Frontal bone fibrous dysplasia in a 6-months-old boy: A distinctive entity.

Fibrous Dysplasia (FD) is a non-malignant condition caused by post-zygotic, activating mutations of the GNAS gene that results in inhibition of the differentiation and proliferation of bone-forming stromal cells and leads to the replacement of normal bone and marrow by fibrous tissue and woven bone. The clinical behavior and progression of FD is variable. The management of this condition is difficult and in every case is strictly individualized. We report a case of frontal fibrous dysplasia in a 6month's old boy who underwent a successfully resection of the lesion with an excellent cosmetic effect.

Diagnostic and Surgical Approach of Thyroglossal Duct Cyst in Children: Ten Years Data Review.

INTRODUCTION: Thyroglossal duct cysts are the most common congenital midline neck swelling in children. AIM: To evaluate the clinical features, treatment, incidence of complications and outcomes in children with congenital midline neck lesions and more specifically with thyroglossal duct cyst, treated in our department the last ten years. It is a retrospective study. MATERIALS AND METHODS: The aggregated data revealed 59 patients with congenital midline neck lesions, of which 33 patients were with thyroglossal duct cyst (TDC). The diagnosis of TDC was made by physical examination, ultrasound (US) in all cases, and for complicated cases a magnetic resonance imaging (MRI) was performed. In all cases followed histopathological conformation of thyroglossal duct cyst. RESULTS: Fifty nine patients were admitted with midline cystic neck mass and the histopathological evaluation revealed, 33 cases (55.9%) of thyroglossal duct cyst, 14 cases (23.7%) of dermoid cysts, 3 cases (5.1%) of second branchial anomalies, 4 cases (6.7%) of inflammation of unknown cause, 4 cases (6.7%) of lymph nodes and 1 case (1.7%) of capillary skin haemangioma. More specifically, 38 patients were admitted with preoperative diagnosis of thyroglossal duct cyst, but in 5 patients pathological investigation demonstrated the presence of dermoid cyst. The mean age of the 33 patients with TDC at the time of surgery was 6.125 years, ranging between 9 months and 13 years with 10 patients younger than 3 years. Of the 38 patients with initial diagnosis of thyroglossal duct cyst, only four patients (10.5%) had a simple cyst excision and complication rate was 25% (1 case with recurrence). In these patients, the histopathological examination showed that three of them had a dermoid cyst and one had thyroglossal duct cyst. The other 34 patients (89.4%) were treated by Sistrunk's procedure, with two cases (5.9%) were proven on histology to be non TDC. In this group the complication rate was 3.03% (1 case with wound infection). CONCLUSION: The inappropriate surgical approach due to misdiagnosis or the incomplete surgical procedure remains the impact factor for recurrence. The modification of Sistrunk's procedure remains the main surgical technique that can provides good results with low rate of complication (3.03%) and recurrence (0%) as shown of our collecting data.

A novel use of dextranomer/hyaluronic acid copolymer.

The aim of this study is to present a novel use of Dextranomer/hyaluronic acid copolymer (Deflux) for the treatment of a complicated iatrogenic chronic urethral injury. A 12-year-old boy with a neuropathic bladder presented a groove in the urethral mucosa due to chronic clean intermittent catheterizations (CICs) and suffered a posterior urethral injury during a failed catheterization for a routine examination. The defect on the urethral wall was repaired using Deflux, a technique that is not described in the literature. After the intervention the patient is continuing CICs without further complications.

Melanotic neuroectodermal tumor of infancy (MNT1) arising in the skull. Short review of two cases.

INTRODUCTION: Melanotic neuroectodermal tumor of infancy (MNT1) is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, and rapidly growing that develops during the first year of life. It most commonly arises from the maxilla, the cranial vault, and the mandible. Early diagnosis and radical surgery are critical for a long-term outcome. METHODS: A literature search through PUBMED revealed 43 cases of MNT1 arising in the skull. We reviewed the available literature and studied the presenting symptoms, diagnostic procedures, treatment, rates of recurrences, malignancy, and data of follow-up. We report two further cases of infants aged 4 and 10 months, respectively, with MNT1 arising from the cranial vault who underwent radical excision of the lesion. CONCLUSION: Melanotic neuroectodermal tumor of infancy should be included in the differential diagnosis of skull lesions in infants. Radical surgery must be considered as the treatment of choice and close follow-up for at least 2 years is necessary.