Prenatal diagnosis and subsequent treatment of an intermediate-risk paraspinal neuroblastoma: case report and review of the literature.

OBJECTIVES: Neuroblastoma is the most common extracranial solid tumor of childhood, and the most common malignancy diagnosed during infancy. In comparison, neonatal neuroblastoma is relatively rare. Improvements in prenatal imaging and widespread use of fetal ultrasonography have led to an increased rate of prenatal diagnoses. METHODS: Case report and literature review. RESULTS: We report a case of an intermediate-risk neuroblastoma, diagnosed at 36 weeks' gestation by ultrasound and subsequently visualized by fetal MRI, that resulted in spinal cord compression and decreased fetal movement. A multidisciplinary team approach resulted in rapid delivery, evaluation, biopsy, staging, and treatment implementation in a successful effort to preserve lower extremity function. CONCLUSION: Prenatal diagnosis of neuroblastoma, management and outcomes are reviewed. Prompt diagnosis can strongly influence perinatal management and improve prognosis.

Fetal ultrasonography to prevent irreversible neurological sequelae of neonatal neuroblastoma.

An intra-abdominal mass was observed by fetal ultrasonography at 32 weeks of gestation. The baby was diagnosed as having neuroblastoma at the time of delivery at 39 weeks and its lower extremities were completely paralyzed. The chemotherapy after birth was quite effective to reduce the mass volume but neurological sequelae failed to improve. By carefully monitoring the movement of extremities, it may have been possible to prevent irreversible by inducing delivery before that state was reached.

Diagnosis and treatment of foregut duplications.

In its broadest sense, the term, foregut duplication encompasses the full spectrum of developmental aberrations of the embryonic foregut (bronchopulmonary and alimentary tract). Evidence is emerging that the notochord may have a pivotal role to play in foregut development through the Shh-GLi signalling pathway. The investigation and management of these lesions depends on the clinical presentation and the level of the foregut affected. The presentation of symptomatic foregut duplications also depends on any space-occupying effect they exert and where specific complications related to the malformation occur, such as when the mucosal lining contains acid-secreting cells. In a minority of cases, (eg, where they cause respiratory compromise or spinal cord compression) urgent intervention is required. In the remainder, precise diagnostic imaging according to the level and location of the foregut duplication provides the necessary information to plan surgical excision of the lesions. Magnetic resonance imaging best shows the relationships of complex bronchopulmonary foregut malformations and associated anomalies of the spine. eg, neurenteric canal. Most lesions can be excised with minimal morbidity. Minimal-access surgical techniques can be applied to the simpler cysts, particularly some bronchogenic cysts. Thoraco-abdominal duplications and neurenteric cysts require careful preoperative delineation and more complex surgery.

Craniovertebral junction anomalies in inherited disorders: part of the syndrome or caused by the disorder?

Patterns of skeletal abnormality at the craniovertebral junction in the normal population and in syndromes such as Down, Morquio etc, are compared and the recent embryological data and comparative anatomy reviewed. The authors' view based on their own clinical and radiological experience is that the os odontoideum is the product of excessive movement at the time of ossification of the cartilaginous dens and is exactly analogous to the unfused Type II odontoid fracture. True hypoplasia of the odontoid peg is part of a wider segmentation defect associated with Klippel Feil, occipitalised atlas and/or basilar invagination; it is hardly ever associated with instability.