Carotid-cavernous sinus fistula presenting as a posterior fossa mass. Case report.

The authors report a case in which trigeminal neuralgia and hearing loss developed 14 years after a severe head injury. Arteriography demonstrated a carotid-cavernous sinus fistula producing a large venous mass in the posterior fossa.     

Lumbosacral intramedullary myolipoma. Case report

Intradural myolipoma is an unusual tumor. A case is described in which extramedullary and intramedullary striated muscle fibers caused gross contraction of a lipomatous tumor as well as of the spinal cord during electrical stimulation.     

Lumbosacral subdural hematoma. Case report

An 18-year-old man presented with a rare spinal subdural hematoma (SSH) manifesting as headache, which developed following diving into the sea the day before. Computed tomography of the head revealed no abnormality. His headache persisted followed by neck stiffness and mild lumbago. He came to see us 6 days after the first visit. He underwent lumbar puncture to eliminate the possibility of meningitis. The cerebrospinal fluid showed xanthochromia. Cerebral angiography showed no abnormality. Magnetic resonance imaging of the spine revealed lumbosacral subdural hematoma. The hematoma and his symptoms regressed spontaneously during several weeks of conservative treatment. A sharp increase in intraabdominal and/or intrathoracic pressures following diving may have caused the bleeding. Headache may be an initial symptom associated with subarachnoid hemorrhage concomitant with SSH. Delayed progression of lumbosacral SSH must be considered if the patient complains of lumbago following minor trauma.     

Bilateral retro-auricular dermal sinus tracts with intradural extension. Case report

The authors describe a previously unreported malformation involving paired, bilaterally symmetrical dermal sinus tracts in the retro-auricular area, both of which passed through the asterion and posterior fossa dura mater to end intracranially. Cranial dermal sinus tracts are congenital lesions that virtually always originate from the midline scalp posteriorly at the external occipital protuberance, anteriorly at the nasion or along the nasal dorsum, or in the posterior parietal midline. A lateral origin is extremely rare, and intracranial extension of a lateral dermal sinus tract has not been reported previously. The authors propose an embryological mechanism to explain the origin of this rare malformation and discuss its management.     

Metastatic juxtaglomerular cell tumor in a 52-year-old man

Juxtaglomerular cell tumor is a rare renal neoplasm arising from the juxtaglomerular apparatus. Approximately 70 cases have been reported in the English literature since it was first described by Robertson et al in 1967. This tumor has been considered benign and resection has so far been curative. In this paper, we report the first metastatic juxtaglomerular cell tumor. The 15-cm tumor occurred in the right kidney of a 46-year-old man. It invaded the renal vein, and was treated by radical nephrectomy in 1995. The diagnosis at that time was renal cell carcinoma. The patient was well for 6 years and then developed bilateral lung masses, which were resected. Microscopically, the tumors from the kidney and the lungs were similar, consisting of solid sheets of uniformly round-to-polygonal cells intermixed with abundant delicate vasculature. Both renal and pulmonary tumors were positive for vimentin, renin, and only focally to CD34. Electron microscopic studies performed on the paraffin-embedded renal tumor and formalin-fixed lung tumor revealed the typical rhomboid crystals of proto-renin. In consideration of the characteristic morphologic features, immunohistochemistry, and the presence of rhomboid crystals of proto-renin, the diagnosis was modified to malignant juxtaglomerular cell tumor.     

Lumbosacral intradural periradicular ossification. Case report.

Intrathecal ossification causing progressive myelopathy or radiculopathy does not occur frequently. The majority of the reported lesions have been in the thoracic spine and seldom in the sacral canal. This report presents the occurrence of disabling low backache and sciatica from intradural periradicular ossification in the lumbosacral region in a previously healthy man.     

Huge mastoid congenital cholesteatoma in a 52-year-old patient

Congenital cholesteatoma may arise in various locations within the temporal bone. The rarest site of origin is the mastoid process. We report an unusual case of a 52-year-old man with mastoid congenital cholesteatoma that manifested as a persistent ear discharge. The preoperative suspicion was based on the imaging findings and the patient's history. A simple mastoidectomy was conducted and the cholesteatoma was completely removed while using facial nerve monitoring. Although rare, mastoid congenital cholesteatoma can be considered as an alternative in the differential diagnosis of persistent otorrhea.     

