Infant Odontogenic Myxoma: a specific entity.

INTRODUCTION: In children, and specifically in infants, odontogenic myxomas are extremely rare. In infants, myxoma seems to display mostly the same clinical, radiological and pathological characteristics. This paper presents a series of odontogenic myxomas in infant patients. MATERIALS AND METHODS: Four infant patients were included in this retrospective study. The clinical, radiological and pathological presentation was characterized and the treatment analysed. RESULTS: All patients presented with a rapidly evolving paranasal swelling. CT-scan showed a maxillary homogeneous unilocular and intraosseous tumour. In all cases, pathological examination revealed a loose myxoid stroma within stellate and spindle shaped cells. All patients underwent conservative surgery through a vestibular approach. CONCLUSION: This patient series and a review of the literature demonstrates that odontogenic myxoma is specific in infant. We propose the name of Infant Odontogenic Myxoma for this entity.

Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009.

OBJECTIVE: To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children. STUDY DESIGN: Correspondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009. RESULTS: 21 cases were identified; 62% of the patients were boys. No familial cases were observed, and the disease appeared to be sporadic. Mean age at diagnosis was 8.0 years [0.8-16.9 years]. Median follow-up was 3 years [0.3-17 years]. The main presenting signs were bone fracture (n = 4) and respiratory distress (n = 7), but more indolent onset was observed in 8 cases. Lung involvement, with lymphangiectasies and pleural effusion, was the most frequent form of extraosseous involvement (10/21). Bisphosphonates, alpha interferon and radiotherapy were used as potentially curative treatments. High-dose radiotherapy appeared to be effective on pleural effusion but caused major late sequelae, whereas antiangiogenic drugs like alpha interferon and zoledrenate have had a limited impact on the course of pulmonary complications. The impact of bisphosphonates and alpha interferon on bone lesions was also difficult to assess, owing to insufficient follow-up in most cases, but it was occasionally positive. Six deaths were observed and the overall 10-year mortality rate was about 30%. The prognosis depended mainly on pulmonary and spinal complications. CONCLUSION: Aggressive vascular abnormalities of bone are extremely rare in childhood but are lifethreatening. The impact of anti-angiogenic drugs on pulmonary complications seems to be limited, but they may improve bone lesions.

Fetal and neonatal ovarian cysts: is surgery indicated?

OBJECTIVES: To evaluate the frequency of ovarian torsion in neonates with ovarian cysts (OCs) and to analyze the outcome after surgical treatment. METHODS: A retrospective review of all infants with OCs managed in our institution during 20 years, was conducted. Our management included a follow-up (FU) of prenatal serial ultrasound (US) scan without prenatal cyst aspiration and early postnatal US scan. Neonatal surgery was indicated for cysts that appeared complex regardless of size and for simple cysts larger than 20 mm. RESULTS: Sixty-seven OCs detected through prenatal diagnosis were identified. Cysts were detected at a median gestational age (GA) of 33 weeks. At diagnosis, median size of the OCs was 40 mm, and 18% were complex. On the postnatal US scan, 55% were complex. Neonatal surgery was performed for 64 OCs : 56% were torsed, 6% were hemorrhagic, and 38% were uncomplicated. Proportions of complex- and simple-appearing cysts on prenatal or postnatal US scan were the only significantly different parameters found between infants with torsed or not torsed OC. CONCLUSION: Our study demonstrated that OC bears a high risk of ovarian loss. These findings have implications regarding information and treatment offered to pregnant women bearing an affected fetus.