Extradural Thoracic Arteriovenous Malformation in a Patient with Klippel-Trenaunay-Weber Syndrome: Case Report

Neurosurgery ◽  
2002 ◽  
Vol 51 (5) ◽  
pp. 1275-1279 ◽  
Author(s):  
Michael J. Alexander ◽  
Peter M. Grossi ◽  
Robert F. Spetzler ◽  
Cameron G. McDougall

Abstract 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.

2001 ◽  
Vol 95 (2) ◽  
pp. 236-238
Author(s):  
Natarajan Muthukumar

✓ Spinal segmental neurofibromatosis (NF) is a rare entity. To date, patients in reported cases of segmental NF (or NF5) have harbored neurofibromas involving the peripheral nerves only. The author reports a rare case of segmental NF that caused spinal cord compression in a 40-year-old woman who presented with a 6-month history of intercostal neuralgia. Examination revealed mild lower-extremity weakness and dysesthesia in the right-sided T-9 dermatome. Magnetic resonance imaging revealed three neurofibromas involving the T-9 region, which were excised, and the patient's neuralgic pain was resolved postoperatively. Traditionally, it has been believed that segmental NF involved only the peripheral nerves. The present case illustrates that although rare, spinal cord compression can also occur in patients with segmental NF.


2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Dilber Ayçiçek Çeçen ◽  
Necati Tatarlı ◽  
Hikmet Turan Süslü ◽  
Selçuk Özdoğan ◽  
Nagehan Özdemir Barışık

Background.Primary spinal dural lymphomas (PSDL) are tumors with characteristic histopathology of a lymphoma, which are completely in the spinal epidural space without any other systemic involvement. Extranodal primary lymphoma involving nervous system prefers thalamus/basal ganglia, periventricular region, cerebellum, eyes, meninges/dura, and cranial nerves or spinal cord. Rare spinal localization with acute spinal cord compression is worth attention.Case Presentation.A 48-year-old male presented with a several-month-long history of upper back pain. Lately, he had numbness and weakness at both lower extremities and was unable to walk for one week. A spinal MRI showed a thoracic lesion with cord compression at T2–T4 levels. The patient underwent surgical decompression, with his final histopathology showing diffuse large B-cell lymphoma. Systemic work-up was negative for nodal disease. Following surgery, he received radiotherapy combined with chemotherapy. He experienced a good outcome after four years.Conclusion.The upper thoracic cord is a rare location for primary spinal lesions/metastases, both of which prefer the lower thoracic and upper lumbar regions. In cases of progressive paraparesis, there should be immediate surgical intervention in the case of denovo disease, followed by combined radiotherapy and chemotherapy procedures.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Nikoletta Proudan ◽  
Kersthine Andre