Endovascular treatment of a transverse-sigmoid sinus aneurysm presenting as pulsatile tinnitus. Case report

The authors report on the case of a 38-year-old woman who had experienced incapacitating pulsatile tinnitus in the left ear for 6 months. Angiographic studies revealed a wide-necked venous aneurysm of the left transverse-sigmoid sinus. Solitary stent placement across the aneurysm neck resulted in a slight modification in the lesion's characteristics. A second session, in which embolization with Guglielmi Detachable Coils was performed, resulted in a 100% occlusion of the aneurysm, with patency of the parent vessel and resolution of the tinnitus.     

Lateral buttock congenital dermal sinus tract

A 6-month-old female presented with purulent discharge from a dimple in the right lateral buttock. A subcutaneous abscess was palpated on the right paravertebral region at the L5-S1 level. She had low-grade fever with laboratory findings of leukocytosis and elevation of C-reactive protein levels. Klebsiella and Enterococcus species were cultured from the pus. Computed tomography (CT) clearly showed a tract traversing the subcutaneous tissue and connecting to the abscess. Magnetic resonance (MR) imaging showed no abnormality in the spinal canal. The diagnosis was infected congenital dermal sinus (CDS) in the right buttock. After normalization of body temperature and laboratory findings in response to antibiotic treatment, the dermal sinus tract was surgically removed. Intraoperative findings showed that the tract gradually tapered and ended at the subcutaneous abscess space over the lumbosacral fascia. Histological examination confirmed the lesion was dermal sinus. Although laterally placed CDS in the buttocks is extremely rare with only 5 previous cases reported, lateral CDS should be included in the differential diagnosis of a dimple in the buttocks. CT as well as MR imaging should be performed to evaluate suspected lateral CDS.     

Cystic fibrosis presenting in a 45-year-old man with infertility

Cystic fibrosis commonly is regarded as a disease of childhood associated with severe pulmonary and pancreatic pathological conditions. We report on a previously healthy 45-year-old man who was diagnosed as having cystic fibrosis after presentation with primary azoospermia. The literature is reviewed. Although references are made to late presentations of cystic fibrosis none was found in which the initial complaint was infertility. Primary azoospermia may be the presenting manifestation of cystic fibrosis in the absence of major respiratory symptoms.     

A 52-year-old man with limited hip movement

This case is presented to illustrate the imaging and clinical findings of a condition of interest to orthopedic surgeons. The initial findings are noted on this page. The clinical and imaging diagnoses are presented on the following pages.     

Charcot-Marie-Tooth disease presenting as a nonhealing ulcer in a 26-year-old man

A 26-year-old man presented with a nonhealing ulcer on the plantar aspect of the left foot of five years duration. Initial investigations were unremarkable. It was only after careful neurological examination that an inherited neuropathy was suspected. This was confirmed by nerve conduction studies and serum electrophoresis. He subsequently underwent partial great toe amputation for the ulcer and underlying first phalangeal osteomyelitis with uneventful healing. Neuropathic ulcers are usually associated with several well-known disorders including diabetes mellitus, tabes dorsalis, pernicious anemia, and sickle cell disease. A rarer cause is Charcot-Marie-Tooth Disease (CMTD). The report gives a review of CMTD and emphasizes that when faced with a nonhealing ulcer in the younger age group, such an underlying hereditary neuropathic cause must be considered.     

Anomaly of venous system in congenital occipital dermal sinus

Summary We report two cases of congenital occipital dermal sinus in which elongation of the vein of Galen, elevation of the straight sinus, division of the superior sagittal sinus, elevation of the confluence of sinuses, elevation of transverse sinus and narrowing of the torcular angle were observed in the venous phase of cerebral angiography. Enhanced computed tomography (CT) revealed enlargement of the supracerebellar cistern, elevation of the straight sinus and of the confluence of sinuses, but no evidence of intracranial lesions. In order to study the relationship between anomalies in the dural venous sinuses and congenital occipital dermal sinus, we examined both cases from an embryological viewpoint.