a). In our case, the total length of small intestine was 70 cm (15 cm distal to the ligament of Treitz and 55 cm proximal to the ileocecal valve). All atretic segments were resected. The remaining segments were preserved (without tapering proximal jejunoplasty) and connected with the end-to-end jejunoileal anastomosis using 5/0 polyglactin sutures in the single extramucosal layer employing the Cheatle technique for size mismatch (Fig. 2b). Appendectomy was also performed. Post-operatively, the patient was placed on mechanical ventilation and extubated on the 6th post-operative day (POD). Total parenteral nutrition (TPN) via a central line along with enteral nutrition via NGT were administered and lasted until the 39th POD. After 2 weeks, enteral feeding gradually improved, so the baby started receiving peroral nutrition via bottle. The patient achieved her full enteral intake on POD 38. The baby was discharged from the hospital on POD 46 with highly improved body weight – 2485 g. At the follow-up of 9 months (Fig. 3), the baby was doing well with a body weight of 7500 g.

Introduction: Transverse colon volvulus is an uncommon cause of bowel obstruction. Predisposing factors are mental retardation, dysmotility disorders, chronic constipation, and congenital megacolon. Case report: We presented a transverse colon volvulus in a 14-year-old girl with mental retardation. Chronic constipation in neurologically impaired patients was a risk factor predisposing to volvulus. The girl was admitted to our Clinic because of problems that last 4-5 days and was followed by abdominal pain, vomiting and lack of stool. Vomiting was once a day. She was sub febrile up to 37.6°C. On examination, the abdomen was distended, tense, diffusely painful. During the surgery, a 360° clockwise volvulus of the transverse colon was found. After the reduction of volvulus, an enormous transverse colon was resected and protective ileostomy was formed. In the postoperative period, ileostoma functioned a good. The definitive surgical treatment was done on the 20th postoperative day when the occlusion of the ileostomy and transanal biopsy of the rectum was done, which showed the presence of ganglia cells. The patient was discharged from our institution after 1 month. Conclusion: Pediatric patients with neurological conditions and mental retardation present an increased risk of colon transversum volvulus due to chronic obstruction.

Aim To identify short-term effects of extracorporeal shock wave lithotripsy (ESWL) on renal function in children and adolescents with single kidney stones. Methods In a 4-year period 30 children (15 boys and 15 girls) from 10 to 18 years of age were treated for unilateral renal stones with ESWL. Inclusion criteria were: up to 18 years of age, kidney stone (from 4 to 20 mm in diameter) visible on X-ray, first ESWL treatment, unilateral lithotripsy treatment without previous kidney surgery, patients without infravesical obstruction, patients without proven urinary infection, repeated use of one (the same) analgesic, patients without anticoagulant and antihypertensive therapy, patients without use of nephrotoxic drugs prior to and during the treatment, normal blood pressure, non-pregnancy patients with normal renal function. Serum enzymes (alkaline phosphatase, lactate dehydrogenase), cystatin C, serum and urine electrolytes (sodium, potassium, chloride), and urine neutrophil gelatinase-associated lipocalin (uNGAL) were tested before, on the first and fifth day after the treatment. Results An increase of alkaline phosphatase and lactate dehydrogenase was statistically significant on day 1 (p<0.05) and values returned to normal on day 5. Serum cystatin C level was also significantly increased during the first four days after ESWL treatment (p<0.05) and returned to baseline on post-treatment day 5. There was a statistically significant difference in the level of uNGAL in urine before and 24 hours after ESWL treatment (p<0.05). Conclusion The ESWL is a safe and curative procedure for the treatment of kidney stones in children and adolescents with no evidence of serious adverse effects on renal function.

Aim To investigate a correlation between resistive index (RI) level changes following extracorporeal shock wave lithotripsy (ESWL) in treated and non-treated kidneys depending on the ESWL treatment intensity. The study was conducted on 60 subjects, which were divided in two groups according to age and treatment protocol. Results In the group of patients younger than 55 years of age there was a significant increase in mean RI values, on the first (p=0.001) and second day after the treatment (p=0.007). In the group older than 55 years of age, the resulting increase in mean RI levels was also significant on the first (p=0.003) and second (p=0.011) day following the treatment. The RI values in the non-treated kidney on the first day after the treatment grew significantly (p=0.033). In the group older than 55, RI values in the non-treated kidney grew significantly on the first day after the treatment (p=0.044). In the group who received 2000 SWs, RI levels grew significantly (p=0.044) as well as in the group who received 4000 SWs during the treatment, where the significance was more pronounced (p=0.007). Conclusion There is a correlation between RI changes and the degree and localization of changes in vascular elements of the kidney. Post-ESWL treatment changes are existent and reversible, over a period of one week after the treatment.