Myelopathy due to scoliosis with vertebral hypertrophy in Klippel-Trenaunay-Weber syndrome

We describe a 37-year-old man complaining of right back pain and gait disturbance. He had a big soft tumor on his right back, hemihypertrophy of the right lower extremity, and right thoracic scoliosis. We diagnosed Klippel-Trenaunay-Weber syndrome based on the pathological findings of the soft tumor. Computed tomography (CT) scan revealed severe spinal stenosis due to a hypertrophic vertebral body and facet joint at T7. Treatment by decompression of hypertrophic bone led to complete neurological recovery. To our knowledge, no case has been reported of Klippel-Trenaunay-Weber syndrome with myelopathy which originated from thoracic scoliosis with a hypertrophic facet joint and vertebral body. We suggest that the cause of myelopathy in Klippel-Trenaunay-Weber syndrome originated not only from arteriovenous fistula, medullary angioma, and extradural hemangioma but also vertebral hypertrophy with scoliosis.     

Klippel-Trenaunay-Weber syndrome with spinal arteriovenous malformation--case report

A 28-year-old female with Klippel-Trenaunay-Weber syndrome associated with an arteriovenous malformation (AVM) of the spinal cord is reported. She was admitted to our hospital with a 4-month history of steadily progressive weakness and dysesthesia of the legs. A nevus flammeus, varices, hypertrophy and elongation of the left leg had been present since her infancy. These symptoms progressed and she became unable to walk and pass water by herself. Myelography disclosed a spinal AVM extending from Th11 to L2. Angiography confirmed the presence of stretched and tortuous vessels as well as an arteriovenous shunt in the left leg. Magnetic resonance imaging showed a high-intensity area extending from Th10 to L2. Following laminectomy from Th10 to L2 and resection of the AVM, her motor and bladder functions worsened. However, 6 months later, her motor function improved to the preoperative state and the bladder dysfunction disappeared. The coexistence of Klippel-Trenaunay-Weber syndrome with spinal AVM, considered to be rare, is discussed, and the pertinent literature is reviewed. The case presented here is the first to have been surgically treated in Japan.     

Neodymium:YAG laser irradiation of bladder hemangioma

Bladder hemangiomas may occur singly or in association with systemic arteriovenous malformations. Previously open surgical removal of these lesions has been necessary and endoscopic resection or biopsy strongly discouraged. We report a case of the Klippel-Trenaunay-Weber syndrome and a massive bladder hemangioma. Hemorrhagic complications were controlled by endoscopic application of neodymium:YAG laser photo-irradiation.     

Intracranial aneurysm in a child with Klippel-Trenaunay-Weber syndrome: case report.

The authors report a case of cerebral aneurysm in an 8-year-old boy with Klippel-Trenaunay-Weber syndrome. This syndrome is a type of neurocutaneous disorder characterized by skin hemangiomas and hypertrophy of soft tissues and/or osseous structures in the involved region. Spinal hemangioma is occasionally found in this syndrome and may cause spinal subarachnoid hemorrhage. To our knowledge, however, no case of cerebral aneurysm in a child with this syndrome has been reported previously in the literature.     

Vertebral and epidural hemangioma with paraplegia in Klippel-Trenaunay-Weber syndrome. Case report.

A patient with a rare case of Klippel-Trenaunay-Weber syndrome presented with paraplegia due to compression by a vertebral and epidural cavernous hemangioma. The metameric distribution of the large cutaneous vascular nevus provided the clinical clue to the nature of the spinal lesion. The association of the two lesions is explained in the basis of developmental anomaly.     

Extradural thoracic arteriovenous malformation in a patient with Klippel-Trenaunay-Weber syndrome: case report

OBJECTIVE AND IMPORTANCE: Spinal cord involvement in Klippel-Trenaunay-Weber (KTW) syndrome is rare. Cases of intradural spinal cord arteriovenous malformations (AVMs) have been associated with this syndrome. Likewise, cases of epidural hemangioma and angiomyolipoma have been reported to occur at the same segmental level as cutaneous hemangioma in KTW syndrome. This report details a rare case of an extradural thoracic AVM in a patient with KTW syndrome. CLINICAL PRESENTATION: A 30-year-old man presented with a 10-month history of progressive myelopathy, bilateral lower-extremity weakness, and numbness, with the right side affected more than the left. His symptoms had progressed to the point that he was unable to walk. The patient had the characteristic manifestations of KTW syndrome, including numerous cutaneous angiomas and cavernomas, limb hypertrophy and syndactyly, and limb venous malformations. A magnetic resonance imaging scan and subsequent angiogram demonstrated a large extradural AVM causing cord compression at the T3-T4 levels. INTERVENTION: The patient underwent two separate endovascular procedures, including embolization of upper thoracic and thyrocervical trunk feeders. Subsequently, he underwent T1-T4 laminectomy and microsurgical excision of the AVM. Clinically, the patient improved such that he could walk without assistance. CONCLUSION: KTW syndrome represents a spectrum of clinical presentations. Although involvement of the spinal cord is uncommon, the manifestations of this syndrome may include both intradural and extradural AVMs in addition to various tumors.     