Abstract Distant metastasis of follicular thyroid cancer to the bone has been well documented. However, spinal cord compression as the initial presentation of metastatic follicular thyroid cancer without any thyroid symptoms is relatively rare. Here we discuss such a case. A 78-year-old female with history of HTN and melanoma presented to the ED with a 1-month history of middle back pain that progressed to lower extremity weakness, numbness, and inability to ambulate. MRI showed a T7 vertebral mass with cord compression and edema. Metastatic work up was unremarkable except for incidental bilateral thyroid nodules, the largest on the right lobe, at 1.6 cm, with peripheral calcifications. The patient underwent T6-T7 laminectomy with vertebral decompression, partial colpectomy, and T4-T10 fusion. Pathology of the thoracic vertebral mass was positive for CAM 5.2, cytokeran 7, TTF-1, and PAX8 consistent with either metastatic pulmonary adenocarcinoma or thyroid carcinoma. The patient denied shortness of breath, dysphagia, hoarseness, or neck tenderness. She had no personal history of hyperthyroidism or hypothyroidism, or radiation exposure. She also did not have any family history of thyroid cancer. Laboratory work up was significant for TSH of 3.71 mcU/mL (0.4-4.0 mcU/mL), Free T4 1.56 ng/dL (0.7-1.9 ng/dL), thyroglobulin (Tg) 6940 ng/mL (1.6-55.0 ng/mL), and thyroglobulin antibody (Tg Ab) 20 IU/mL (0-115 IU/mL). FNA of the right thyroid nodule showed follicular neoplasm with very similar morphological features to the epidural pathology, favoring a follicular carcinoma. She underwent total thyroidectomy. Pathology showed a 1.6 x 1.1 cm follicular carcinoma with capsular and angiolymphatic invasion, but with uninvolved margins of resection. TNM staging was pT1b, pNx, pM1. She was ablated with 109 mCi of I-131 after withdrawal therapy. Whole body scan after treatment revealed radioiodine avid metastatic disease at T7 and activity in the thyroid bed compatible with residual thyroid tissue. Patient completed 10 fractions of external beam radiotherapy to the spine for a total of 30 Gy. Three months follow up lab work showed Tg 580 ng/mL and negative Tg Ab with a suppressed TSH. Thyroid bed ultrasound did not show any residual tissue or abnormal lymph nodes. Ten-year survival rates in patients with bony metastatic differentiated thyroid cancer range from 13-21% (1). Metastatic thyroid carcinoma should be considered in the differential diagnosis of every patient with new onset bony metastasis and thyroglobulin should be considered as a tumor marker in the initial work up. Research shows increased survival with I-131 avidity and complete bone metastasis resection (1). 1. Ramadan, Sami et al. “Spinal metastasis in thyroid cancer.” Head & neck oncology vol. 4 39. 25 Jun. 2012, doi:10.1186/1758-3284-4-39


Author(s):  
Tony El Murr

A 59 year old male with a past medical history of cholecystectomy and essential hypertension, presented to the emergency department with severe back pain, abdominal discomfort, poor appetite, generalized fatigue and progressive weight loss of 10 kgs over a three-month period. He has never been a smoker and drinks alcohol occasionally. He has no known allergies and no familial history of cancer. His current home medication includes beta blocker; angiotensin receptor blocker and low dose acetylsalicylic acid. His back pain started two months prior to presentation; it is not well localized to specific vertebra, not irradiating to lower limbs and sometimes related to weight lifting and cough. It is rarely exacerbated at night. His orthopedic surgeon attributed it to osteoarthritis since the patient used to practice hard manual work and weights lifting at his shop. He has been treated since that time with NSAIDs, muscle relaxant and opioids without complete analgesic response. At presentation, he had no fever, chills or night sweats. He had no urinary or sexual complaints that would suggest prostatic disease and was otherwise asymptomatic. The physical examination showed diffuse pain on lumbar vertebral percussion and abdominal tenderness in the right upper quadrant. He has no skin lesions and no palpable peripheral lymph nodes. His neurologic examination was also normal. On admission, significant laboratory findings showed hemoglobin 11.8 g/dl, platelets of 97,000 k/ul, white blood count 4,400 k/ul, ESR=40, creatinine 0.4 mg/dl,CRP 7.5 mg/L, lactate dehydrogenase 2256 U/L, alanine aminotransferase (ALAT) 70 U/L, gamma-glutamyl transferase (GGT) 1500 U/L, total bilirubin 2.32 mg/dl, and a creatine kinase (CK) 167 U/L. Prostatic specific antigen (PSA) was normal and equal to 1.17 ng/ml. Peripheral smear displayed normal pattern and thyroid tests were all within normal ranges. He was negative for salmonellosis, brucellosis and HIV. Tuberculin PPD test was negative as well. His chest X-ray was normal and abdominopelvic ultrasound showed only multiple liver nodules and mild prostate hypertrophy without ascites or significant abdominal lymph nodes. Lumbar MRI done one week before his admission revealed multiple vertebral lytic lesions without spinal cord compression. PET/CT done on the third day at hospital revealed a significant uptake of radiotracer by multiple small nodules in the right lung, in the liver and by multiple lymph nodes within the abdominal cavity. Moreover there were many lytic lesions in the dorsal and lumbar vertebrae. Surprisingly, the scan also showed focal, intense uptake of the prostate with a SUVmax of 8, 28 with evidence of seminal vesicles invasion. Both gastroscopy and colonoscopy was normal as well as his brain MRI. Ultrasound-guided liver nodules biopsies performed on the fifth day after stopping the acetylsalicylic acid revealed neuroendocrine small cell carcinoma as it showed immunohistochemical (IHC) positivity for synaptophysin and CD56. IHC was negative for TTF1 and PSA. Ultrasound-guided biopsy of the prostate was not performed for medical reasons as on his hospital day 7, the patient started feeling numbness and muscle weakness in his lower limbs more severe on the left side associated with urinary incontinence and revealing a spinal cord compression by secondary bone lesions. Regarding this rapidly progressive disease, high dose dexamethasone subcutaneous therapy and ten sessions of focused proton beam radiotherapy has been conducted on daily basis to release the spinal cord compression without significant improvement. Meanwhile, and based on liver biopsy findings, treatment by octreotide 100mg S/C twice daily was started, followed by chemotherapy with Carboplatin, Etoposide, and Atezolizumab (TECENTRIQ) 1200mg. Few days after, the patient developed severe prolonged pancytopenia requiring blood and platelets transfusions and treatment with double dose of granulocyte stimulating growth factor. His prolonged neutropenia was complicated by CMV colitis with unretractable diarrhea, bilateral pneumonia and pseudomonas aeroginosa septicemia. After one month of large spectrum antibiotics, antiviral and antifungal treatment, and while he was still neutropenic, bone marrow biopsy revealed severe infiltration by neuroendocrine small cell carcinoma. The patient was still deteriorating clinically and showing further weight and appetite loss, total muscle weakness and asthenia. At this point, he was no longer a candidate for chemotherapy but only symptomatic treatment was maintained. He died 45 days after his admission.


