Large aplasia cutis congenita of the vertex conservative management.

BACKGROUND: Aplasia cutis congenita (ACC) of the vertex with bone defect is a rare and begnin anomaly that can involve the epidermis, dermis, and subcutaneous tissues of the scalp with significant bone defect Bajpai and Pal (J Pediatr Surg 38(2):e4, 2003). When associated with skull defect, this rare malformation carries the risk of severe complications such as rupture of the superior sagittal sinus or infections. METHODS AND RESULTS: We report a case of aplasia cutis congenita of the scalp with skull defect measuring 9 × 10 cm and an exposed sagittal sinus in a newborn. Both conservative and surgical methods have been proposed to treat this condition. In our case, conservative treatment was planned led to complete epithelization and the patient was healing well at 5 years of follow-up. CONCLUSIONS: ACC of the vertex with a large scalp defects present a management dilemma Rocha et al. (Clin Case Rep 3(10):841-4, 2015). Based on a review of the literature, we report this case to demonstrate that even for the largest skin and bone defects, an initial conservative approach may allow for complete wound closure without the need for early surgical intervention.

Spinal epidural angiolipomas: Clinical characteristics, management and outcomes.

PURPOSE: The spinal epidural angiolipomas are rare expansive processes made of mature lipomatous and angiomatous elements. They often have a benign character. Their etiology, pathogenesis remains uncertain, and it is a cause of spinal cord compression. The magnetic resonance imaging is the most important neuroradiological examination. Histological examination is the only examination to confirm the diagnosis. Surgery is the treatment of choice. METHODS: A retrospective study of all patients operated on for a spinal epidural angiolipoma at the Department of Neurosurgery at the National Institute of Neurology of Tunis between January 2000 and December 2014 (15 years) was performed. The aim of this study is to describe the clinical, radiological, histological characteristics and the treatment of this tumor. RESULTS: A total of nine patients were operated from January 01, 2000 to November 30, 2014. The average age of our patients was 51 years with ages that ranged from 29 to 65 with a male predominance. The period between onset of symptoms and diagnosis ranged from 24 months with an average 12 months. Posterior localization of the tumor was seen in all patients. Surgical resection was performed for all cases. The postoperative course has been satisfactory, with a complete recovery of neurological functions in all patients. CONCLUSIONS: The spinal epidural angiolipomas is rare expansive process causing spinal cord compression. Treatment is exclusively surgical resection. The functional outcome of spinal epidural angiolipomas is particularly favorable with a complete neurological recovery is if the patient was quickly operated.

Secondary Chondrosarcoma of the Upper Thoracic Costovertebral Junction with Neural Foraminal Extension and Compressing the Spinal Cord.

BACKGROUND: Chondrosarcoma is a rare malignant tumor of bone. This family of tumors can be primary malignant tumors or a secondary malignant transformation of an underlying benign cartilage tumor. Secondary chondrosarcoma arising from a benign solitary costal osteochondroma is extremely rare. Data show that the reported incidence of costal osteochondroma is very low and they are usually found in the anterior region at the costochondral junction. To our knowledge, however, there have been no previous reports, in English literature, describing osteochondroma malignant transformation located in the thoracic costovertebral junction. CASE DESCRIPTION: We report the case of a man with chondrosarcoma arising from the malignant degeneration of an osteochondroma at the right first thoracic costovertebral junction with neural foraminal extension and compressing the spinal cord. CONCLUSIONS: Although it is rare in solitary osteochondromas of rib, malignant transformation must always be considered.

Intradural Extramedullary Capillary Hemangioma in the Upper Cervical Spine: First Report.

BACKGROUND: The occurrence of intradural extramedullary capillary hemangiomas is exceedingly rare. To date, only 39 cases of intradural extramedullary capillary hemangiomas have been reported in the English literature, and all of these cases have been described at the lumbar and thoracic spinal levels. To our knowledge, this report is the first case of capillary hemangiomas of the cervical spine in the literature. In general, this entity is misdiagnosed preoperatively as a neoplasm. CASE DESCRIPTION: A 29-year-old man presented with neck pain and progressive gait disturbance, and was diagnosed with an intradural extramedullary capillary hemangioma in the cervical region. CONCLUSIONS: Although rare, our case demonstrates that capillary hemangioma should be considered in the differential diagnosis of intradural extramedullary tumor of the cervical spine.

Primary sporadic Burkitt lymphoma of the orbit, clinical characteristics, management, and outcomes: a case study.

BACKGROUND: Involvement of the orbit with Burkitt's lymphoma is a very rare presentation of extra-nodal lymphoma. ILLUSTRATIVE CASE: We report a case of a 2-year-old female presented an unusual location of sporadic Burkitt's lymphoma arising in the orbital region. Diagnostic magnetic resonance imagining identified an oval-shaped mass on the lateral rectus of the right orbit that caused dislocation of eyeball, for which she underwent a biopsy from the periorbital swellings. The mass was histologically confirmed as Burkitt's lymphoma, and postoperative aggressive chemotherapy was initiated. We describe clinical diagnosis, histological aspects, radiological features, and current management of this rapidly growing malignant tumor. CONCLUSION: Because of the rapid progression of Burkitt lymphoma, and considering that it responds well to treatment, early recognition and appropriate management are important factors for survival and to preserve visual function.

Temporal Dermoid Cyst with Unusual Imaging Appearance: Case Report.

Intracranial dermoid cysts are benign, slow growing tumors derived from ectopic inclusions of epithelial cells during closure of neural tube. These lesions, accounting for less than 1% of intracranial tumors, have characteristic computed tomography (CT) and magnetic resonance imaging (MRI) appearances that generally permits preoperative diagnosis. However, the radiologic features are uncommon and the cyst can be easily misdiagnosed with other tumors in rare cases. Herein, we report a case of a left temporoparietal dermoid cyst in a 48-year-old woman that was peroperatively and histopathologically proven but not advocated on CT and MRI. Clinical, radiological and histopathological features of a dermoid cyst are reviewed.

Clinical and imaging findings in a rare case of sinus pericranii.

BACKGROUND: Sinus pericranii is a rare, usually asymptomatic condition that is characterized by an abnormal communication between the intra- and extracranial venous drainage pathways. The etiology is unknown but both congenital and post-traumatic etiologies have been proposed. Treatment is primarily surgical but newer minimally invasive endovascular approaches have been reported and is indicated due to cosmesis, hemorrhage, and air embolism. ILLUSTRATIVE CASE: We present a case of an 11-month-old boy having sinus pericranii, who was referred for a slowly growing tumor located frontally in the midline on his scalp since 6 months of age. CT-scan with three-dimensional CT (3D-CT) reconstruction and magnetic resonance imaging along with venography was performed which confirmed the diagnosis. Simple surveillance was decided because of a limited esthetic prejudice and the absence of any functional disorder. CONCLUSION: The prognosis is nearly always good with a low risk of bleeding. A simple follow-up is often proposed because of the usual absence of complications.