Segmental cutaneous hemangioma and spinal arteriovenous malformation (Cobb syndrome). Case report and historical perspective

The discovery of either a dermatomal cutaneous nevus or a spinal arteriovenous malformation (AVM) should raise the suspicion of Cobb syndrome. The Cobb syndrome is a neurocutaneous syndrome in which there are metameric cutaneous and spinal AVMs. The authors present the case of a patient with acute cervical myelopathy and subtle cutaneous hemangiomas in whom a cervical perimedullary fistula was discovered. They also acknowledge Harvey Cushing's contribution to the recognition of this syndrome.     

Klippel-Trenauney-Weber syndrome associated with hemoptysis

We report a case of Klippel-Trenaunay-Weber syndrome presenting with hemoptysis.     

Paraplegia in pregnancy: a case of spinal vascular malformation with klippel-trenaunay syndrome

STUDY DESIGN.: A case report. OBJECTIVE.: To present improvement of paraplegia due to spinal vascular malformation after delivery with no intervention. SUMMARY OF BACKGROUND DATA.: Pregnancy has been reported rarely in patients with Klippel-Trenaunay syndrome (KTS). A combined case of spinal arteriovenous malformations (AVMs) within KTS has not been reported before. METHODS.: A case report of KTS with paraplegia is presented and the pertinent literature is then reviewed. RESULTS.: A pregnant woman who was diagnosed with KTS in childhood presented with newly developed paraplegia due to spinal AVMs. Magnetic resonance image showed a vascular malformation at the T9-T12 levels without evidence of spinal cord hemorrhage. Diagnostic angiography that was performed after delivery revealed a high-flow arteriovenous fistula with AVM. CONCLUSION.: Pregnancy complicated by paraplegia is thought to be secondary to venous engorgement and to the resulting spinal cord ischemia in this case. Her neurological symptoms and signs gradually improved over the few days after cesarean delivery.     

Traumatic spinal perimedullary arteriovenous fistula: a case report

Spinal perimedullary arteriovenous fistula, a subtype of spinal arteriovenous malformation, is generally considered to be a congenital lesion by maldevelopment of the spinal vascular system. However, several cases of perimedullary arteriovenous fistula had been reported in the literature, which were thought to be acquired lesions. We presented a case of perimedullary arteriovenous fistula, which was believed to be derived from spinal cord trauma. The etiology and pathophysiology of this case were discussed, and it might provide further insight into the pathogenesis of such lesions.     

Neurofibromatosis with spinal paralysis due to arteriovenous fistula

Summary A case of neurofibromatosis with spinal paralysis due to arteriovenous fistula is reported. Preoperatively, the appropriate diagnosis was missed because angiography had not been performed. Post-operative angiography disclosed that an arteriovenous fistula formed a tumor-like mass, and that a part of the mass had invaded the spinal canal, compressing the spinal cord. It is concluded that the possibility of an arteriovenous fistula should be kept in mind in neurofibromatosis patients with spinal cord symptoms.     

Spontaneous thrombosis of a spinal conus perimedullary arteriovenous fistula. Case report

A 49-year-old male presented with a rare case of acute lower extremity paresis caused by spontaneous thrombosis of a spinal conus perimedullary arteriovenous fistula (AVF) after a subacute myelopathic course. Magnetic resonance imaging obtained after deterioration showed that the flow voids around the conus medullaris had changed from hypointense to hyperintense lesions. Surgery with thoracic laminoplasty was performed to determine the nature of the lesion because angiography was negative. During surgery, thrombosed abnormal vessels were observed, consistent with thrombosis of a spinal conus perimedullary AVF. Histological specimens of the thrombosed vessels exhibited vascular wall injury such as intimal alteration, wall dissection, and mural thrombus. Hemorrhage and infection were excluded. Vascular wall injury of draining veins and varices were probably one of the causes of thrombosis in the present case. Spinal arteriovenous malformation generally causes progressive venous congestive myelopathy, but the congestive myelopathy may rarely rapidly deteriorate with spontaneous thrombosis, known as Foix and Alajouanine syndrome.     