2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Nedal Bukhari ◽  
Bachar Harfouch ◽  
Majid Shallal Alotaibi ◽  
Hulayel Al-Harbi ◽  
Omar Chamdine

We report a case of a 31-year-old female patient with high-risk neuroblastoma (NBL) who presented with a history of static back pain and bilateral lower limb weakness for almost a month. Her primary tumor was located in the right paraspinal region, causing spinal cord compression (SCC). Chemotherapy was administered with an immediate clinical improvement noted after 24 hours of starting treatment. We herein report the efficacy of chemotherapy in an adult neuroblastoma (aNBL) patient presenting with spinal cord compression.


2020 ◽  
Vol 27 (5) ◽  
pp. 801-804
Author(s):  
Catharina Gronert ◽  
Nikolaos Tsilimparis ◽  
Giuseppe Panuccio ◽  
Ahmed Eleshra ◽  
Fiona Rohlffs ◽  
...  

Purpose: To report a case of chronic intermittent spinal cord ischemia (SCI) after thoracic endovascular aortic repair (TEVAR) and its successful treatment using hypogastric artery stenting. Case Report: A 79-year-old patient presented in May 2013 with a thoracic aortic aneurysm (TAA) and a contained rupture. He urgently underwent TEVAR that covered 274 mm of descending thoracic aorta without immediate postoperative signs of acute SCI. At 3-month follow-up, he reported repeating incidents of sudden lower extremity weakness leading to a fall with a humerus fracture. A neurological consultation revealed the tentative diagnosis of intermittent SCI caused by TEVAR and initially recommended a conservative approach. During the following year there was no clinical improvement of the symptoms. Computed tomography angiography showed a high-grade stenosis of the right hypogastric artery, which was stented in November 2014 to improve the collateral network of spinal cord perfusion. Following treatment, the patient had no further neurological symptoms; at 32 months after the reintervention, the imaging follow-up documented a patent stent and continued exclusion of the TAA. Conclusion: Intermittent neurological symptoms after TEVAR should be suspected as chronic intermittent SCI. The improvement of collateral networks of the spinal cord by revascularization of the hypogastric artery is a viable treatment option.


