Assessment of postoperative cosmetic outcomes of distal form hypospadias repair with the Hypospadias Objective Scoring Evaluation (HOSE).

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.

The complete spectrum of pentalogy of Cantrell in one of a set of dizygotic twins: A case report of a rare congenital anomaly.

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.

Unexpected adrenal pheochromocytoma associated with a generalized tonic-clonic seizure in a prepubertal boy: A case report.

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.

Short-term changes in renal function in children and adolescents undergoing extracorporeal shock wave lithotripsy.

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.

Extracorporeal shock wave lithotripsy effect on renal arterial resistive index changing.

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.

Quality and Safety of General Anesthesia with Propofol and Sevoflurane in Children Aged 1-14 Based on Laboratory Parameters.

INTRODUCTION: Knowledge of anatomic, physiological, biochemical and physical characteristics of children of all age groups, the existing illness and possible pathological response of the organism to the existing situation, require a pediatric anesthesiologist to participate in the preparation of a child for surgical treatment, to choose the best anesthesia technique and medications, and manipulative techniques to enable the scheduled surgical treatment with minimum anesthesia risks. The aim of this clinical study was to prove reliability and quality of propofol or sevoflurane general anesthesia in children in the age group of 1-14 years from the ASA I group and in the elective surgical treatments in duration of 60 minutes, based on preoperative and postoperative levels of laboratory findings (transaminases, blood sugar, urea and creatinine). MATERIALS AND METHODS: the study included 160 patients randomized in two groups based on different approaches: total intravenous anesthesia was used for the propofol group (n=80) (TIVA) and the inhalation technique was used for the sevoflurane group (n=80). RESULTS: statistical evaluation of the obtained results indicates stability of laboratory findings in the immediate postoperative course (after 24 hours) in respect to the preoperative period. Based on the Mann Whitney test (P), preoperative and postoperative blood sugar levels in the sevoflurane vs. propofol group were P=0.152 vs. 0.021; creatinine levels P=0.113 vs. 0.325; urea levels P= 0.016 vs. 0.900; AST levels P=0,031 vs. 0,268 and ALT levels P=0.021 vs. 0.058. Level of significance was P<0.5. CONCLUSION: Analysis of the examined laboratory parameters show that propofol and sevoflurane provide full security and quality of general anesthesia in children age group 1-14 years, from the ASA I group. All analyzed laboratory levels in the postoperative course remained in their referential values in both groups of participants.