Obliteration of a tentorial dural arteriovenous fistula causing spinal cord myelopathy using the cranio-orbito zygomatic approach

BACKGROUND: Intracranial dural arteriovenous fistulas account for 10 to 15% of all intracranial arteriovenous malformations. Tentorial dural arteriovenous fistulas with spinal medullary venous drainage causing spinal cord myelopathy are very rare, but have been previously described. We describe a case using a cranio-orbito zygomatic approach with intraoperative angiography for the surgical treatment of a tentorial artery dural arteriovenous fistula causing spinal cord myelopathy. CASE PRESENTATION: A 42-year-old male presented complaining of a 1-year history of incoordination and dizziness and a 2-month history of progressive myelopathy with bowel and bladder incontinence. The patient had magnetic resonance imaging (MRI) performed along with cerebral and spinal angiography that revealed a right tentorial artery dural arteriovenous fistula with spinal medullary venous involvement down to T11. Angiographic embolization was attempted, but selective catheterization was unsuccessful. The patient underwent a cranio-orbito zygomatic approach with obliteration of the dural arteriovenous fistula. An intraoperative angiogram confirmed complete obliteration of the dural arteriovenous fistula. CONCLUSION: Intracranial dural arteriovenous fistulas are a rare cause of spinal cord myelopathy. When a patient presents with suspicion of spinal dural fistula and negative spinal angiography, an intracranial origin should be suspected and a cerebral angiogram performed. Skull base approaches along with intraoperative angiography provide an alternative modality for obliteration of the dural arteriovenous fistula nidus, thereby eliminating the venous congestion and hence the spinal cord ischemia.     

Spinal cord arteriovenous malformation presenting as chest pain in a child

STUDY DESIGN: A case report of spinal arteriovenous malformation presented as chest pain in a child. OBJECTIVES: To illustrate the atypical clinical presentation as right chest pain in a child with spinal cord arteriovenous malformation. SETTING: Chang Gung Memorial Hospital and Chang Gung University, Taiwan. CASE REPORT: An 8-year-old boy was diagnosed with spinal cord arteriovenous malformation after paraplegia. However, early diagnosis was delayed for the atypical initial clinical presentation as chest pain. After surgery and rehabilitation for 6 months, the patient could walk without assistance. CONCLUSIONS: Correct diagnosis of spinal cord arteriovenous malformations is sometimes difficult in children with atypical presentations. Early diagnosis relies on detailed examination and high index of suspicion.     

Vesical hemangioma in patient with Klippel-Trenaunay-Weber syndrome

Klippel-Trenaunay-Weber syndrome is characterized by cutaneous hemangiomas, varicosity and bony hypertrophy of extremities. Urinary tract hemangiomas may occur in 3 to 6% of these patients. This work intends to report a case of a patient with a huge vesical hemangioma, who presented this syndrome. A 5 year-old boy with Klippel syndrome sought our services due to 3 episodes of gross hematuria in the past 30 days. Excretory urography and computerized tomography were performed, indicating the presence of a swelling in vesical dome. An exploratory cystotomy was conducted and the dark colored mass in vesical dome was excised by partial cystectomy. The histopathologic finding confirmed that it was a vesical hemangioma. Though rare, urinary tract hemangiomas must always be considered in patients with Klippel-Trenaunay syndrome.     

Spinal dural arteriovenous fistula and venous aneurysm: an unusual association

OBJECTIVE: Although spinal intradural arteriovenous malformations have frequently been associated with venous aneurysms, spinal dural arteriovenous fistulas typically are not. We describe a case of conus medullaris compression by a large saccular venous aneurysm of a spinal dural arteriovenous fistula. As these lesions are slow-flow arteriovenous shunts, development of a venous aneurysm is an unexpected condition. Pathogenesis of these aneurysms is briefly discussed. METHODS: A 61-year-old man presented with progressive spastic paraparesis of 1-year duration. Spinal magnetic resonance (MR) suggested abnormal serpiginous vessels and a partially thrombosed aneurysm at the level of conus medullaris. Spinal angiography was performed but some lower thoracal and upper lumbar segments could not be catheterized because of severe atherosclerosis. As a result, vascular anomaly could not be demonstrated angiographically, but surgical exploration was found to be necessary because of positive clinical and MR findings. RESULTS: An arteriovenous fistula that entered the spinal canal beneath the L1 pedicle on the left was identified. It was easily coagulated and cut. The partially thrombosed aneurysm within the conus medullaris was also resected. CONCLUSIONS: Our case illustrates two important points related to patients harboring spinal dural arteriovenous fistulas (AVF): First, clinical suspicion and MR findings are important in diagnosis of these lesions and surgical exploration is indicated despite negative or nondiagnostic angiographic results in such cases. Second, venous aneurysms may be associated with spinal dural AVFs, and although spinal dural AVFs are low-flow lesions, the development of the venous aneurysm is probably a result of high venous pressure.     

