Cobb Syndrome Research Articles

Segmental Neurofibromatosis Type 1 (NF1) Associated with Cobb Syndrome: Case Report

We present a 3-month-old girl who showed segmental NF1 and Cobb syndrome. She has a cutaneous vascular malformation located on the middle T (4)-T (6) region superimposed on a giant cutaneous café-au-lait spot. Magnetic resonance arteriography (MRA) revealed bilateral renal artery stenosis, extensive hypertrophy of the spinal epidural venous plexus, coarctation and tubular hypoplasia of the aortic arch and proximal portion of descending aorta. To the best of our knowledge the association of both neurocutaneous disorders has not being previously described.

Cobb Syndrome: A Case Report and Systematic Review of the Literature

We report on a case of Cobb syndrome, a rare metameric disorder, characterized by a vascular abnormality of the spinal cord, with an associated vascular skin lesion of the same metamere, in an 8-year-old girl presenting with leg weakness. Magnetic resonance imaging revealed a spinal arteriovenous malformation at the level of T(10)-L(5). This finding, together with a large, pigmented nevus at this level, is diagnostic for Cobb syndrome. A systematic review of the literature on Cobb syndrome, with a focus on treatment and outcomes, is presented. Current treatment options include combinations of embolization, neurosurgical intervention, corticosteroid therapy, and radiotherapy.

Clinical characteristic of spinal vascular malformation in pediatric patients

Seventy-two pediatric spinal vascular malformation cases were reviewed and the characteristics of their clinical symptoms, diagnoses, and therapies were analyzed. A thorough overview was compiled examining patient sex, age, location, history, development, treatment, clinical, and anatomical results. Spinal cord arteriovenous malformation was the most common (44.4%) subtype to be seen in these pediatric patients, while subdural perimedullary arteriovenous fistula (23.6%) was the second, followed by Cobb's syndrome (13.9%) and intramedullary cavernous angioma (5.6%). No spinal dual arteriovenous fistulae were found in infants. The highest incidence was seen during the infant and adolescent periods. Sixty-nine cases were treated by surgeries, embolizations, or a combination of both, and 71.5% of them had improved. Early diagnosis and treatment are required. Surgery and embolization, or a combination of the two, are the current candidates for treatment.

Acute paralysis in a 17‐year‐old man with subtle cutaneous vascular malformations: an unusual case of Cobb syndrome

Journal of the European Academy of Dermatology and VenereologyVolume 22, Issue 4 p. 525-526 Acute paralysis in a 17-year-old man with subtle cutaneous vascular malformations: an unusual case of Cobb syndrome DA Wetter, DA Wetter Department of Dermatology, Mayo Clinic, Rochester, MN, USASearch for more papers by this authorMDP Davis, MDP Davis Department of Dermatology, Mayo Clinic, Rochester, MN, USASearch for more papers by this authorJL Hand, JL HandSearch for more papers by this author DA Wetter, DA Wetter Department of Dermatology, Mayo Clinic, Rochester, MN, USASearch for more papers by this authorMDP Davis, MDP Davis Department of Dermatology, Mayo Clinic, Rochester, MN, USASearch for more papers by this authorJL Hand, JL HandSearch for more papers by this author First published: 26 July 2007 https://doi.org/10.1111/j.1468-3083.2007.02395.xCitations: 8 *Corresponding author, Department of Dermatology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA,E-mail: hand.jennifer@mayo.edu DOI: 10.1111/j.1468-3083.2007.02395.x Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume22, Issue4April 2008Pages 525-526 RelatedInformation

Low-back Pain and Unrecognized Cobb Syndrome in a Child Resulting in Paraplegia

Low-back Pain and Unrecognized Cobb Syndrome in a Child Resulting in Paraplegia

Cobb Syndrome in an Indian Girl

Cobb Syndrome in an Indian Girl

Segmental cutaneous hemangioma and spinal arteriovenous malformation (Cobb syndrome)

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.

The medical imaging findings in Cobb syndrome(report of 2 cases and literature review)

目的 分析Cobb综合征的影像学表现.方法 2例患者,均为男性,年龄分别为 22和13岁.临床症状为下肢无力,查体见皮肤血管瘤、下肢肌力下降.均行MRI、CT、DSA检查并经手术治疗.结果 MRI显示2例椎体多发血管瘤,T1WI呈高或略高信号,T2WI呈高信号,压脂像(STIR)呈低信号;相应节段椎管及肌肉内见不规则长T1、长T2信号,有流空现象;增强扫描椎管内病灶呈明显强化.CT平扫仅显示椎体血管瘤,表现为局部骨小梁增粗,呈网眼状,冠、矢状面重建可见栅栏征.DSA示肋间动脉、肋下动脉增粗、紊乱,周围静脉提前显影并增粗迂曲,多个椎体内可见紊乱血管团.结论 MRI能明确显示Cobb综合征椎体、椎管、脊髓、肌肉等组织的病变及其范围,结合皮肤表现对该病有诊断价值。

Embolization of multiple spinal arteriovenous malformations in Cobb syndrome causing high output heart failure: report of a case

Cobb syndrome is the combination of a vascular skin nevus and an angioma in the spinal cord corresponding to the dermatome involved. We report a case of Cobb syndrome in a female with high output heart failure who was successfully recovered by embolization with Contour emboli and microcoils.

