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.