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.

Aim To evaluate modalities of extensor tendons repair of hand and forearm in specific anatomical zones with regard to etiological factors and presence of associated injuries of adjacent anatomical structures. Methods This cross-sectional study included 279 patients referred for extensor tendon repair of hand and forearm in specific anatomical zones. Available treatment modalities were evaluated concerning etiological factors, anatomical zones, and associated injuries. Statistical significance was analysed in the occurrence of early and late postoperative complications according to anatomical zones. Results Direct repair of extensor tendon lesions was found to be the most common modality of reconstruction, 230 (93.5%), of which blade injuries were predominant, 120 (48.7%). Direct tendon repair was mostly indicated in Zone VI and Zone III, in 55 (23.9%) and 42 (18.3%) patients, respectively. Statistically, a significant correlation was confirmed between treatment modalities, injuries in specific anatomical zones, and type of etiological factor (p<0.0001). Statistical correlation was confirmed between zones of injuries and the occurrence of early and late complications (p=0.002). Conclusion Successful postoperative recovery was correlated with the recognition of functional failure in specific zones, assessment of potential associated injuries, and selection of the most optimal modality of reconstruction.

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.

INTRODUCTION Gastric acidity plays an important role in the protection of infants against various pathogens from the environment. The histamine-2 receptor blockers (H2-blockers) are off-labeled drugs that are frequently prescribed in preterm neonates to prevent stress ulcers. The impact of the H2-blockers on the development of the necrotizing enterocolitis (NEC) in preterm infants is still controversial, particularly in the developing world. MATERIALS AND METHODS One hundred twenty-two preterm infants were enrolled in the study. The multivariate logistic regression model was used to identify potential postnatal risk factors associated with NEC. RESULTS Preterm infants (n = 51) with total NEC, medical NEC, and surgical NEC had the highest rate of receiving ranitidine compared with controls (n = 71) (39.2%, 19.6%, and 47.6%, p < 0.05). Logistic regression analysis revealed that ranitidine use and nosocomial infections were significantly associated with NEC development (odds ratios 1.55 and 3.3). CONCLUSIONS We confirm that ranitidine administration was associated with an increased risk of NEC in preterm infants. H2-blockers use should be only administered in very strictly selected cases after careful consideration of the risk-benefit ratio.

Objective – We present herein a new case and survey comprehensively literature on this rare condition. Case report – A 9-yearold girl with a medical history of surgical correction of clubfoot three months earlier presented to our department with an incidentally detected abdominal mass during diagnostic workup for orthopedic surgery. A CT scan revealed a solid right extra-adrenal mass measuring 7×6 cm. It was compressing/involving the infrahepatic part of inferior vena cava, right renal vein and artery with an incomplete encasing of the abdominal aorta. The surgery was successfully performed. The histopathological analysis confirmed GN. Conclusions – Although pediatric extra-adrenal retroperitoneal ganglioneuroma (GN) are rare, their propensity for enveloping major blood vessels is not. GNs should be considered in the differential diagnosis of any circumscribed retroperitoneal mass. These tumors can be successfully treated with surgery that leads to an excellent outcome, even in case of incomplete resections with tumor residuals <2 cm.

Objectives To evaluate the clinical outcome and complications in the pediatric population who had splenectomy at our institution, emphasizing the incidence of postplenectomy reactive thrombocytosis (RT) and its clinical significance in children without underlying hematological malignancies. Materials and methods The medical records of pediatric patients undergoing splenectomy were retrospectively reviewed for the period 1999–2018. The following variables were analyzed: Demographic parameters (age, sex), indications for surgery, operative procedures, preoperative and postoperative platelet count (postplenectomy RT), the use of anticoagulant therapy, and postoperative complications. The patients were divided into two groups according to indications for splenectomy: The non-neoplastic hematology group and the non-hematology group (splenectomy for trauma or other spleen non-hematological pathology). Results Fifty-two pediatric (37 male and 15 female) patients who underwent splenectomy at our institution were reviewed. Thirty-four patients (65%) were in the non-hematological group (splenic rupture, cysts, and abscess) and 18 patients (35%) in the non-neoplastic hematological group (hereditary spherocytosis and immune thrombocytopenia). The two groups did not differ significantly in regards to the patients’ age, sex, and preoperative platelet count (P>0.05 for all variables). Forty-nine patients (94.2%) developed postplenectomy RT. The percentages of mild, moderate and extreme thrombocytosis were 48.9%, 30.7%, and 20.4%, respectively. The comparisons of RT patients between the non-neoplastic hematology and the non-hematology group revealed no significant differences in regards to the patients’ age, sex, preoperative and postoperative platelet counts, preoperative and postoperative leukocyte counts, and the average length of hospital stay (P>0.05 for all variables). None of the patients from the cohort was affected by any thrombotic or hemorrhagic complications. Conclusions We confirm that RT is a very common event following splenectomy, but in this study it was not associated with clinically evident thrombotic or hemorrhagic complications in children undergoing splenectomy for trauma, structural lesions or non-neoplastic hematological disorders.

Abstract Rationale: Circumcision like any other surgical procedure is not devoid of complications. Serious complications are rare and include iatrogenic hypospadias, glans ischemia/necrosis, and glans amputation, all of which require an emergent treatment. Patient concerns: We report here a case of 6 months-old-boy with a superficial glans ischemia following circumcision. Diagnosis: Physical examination revealed a severely cyanotic glans with the moderate edema of the dorsal penile skin. Plasma levels of D-dimer were 8.57 mg/L. Urine passage was unremarkable while color Doppler ultrasonography revealed a normal blood flow. Interventions: The patient was successfully treated with subcutaneous injection of enoxaparin (low-molecular-weight heparin) and topical 2.5% dihydrotestosterone. Outcomes: The appearance of the glans penis on the 5th day was close to normal while the control levels of D-dimer dropped to the reference range. The patient was discharged from the hospital on the 6th day. At 6-month follow-up, the appearance of the glans penis was normal. Lessons: Acute glans penis ischemia following circumcision is a rare complication. Its successful treatment with enoxaparin and topical dihydrotestosterone has not been previously reported in the literature.