2021 ◽  
Author(s):  
Tyler D. Alexander ◽  
Anthony Stefanelli ◽  
Sara Thalheimer ◽  
Joshua E. Heller

Abstract BackgroundClinically significant disc herniations in the thoracic spine are rare accounting for approximately 1% of all disc herniations. In patients with significant spinal cord compression, presenting symptoms typically include ambulatory dysfunction, lower extremity weakness, lower extremity sensory changes, as well as bowl, bladder, or sexual dysfunction. Thoracic disc herniations can also present with thoracic radiculopathy including midback pain and radiating pain wrapping around the chest or abdomen. The association between thoracic disc herniation with cord compression and sleep apnea is not well described.Case PresentationThe following is a case of a young male patient with high grade spinal cord compression at T7-8, as a result of a large thoracic disc herniation. The patient presented with complaints of upper and lower extremity unilateral allodynia and sleep apnea. Diagnosis was only made once the patient manifested more common symptoms of thoracic stenosis including left lower extremity weakness and sexual dysfunction. Following decompression and fusion the patient’s allodynia and sleep apnea quickly resolved.ConclusionsThoracic disc herniations can present atypically with sleep apnea – a symptom which may resolve with surgical treatment.


2019 ◽  
Vol 10 ◽  
pp. 61
Author(s):  
Charandeep Singh Gandhoke ◽  
Siu Kei David Mak ◽  
Nishal Kishinchand Primalani ◽  
Eng Tah Goh ◽  
Hwei Yee Lee ◽  
...  

Background: Juxtafacet cysts, synovial and ganglion cysts, emanate from the facet joints. Patients with these cysts are typically asymptomatic but may rarely present with radiculopathy and/or myelopathy. Case Description: A 72-year-old female presented with a 1-month history of progressive lower extremity weakness (left more than right), numbness, and urinary incontinence. Notably, she also had a C7 sensory level to pin appreciation of 1-month duration. The magnetic resonance imaging showed an extradural C7 cystic lesion whose capsule enhanced with gadolinium, causing severe cord compression. The patient underwent a left C7 hemilaminectomy for complete excision of the cyst; postoperatively in 2-weeks duration, she regained full neurological function. The final histopathology was consistent with a ganglion cyst. Conclusion: Cervical juxtafacet cysts rarely cause compressive myelopathy. They may be readily diagnosed and resected with excellent postoperative outcomes.


Neurosurgery ◽  
2010 ◽  
Vol 66 (3) ◽  
pp. E620-E622 ◽  
Author(s):  
Alexander Taghva ◽  
Khan W. Li ◽  
John C. Liu ◽  
Ziya L. Gokaslan ◽  
Patrick C. Hsieh

Abstract OBJECTIVE Metastatic epidural spinal cord compression is a potentially devastating complication of cancer and is estimated to occur in 5% to 14% of all cancer patients. It is best treated surgically. Minimally invasive spine surgery has the potential benefits of decreased surgical approach–related morbidity, blood loss, hospital stay, and time to mobilization. CLINICAL PRESENTATION A 36-year-old man presented with worsening back pain and lower extremity weakness. Workup revealed metastatic adenocarcinoma of the lung with spinal cord compression at T4 and T5. INTERVENTION AND TECHNIQUE T4 and T5 vertebrectomy with expandable cage placement and T1–T8 pedicle screw fixation and fusion were performed using minimally invasive surgical techniques. RESULT The patient improved neurologically and was ambulatory on postoperative day 1. At the 9-month follow-up point, he remained neurologically intact and pain free, and there was no evidence of hardware failure. CONCLUSION Minimally invasive surgical circumferential decompression may be a viable option for the treatment of metastatic epidural spinal cord compression.


Sign in / Sign up

Export Citation Format

Share Document