[Vertex aplasia cutis congenita with bone defect: Management synthesis and review of the literature]. / Aplasia cutis congenita du vertex avec atteinte osseuse : synthèse sur la prise en charge et revue de la littérature.

Aplasia cutis congenita (ACC) of the vertex is a rare and potentially severe malformation. We report the case of a newborn boy presenting ACC of the vertex with a skull defect. Surgical closure of the skin was performed 5 days following birth, which allowed skin healing. The skull spontaneously remodelled perfectly 6 months later. When associated with skull defect, this rare malformation characterized by the absence of skin carries the risk of severe complications such as rupture of the superior sagittal sinus or infections. Based on a review of the literature, we purpose an overview of the medical and surgical management of vertex ACC, which depends on the size of the skin defect and the presence of a skull defect.

[Anatomic study and CT angiography with 3D reconstructions of the pulmonary arterial tree]. / Etude anatomique et angioscanographique avec reconstructions 3D de l'arbre artériel pulmonaire.

AIM OF THE STUDY: To prove in vivo and on cadaveric lungs, the constancy of the collateral type of distribution for the right and left pulmonary arteries as described in classical books and modern studies; to estimate the frequency of the main branches observed and define an arterial tree of reference set up with the most common branches for reading the CT pulmonary angiographies. PATIENTS AND METHODS: Sixty three-dimensional reconstructions of pulmonary arterial trees (right: 30; left: 30) using the Volume Rendering Technique (VRT) performed from CT angiographic studies of 30 patients without bronchial, arterial or pulmonary pathology. Dissection of 16 pulmonary arteries from eight fresh cadavers injected with latex. Finally, 76 pulmonary arteries (right: 38; left: 38) were examined. RESULTS: The most common division of the pulmonary artery is a collateral distribution but in seven cases from 38 (18.4%) at right and one case from 38 (2.6%) at left the artery ended in two or three terminal lobar trunks which provided the segmental arteries. The mediastinal artery for the upper right lobe was always found, with a complementary scissural artery in 89.5% of cases; a middle lobar trunk was observed in 22 of the 38 right lungs (57.9%). On the left tree, four different but usually not coexisting mediastinal arteries were identified for the upper lobe, three for the culmen and one for the lingula; six various scissural arteries were noted, three for the culmen and three for the lingula; the segment 6 received one or two segmental arteries, exceptionally three. An arterial tree of reference could be defined for both sides. CONCLUSION: The division in terminal lobar trunks of the pulmonary arteries is a variation demonstrated for the first time. The data obtained from 3D reconstruction imaging in vivo are in conformity with the results of studies performed on injected cadaveric lungs for this point and to define the arterial tree of reference.

[Chondrosarcomas of skull base treatment]. / Traitement des chondrosarcomes de la base du crâne.

BACKGROUND AND PURPOSE: Skull base chondrosarcomas are rare. Gross total removal is the treatment of choice, but can be difficult depending on the closeness of noble structures. Proton beam therapy can be associated in most cases. METHODS: Retrospective study of five cases treated in 13 years and study of the literature. RESULTS: Median age of patients was 34 years [28-46]. Cranial nerve palsy was the common clinical presentation. Tumor location was variable but always off midline. Treatment was surgical in all patients with a maximal resection and proton beam therapy associated for two cases. Surgical complications were rare with cranial nerve palsy as the main side effect. Outcomes were good with a median follow-up of 12.4 years [4.3-16.2]. DISCUSSION: The review of the literature showed that chondrosarcomas of skull base are rare. The best outcome is achieved with total surgical resection. Medical imaging can only give clues to the diagnosis. Pathology is required to obtain a precise immunohistochemistry diagnosis. Multidisciplinary treatment using proton beam therapy and surgical removal enables a good local control (90-100%) at 5 years with good quality-of-life. It is difficult to determine how many cases have been published (around 220 cases in the literature) since many surgical or radiotherapy series included the same patients.

Split cervical spinal cord malformation and vertebral dysgenesis.

