[Cervicothoracic neuroblastomas : benefits of transmanubrial osteomuscular-sparing approach]. / Neuroblastomes cervico-thoraciques : les avantages de l'approche transmanubriale avec épargne ostéo-musculaire.

We report the case of a child suffering from a neonatal cervicomediastinal neuroblastoma encasing the left subclavian artery and the left vertebral artery. There is only a few pediatric tumors extending from the neck to the upper part of the thorax. Because of the complex vascular and neurological anatomy of this area, the surgical excision of these cervicothoracic neuroblastomas is a real challenge. It is why, when we decided to propose a surgical management, we used the Transmanubrial Osteomuscular-Sparing Approach (TOSA), of which technique and benefits will be explained in this article.

Nous rapportons le cas d'un enfant présentant un neuroblastome cervico-médiastinal néonatal engaînant les artères sous-clavière et vertébrale gauches. Les tumeurs s'étendant du cou à la partie supérieure du thorax sont rares en pédiatrie. De plus, l'anatomie vasculo-nerveuse complexe de cette région rend l'exérèse difficile. C'est pourquoi, à l'âge de 20 mois, lorsqu'une prise en charge chirurgicale a été décidée, nous avons utilisé l'approche transmanubriale avec épargne ostéo-musculaire ou TOSA (Transmanubrial Osteomuscular-Sparing Approach) dont nous détaillons la technique et les avantages.

Nasal heterotopia versus pilocytic astrocytoma: A narrow border.

Failure of the anterior neuropore can lead to three main types of anomalies: nasal dermal sinus, encephalocele and nasal glioma or heterotopia. In this report, we describe a case of intracranial and extracranial glial heterotopia that probably resulted from a common failure of anterior neuropore development. We describe the prenatal radiological assessment based on ultrasound and MRI results, and consider their limitation for early fetal diagnosis. We also discuss the embryogenesis and the possible pathogenic mechanisms involved.

[When rhinosinusitis reveals a systemic disease]. / Quand la rhinosinusite révèle une pathologie systémique.

This retrospective analysis is concerned with 10 patients suffering from granulomatosis with polyangitis (GPA, Wegener's disease), who were followed up in a tertiary care Ear, Nose, and Throat (ENT) department. The inaugural events took place in the ENT field (8 patients), the lung (2 patients), the vestibule (1 patient), or the oral cavity (1 patient). The ENT manifestations during the disease evolution involved the rhinologic (osetocartilaginous--6 cases; mucosal--9 cases), the otologic (3 cases), or the laryngeal area (2 cases). Facial pain was noted in 6 cases and residual hyposmia in 5. We observed 5 cases of lung involvement, 3 cases of renal involvement, and 4 cases of ocular involvement. An aseptic meningitis was seen in 1 case and the muscles were affected in 6 cases. The average delay between symptom onset and diagnosis was 26 months. Endoscopy, imaging techniques, and determination of antineutrophil antibodies (ANCA) were used to reach the diagnosis. Sinus biopsies were contributive in 6 cases. The patients were treated with immunosuppressive drugs and/or surgery. After treatment, remission was obtained in 6 patients.

Familial adhesive arachnoiditis associated with syringomyelia.

Adhesive arachnoiditis is a rare condition, often complicated by syringomyelia. This pathologic entity is usually associated with prior spinal surgery, spinal inflammation or infection, and hemorrhage. The usual symptoms of arachnoiditis are pain, paresthesia, and weakness of the low extremities due to the nerve entrapment. A few cases have had no obvious etiology. Previous studies have reported one family with multiple cases of adhesive arachnoiditis. We report a second family of Belgian origin with multiple cases of arachnoiditis and secondary syringomyelia in the affected individuals.

Sylvius aqueduct septum.

We present a case of chronic hydrocephalus discovered in adulthood through an episode of acute decompensation. Multimodal imaging revealed the cause of this hydrocephalus to be a membranous septum of the aqueduct of Sylvius, a condition for which few reports exist.

Diastematomyelia: pre- and postnatal multimodal diagnostic approach.

Diastematomyelia is a relatively rare congenital abnormality presenting as a sagittal separation of the spinal cord. Although cases of diastematomyelia have been previously reported, fully documented approaches by both prenatal and postnatal diagnostic workup are rare in the literature. We present a fully studied case of diastematomyelia type I investigated by prenatal US and MRI and postnatal US, MRI and radiography.

Intracranial germ cell tumor.

Germ cell tumours represent about 3 to 8% of pediatric brain tumours. Occurrence of diabetes insipidus is common in the case of suprasellar germ cell tumors. The diagnosis may be advanced by MRI owing to the location and relatively univocal characteristics of the lesion signal. The existence of a bifocal mass developed in both suprasellar region and pineal zone is highly suggestive of a germinoma. The most important notion is to recognize that at the time of diabetes insipidus diagnosis in a child, the cerebral mass might be too small to be identified by MRI. In such patients, repeating imaging study should be obtained.