A Difficult Spinal Vascular Malformation: Challenges in Its Endovascular Management

Sir, An 11-year-old male child, a product of nonconsanguineous marriage, known case of CLOVES syndrome with D12–L1 spinal perimedullary fistula. Initially, the child presented with complaints of backache and bilateral lower limb weakness. Magnetic resonance imaging (MRI) and digital subtraction angiogram evaluation revealed perimedullary arteriovenous fistula at conus with extensive epidural and paravertebral venous drainage and venous hypertension of the cord. The patient underwent embolization in 2016 at the age of 3 years with Guglielmi detachable coil and glue. In the postintervention phase, the patient developed conus and epiconus syndromes. His residual weakness of the left lower limb remained. The child was mobile without support. In the follow-up evaluation for progressive weakness and pain in lower limbs (left > right), recurrence of the perimedullary fistula at the same site was identified. Clinical examination revealed overgrowth and edema of the left lower limb, nevi at the left half of the body, and other stigmata of CLOVES syndrome. The patient underwent MRI and spinal angiography revealing left D10 hypertrophied feeders leading to fistula. The child was taken up for endovascular embolization under general anesthesia in a separate sitting after appropriate counseling of the parents and obtaining informed high-risk consent. The right common femoral artery puncture was done. 5 F short sheath inserted. 5 F × 65 cm Cobra catheter used for diagnostic spinal angiogram. Once the magnified images and three-dimensional projections for treatment planning were obtained, the fistulous site was accessed through magic microcatheter. Under intermittent fluoroscopic guidance, NBCA/glue was injected under negative road map. Postprocedure angiogram revealed complete obliteration of the fistula and the presence of the glue cast. In the postoperative period, the patient was given anticoagulation for 24 h to prevent rapid thrombosis. He was put on injection methylprednisolone for 24 h as anti-edema measure. Postprocedure period was uneventful. The patient experienced reduction in clinical symptoms and limb weakness during his course in the hospital. The patient got discharged 3 days postprocedure. Spinal vascular diseases are rare entities. Depending on the type of spinal vascular disease, initial symptoms may vary between acute and chronic onset. The intradural perimedullary arteriovenous fistulas (IPAVFs) were first described by Djindjian et al.,[1] in 1977. IPAVF prevalence is approximately 8%–19% of spinal vascular shunts.[2] Syndromic associations with IPAVFs have been reported, including Osler–Weber–Rendu, Proteus, Klippel–Trenaunay, and Down syndromes. In the spinal perimedullary fistula, T2-weighted MRI demonstrates prominent perimedullary flow voids typically along the ventral cord surface, in contrast to, spinal dural arteriovenous fistulas, which are typically dorsal. These lesions consist of a single giant fistula supplied by multiple, enlarged feeders from the anterior and posterolateral spinal arteries. They are located on the thoracic cord or less commonly the cervical cord.[3] Therapy generally consists of endovascular embolization, surgery, or a combination of both. Because type IV lesions are rare, published series are limited, and firm conclusions about treatment cannot be made. Most authors recommend prompt diagnosis and treatment of perimedullary fistulae to minimize neurological injury. In our case, the neurosurgery team felt the lesion was not fit for surgical intervention. Hence, the case was taken for endovascular management, and the lesion was successfully cured. Repeat endovascular treatment for this condition is rarely described. The case presents an interesting and rare case. Few case reports describe CLOVES syndrome and management of the associated spinal vascular malformations.[4] CLOVES syndrome is an acronym for congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal/scoliosis/spinal anomalies. The condition is considered an overgrowth syndrome. Abnormalities have a truncal predominance and can have associated spinal vascular malformations (like in our case) [Figures 1-4].Figure 1: Clinical: CLOVES syndrome and magnetic resonance imaging T2WI showing fistula, Blue arrows are showing spinal vascular malformationFigure 2: Extent of fistula depicted in the lower dorsal and lumbar spine: T2WI and T1W1 sagittal view spine magnetic resonance imaging, Blue arrows are showing spinal vascular malformationFigure 3: Selected catheter angiogram showing spinal perimedullary fistula at D10 with venous egress. Note is made of previous coiling (with recurrence) status of the fistula, Blue arrows are showing spinal vascular malformationFigure 4: Post- N Butyl Cyanoacrylate embolization of the fistula showing complete obliteration of the spinal perimedullary fistula, Spinal angiogramDeclaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.