Obstetric management of Klippel-Trenaunay syndrome. A case report

A rare case of pregnancy in a patient with Klippel-Trenaunay-Weber syndrome is described. The arterio-venous anomalies in this patient originally occurred in her left leg. Her pregnancy was uneventful. A Cesarean section was performed at term. There were no signs of intravascular coagulation or cardiac decompensation. Prophylactic anticoagulant treatment was given during the 3rd trimester and into the early puerperium The clinical course and the risks of a pregnancy with this condition are discussed. Conclusion: Klippel-Trenaunay syndrome was once thought to be a contraindication to pregnancy. With careful management, successful pregnancies can be achieved.     

Spinal dural arteriovenous fistula with hematomyelia caused by intraparenchymal varix of draining vein

Background ContextHemorrhage that results from spinal dural arteriovenous fistula (Type I arteriovenous malformation [AVM]) is uncommon. There are some reports of subarachnoid hemorrhage and subdural hematoma caused by Type I spinal AVM, but there are few reported cases of hematomyelia caused by spinal dural arteriovenous fistula.PurposeTo describe an interesting patient who had hematomyelia caused by a dural arteriovenous fistula (Type I spinal AVM).Study designA case report.MethodsWe present a case of a 51-year-old man who presented acute onset epigastric pain, paraplegia, and sensory loss below his nipples. Magnetic resonance imaging and selective spinal angiogram demonstrated hematomyelia, subarachnoid hemorrhage, and spinal arteriovenous fistula fed by the right Th7 intercostal artery. By laminotomy of Th6–8, the varix-like draining vein and intramedurally hematoma were partially removed and the arterial supply was interrupted by coagulation of the right Th7 segmental artery.ResultsOne month after surgery, he regained movement against gravity at the left ankle and toe but no functionally significant improvement.ConclusionsIt must be kept in mind that spinal dural arteriovenous fistulas (Type I spinal AVM) has possibility of hematomyelia origin, despite the fact that it is extremely rare.     

Angiogenic and inflammatory factor expressions in cutaneomeningospinal angiomatosis (Cobb’s syndrome): case report

We report on a case of cervical cutaneomeningospinal angiomatosis (Cobb’s syndrome), a rare somatic disorder, characterized by vascular abnormalities of the spinal cord, with a triad of associated vascular skin, muscle, bone, and dura involvement at the same somite. This case follows an 18-year-old male patient presenting with left extremity weakness and back cervical pain. Magnetic resonance imaging (MRI) revealed a spinal cord arteriovenous malformation (AVM) at the C3-C5 level. Cobb’s syndrome was diagnosed by coexistence of cutaneous naevi in a dermatomal pattern and neurological signs of a spinal cord lesion together with cervical MRI and angiography. The patient underwent a combination of staged endovascular embolization and microsurgical resection. Multiple biopsies of the mass including the skin, muscle, dura, and spinal cord at the same somite revealed that the lesions had a similar pathology. Post-operative immunohistochemical characterizations on specimen included CD31, smooth muscle actin (SMA), vascular endothelial growth factor (VEGF), and matrix metalloproteinase (MMP-9). The unique associations of somatic and spinal cord lesion as well as angiogenic and inflammatory factor expressions in all specimens are reported.     

Sciatic nerve enlargement in the Klippel-Trenaunay-Weber syndrome.

The case of a 35-year-old woman with Klippel-Trenaunay-Weber syndrome (KTWS) showing clinical symptoms of a peroneal nerve lesion is presented. An immense nerve enlargement along most of the sciatic, peroneal and tibial nerve was found to be due to a lipoma arising from the epi- and perineurium. Treatment consisted of extensive microsurgical neurolysis and excision of the tumor resulting in decompression of the affected nerves. Although rare, a perineural lipoma should be kept in mind in patients with KTWS showing neurological abnormalities.