ABSTRACT
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by recurrent epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations (AVM). Of these, spinal AVM is a rare manifestation that concerns mainly children. In this report, we describe two cases of spinal AVM revealed by acute paraparesis due to subarachnoid hemorrhage in children with HHT and reviewed the literature on spinal arteriovenous malformations in HHT. In most of the cases reported, the clinical presentation was acute in the pediatric population and insidious during adulthood. The prognosis of spinal AVM mainly depends on the presence or not of medullar signs and symptoms and on the delay before treatment. In conclusion, any child with a family history of HHT should be considered at risk for spinal AVM in order to improve management of such complications and to decrease the risk of neurological sequellae.
Subject(s)
Arteriovenous Fistula/complications , Arteriovenous Fistula/genetics , Paraplegia/etiology , Spinal Cord/blood supply , Telangiectasia, Hereditary Hemorrhagic/complications , Acute Disease , Arteriovenous Fistula/diagnosis , Arteriovenous Fistula/therapy , Child , Chromosome Aberrations , Diagnosis, Differential , Embolization, Therapeutic , Follow-Up Studies , Genes, Dominant , Humans , Infant , Magnetic Resonance Angiography , Magnetic Resonance Imaging , Male , Neurologic Examination , Spinal Cord/pathology , Spinal Cord Ischemia/diagnosis , Spinal Cord Ischemia/genetics , Spinal Cord Ischemia/therapy , Spinal Cord Vascular Diseases/diagnosis , Spinal Cord Vascular Diseases/genetics , Spinal Cord Vascular Diseases/therapy , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Telangiectasia, Hereditary Hemorrhagic/genetics , Telangiectasia, Hereditary Hemorrhagic/therapyABSTRACT
OBJECTIVE: To identify petrous internal carotid bleeding aneurysm as a complication of gasserian ganglion thermocoagulation. PATIENTS: A single case presenting with epistaxis and otorrhagia 1 month after gasserian ganglion thermocoagulation in the treatment of refractory trigeminal neuralgia. INTERVENTION(S): Gasserian ganglion thermocoagulation, computed tomographic scan, and angiocomputed tomographic scan revealing petrous internal carotid ruptured aneurysm and internal carotid embolization. MAIN OUTCOME MEASURE(S): Radiologic diagnosis of the vascular injury after gasserian ganglion thermocoagulation. RESULTS: Radiologic identification of ruptured internal carotid artery as the cause of simultaneous epistaxis and otorrhagia. CONCLUSION: Gasserian ganglion thermocoagulation may cause aneurysm and rupture of the petrous portion of the internal carotid artery.
Subject(s)
Carotid Artery Injuries/therapy , Carotid Artery, Internal/pathology , Electrocoagulation/methods , Trigeminal Ganglion/pathology , Aged , Carotid Artery Injuries/pathology , Humans , Magnetic Resonance Angiography , MaleABSTRACT
Between 1992 and 2002, 542 patients underwent a surgical treatment for hyperparathyroidism in our department. Twenty-three selective venous sampling procedures (SVS) were performed because of the failure of the other methods of diagnosis. These patients have recurrent or persistent hyperparathyroidism. Noninvasive methods of topographical diagnosis have failed or they have given contradictory results. In our experience, the surgeon needs a precise localization of the pathological glands in these difficult cases. In our series of SVS, specificity was 85.7% and sensitivity was 94.7%. Our results show that a high postoperative gradient of parathyroid hormone in the internal thoracic veins indicates an ectopic pathological gland in the thymus. A high gradient in a vertebral vein indicates a pathological superior parathyroid gland, usually in a retro-esophageal position.
Subject(s)
Blood Specimen Collection/methods , Hyperparathyroidism/diagnosis , Adult , Aged , Female , Humans , Hyperparathyroidism/surgery , Male , Middle Aged , Parathyroid Glands/abnormalities , Recurrence , Sensitivity and SpecificityABSTRACT
Bilateral inferior petrosal sinus sampling (BIPSS) is the most reliable procedure for distinguishing Cushing's disease from ectopic ACTH secretion. However, it is less reliable at predicting the lateralization of the pituitary corticotroph microadenoma. We sought to determine whether this could be improved by taking into account the pattern of venous drainage and the precise location of the catheters. We retrospectively studied data from 86 patients who underwent BIPSS. Cushing's disease was predicted in 74 patients, of whom 69 underwent transsphenoidal surgery. Surgical cure was obtained in 65 patients, with identification of a corticotroph microadenoma in 58 cases. In 49 patients the location of the microadenoma predicted by the intersinus ACTH gradient could be compared with the pathologist's data. BIPSS accurately predicted the lateralization of the microadenoma in only 57% of these patients. Prediction was improved to 71% when both venograms and catheters were symmetric (35 patients). In this subgroup accuracy was 86% in patients with both catheters in the inferior petrosal sinuses compared with 50% in patients with both catheters in the cavernous sinuses (CS). Two transient sixth nerve palsies occurred during CS catheterization. Our data suggest that BIPSS results are much improved when venous drainage is symmetric. Catheterization of CS did not improve the results and was less safe.
