Percutaneous nephrolithotomy in older children

PURPOSE: The aim of this study was to evaluate the safety and efficacy of percutaneous nephrolithotomy (PCNL) in pediatric patients, older than 8 years, without any technical and instrumental modifications. METHODS: The authors retrospectively evaluated the data of 16 percutaneously treated kidneys of 14 patients with a mean age of 11 years (range, 8 to 17). The mean stone burden was calculated as 301 mm2 (range, 120-750). Percutaneous accesses were done under fluoroscopic control, and 24F to 30F Amplatz dilators were used depending on patient size. Ultrasonic and laser lithotripsy were performed to fragment calculi. RESULTS: Mean operating time was 111 minutes (range, 60 to 210 min) and no intraoperative or postoperative major complication was observed. Mean hemoglobin drop after the procedure was 1.16 g/dL (range, 0.3 to 2.8). Blood transfusion was required in only 1 patient, and mean postoperative hospitalization was 4.6 days (range, 3 to 10). Stone-free rate was calculated as 69% (11 of 16), but with residual fragments smaller than 4 mm, the success rate becomes 100% at patient's discharge. CONCLUSIONS: PCNL is an effective and safe form of therapy in pediatric stone disease. Especially in older children the use of the same instruments and technique as in adults may achieve equal results without any increased risk of possible morbidity and need of blood transfusion.     

Unusual bifid crista galli in a patient with cleft lip and palate

One of the most common craniofacial malformations observed in newborn babies is cleft lip and palate (CLP). This syndrome presents with some anatomic variations. The aim of this study was to understand an anatomic variation of the crista galli that had not been reported previously in patients with CLP. A 17-year-old boy with CLP was referred to an orthodontic clinic for cone-beam computed tomography (CBCT) imaging before treatment. Axial and coronal sections and three-dimensional images showed that the crista galli had bifid heads that were attached to each other. Incomplete ossification had resulted in a bifid crista galli and the appearance of a suture in the anterior coronal section of the CBCT images. This anatomic variation could allow penetration of the anterior fossa during endoscopic surgery, which could be dangerous for the patient.     

A Combination of Nifedipine and OctreotideTreatment in an HyperinsulinemicHypoglycemic Infant

Hyperinsulinemic hypoglycemia (HH) is the commonest cause of persistent hypoglycemia in the neonatal and infancy periods. Mutations in the ABCC8 and KCNJ11 genes, which encode subunits of the ATP-sensitive potassium channel in the pancreatic beta cell, are identified in approximately 50% of these patients. The first-line drug in the treatment of HH is diazoxide. Octreotide and glucagon can be used in patients who show no response to diazoxide. Nifedipine, a calcium-channel blocker, has been shown to be an effective treatment in a small number of patients with diazoxide-unresponsive HH. We report a HH patient with a homozygous ABCC8 mutation (p.W1339X) who underwent a near-total pancreatectomy at 2 months of age due to a lack of response to diazoxide and octreotide treatment. Severe hypoglycemic attacks continued following surgery, while the patient was being treated with octreotide. These attacks resolved when nifedipine was introduced. Whilst our patient responded well to nifedipine, the dosage could not be increased to 0.75 mg/kg/day due to development of hypotension, a reported side effect of this drug. Currently, our patient, now aged 4 years, is receiving a combination of nifedipine and octreotide treatment. He is under good control and shows no side effects. In conclusion, nifedipine treatment can be started in patients with HH who show a poor response to diazoxide and octreotide treatment.