We report a case of vertebral malformation associated with diplomyelia believed to be a type II split cord malformation. Cervicothoracic level cases are exceptional. This article reports the case of an 11-year-old boy with no neurological symptoms who had not undergone surgery. The diagnosis was made during pregnancy by prenatal screening with ultrasound and MRI. Several embryological theories have been offered to provide an explanation for this syndrome. Close follow-up is mandatory. Surgery must only be considered if neurological deterioration occurs.

[Anterior lumbar interbody fusion for treatment of failed back surgery syndrome: a retrospective study of 46 cases]. / Arthrodèses lombaires par voie antérieure dans le traitement des lombalgies et lomboradiculalgies récidivantes postchirurgicales : étude rétrospective de 46 cas.

BACKGROUND AND PURPOSE: Anterior lumbar interbody fusion (ALIF) has gained popularity for the treatment of degenerative disease of the lumbar spine. In this report, we present our experience with the ALIF procedure for treatment of failed back surgery syndrome following lumbar discectomy in a noncontrolled retrospective cohort. METHODS: From 1st January to 31 December 2005, we performed an ALIF in 46 patients presenting with low back pain with or without radiculopathy. All patients had a history of intractable pain resistant to conventional medical treatment and failed posterior lumbar surgery. Clinical and radiological outcomes were recorded. Neurological pain and functional outcomes were measured postoperatively (at 1, 3 and 12 months). Operative data, intraoperative complications, and the fusion rate were recorded. RESULTS: Forty-six patients with a preoperative diagnosis of failed back surgery syndrome underwent ALIF. The mean follow-up was 21 months. Back pain and leg pain completely disappeared in 60.9% of patients, decreased but required occasional medication in 28.3%, and 10.8% declared no benefit from ALIF surgery. CONCLUSION: On the basis of our results, we found ALIF to be a safe and effective procedure for the treatment of failed back surgery syndrome.

Chondrosarcoma of the skull base in Maffucci's syndrome.

We report a new case of skull base chondrosarcoma in Maffucci's syndrome. Maffucci's syndrome combining enchondromatosis with cutaneous haemangioma is rarely associated with chondrosarcoma. The review of literature highlights a small number of this pathological association.

[Transmaxillary approach to the skull base: surgical anatomy and guidelines]. / Voie d'abord transmaxillaire de la base du crâne : anatomie chirurgicale et repères.

BACKGROUND: A surgical and anatomic approach to the skull base using the transmaxillary route is presented. This route is well-known and used for a long time for sinus conditions. METHOD: This study was performed on injected cadavers. This study describes step by step this approach in microsurgical conditions following a vital lead: the infraorbital nerve. RESULTS: Anatomical landmarks are located in order to avoid complications. These complications are on one hand, hemorrhages by vascular lesions and on the other, definitive nerve palsy. CONCLUSION: Several skull base approaches exist, transfacial routes produce cosmetic damages. This route preserves the functional anatomy of the nose because it preserves the integrity of the lateral wall of the nasal cavity.

Spontaneous resolution of arachnoid cysts: review and features of an unusual case.

Most intra-cranial arachnoid cysts are quiescent and remain asymptomatic throughout life. Within the natural course of arachnoid cyst evolution, spontaneous resolution has been known to occur rarely, but its frequency is probably underestimated due to lack of systematic detection and long-term observation. We illustrate the spontaneous regression of arachnoid cysts with a patient which was conjointly diagnosed with an arachnoid cyst and a post-traumatic epidural haematoma. Cyst regression was observed 16 months later, upon examination following a second benign cranial trauma. Mechanisms underlying the resolution of the arachnoid cyst are discussed.

[Dumbell hemangioblastoma of the eighth cervical nerve root]. / Hémangioblastome "en sablier" de la huitième racine cervicale.

Hemangioblastomas involving cervical nerve roots are extremely rare. Only one case has been previously reported in the literature. We report the case of a 33-year-old man presenting with a 6-month history of upper limb pain. MRI and cervical angiography demonstrated the presence of a dumbell (intra and extradural) and highly vascularized tumor of the right C7-T1 foramina. Histological examination eventually confirmed the diagnosis of hemangioblastoma. Total removal of such a lesion may require combined (anterior and posterior) approaches and preoperative embolization.