Embolization of multiple spinal arteriovenous malformations in Cobb syndrome causing high output heart failure: report of a case

Embolization of multiple spinal arteriovenous malformations in Cobb syndrome causing high output heart failure: report of a case

Classification of Spinal Cord Arteriovenous Shunts: Proposal for a Reappraisal???The Bic??tre Experience with 155 Consecutive Patients Treated between 1981 and 1999

Spinal cord arteriovenous shunts (SCAVSs) are currently classified according to their morphological features. Certain shunts cannot be fully integrated into the predetermined categories that are usually described, however. Can these classifications be reevaluated on the basis of recent anatomic, biological, and genetic advances? We reviewed the clinical and radiological files for 155 SCAVSs that were treated at Hôpital Bicêtre between 1981 and 1999. The lesions were examined with respect to their number (single or multiple), their primary architectural type (nidus or fistula), and their possible links with associated metameric lesions. All SCAVSs were either arteriovenous malformations or fistulae, with the latter being either micro- or macrofistulae. All SCAVSs corresponded to three categories, i.e., genetic hereditary lesions (macrofistulae and hereditary hemorrhagic telangiectasia), genetic nonhereditary lesions (all of which were multiple lesions with metameric or myelomeric associations), and single lesions (which could represent incomplete presentations of one of the previous groups). Of the SCAVSs in our series, 81% were single lesions and 19% were multiple; among these, 59% were true intradural shunts with metameric features. Ten cases of Cobb syndrome, three cases of Klippel-Trenaunay syndrome, and two cases of Parkes-Weber syndrome, all with associated cord lesions, were observed. Nineteen percent of SCAVSs were fistulae; 23% of those were macrofistulae, of which 83% were related to Rendu-Osler-Weber disease. It seems legitimate to propose a categorization that takes into consideration a primary malformation (nidus or fistula) that evolves with time and in which angioarchitectural changes occur. Recognition of the factors originally responsible for the shunt (e.g., genetic hereditary or genetic nonhereditary) allows a different classification of SCAVSs.

Classification of Spinal Cord Arteriovenous Shunts: Proposal for a Reappraisal—The Bicêtre Experience with 155 Consecutive Patients Treated between 1981 and 1999

Abstract OBJECTIVE Spinal cord arteriovenous shunts (SCAVSs) are currently classified according to their morphological features. Certain shunts cannot be fully integrated into the predetermined categories that are usually described, however. Can these classifications be reevaluated on the basis of recent anatomic, biological, and genetic advances? METHODS We reviewed the clinical and radiological files for 155 SCAVSs that were treated at Hôpital Bicêtre between 1981 and 1999. The lesions were examined with respect to their number (single or multiple), their primary architectural type (nidus or fistula), and their possible links with associated metameric lesions. RESULTS All SCAVSs were either arteriovenous malformations or fistulae, with the latter being either micro- or macrofistulae. All SCAVSs corresponded to three categories, i.e., genetic hereditary lesions (macrofistulae and hereditary hemorrhagic telangiectasia), genetic nonhereditary lesions (all of which were multiple lesions with metameric or myelomeric associations), and single lesions (which could represent incomplete presentations of one of the previous groups). Of the SCAVSs in our series, 81% were single lesions and 19% were multiple; among these, 59% were true intradural shunts with metameric features. Ten cases of Cobb syndrome, three cases of Klippel-Trenaunay syndrome, and two cases of Parkes-Weber syndrome, all with associated cord lesions, were observed. Nineteen percent of SCAVSs were fistulae; 23% of those were macrofistulae, of which 83% were related to Rendu-Osler-Weber disease. CONCLUSION It seems legitimate to propose a categorization that takes into consideration a primary malformation (nidus or fistula) that evolves with time and in which angioarchitectural changes occur. Recognition of the factors originally responsible for the shunt (e.g., genetic hereditary or genetic nonhereditary) allows a different classification of SCAVSs.

Treatment with Embolization of Cobb's Syndrome Causing High-output Heart Failure

Treatment with Embolization of Cobb's Syndrome Causing High-output Heart Failure

Trifocal Monomyelomeric Spinal Cord Arteriovenous Fistulae in a Seven-Year-Old Boy

We describe a rare case of multiple arteriovenous fistulae of the spinal cord (SCAVF) in the same myelomer in a five-year-old boy. This case report consists of a trifocal SCAVF at the Th12 myelomeric level without communication between the three different fistulae. Two AVF were located posteriorly, bilateraly, in the spinal cord, fed by left and right posterior radiculopial arteries and one anteriorly in the anterior spinal axis. The venous drainage was independent for each lesion. The patient presents associated lesions characterized by cutaneous stain and inferior limb asymmetry. A metameric distribution is the explanation for the multiplicity of these lesions in a syndromic association related to Cobb syndrome. The patient was treated by transarterial embolization using glue with occlusion of the three different fistulae. The patient achieved a good improvement in neurological status.

Adult Cobb Syndrome: Case Report and Literature Review

Adult Cobb Syndrome: Case Report and Literature Review

A CASE OF COBB SYNDROME ASSOCIATED WITH RETRO-PERITONEAL GIANT LYMPHANGIOMA

A 19-year-old woman was admitted to the hospital because of abdominal distension and an intra-abdominal cystic tumor. She had several hemangiomas in the right side of the body and a history of undergo-ing a resection of cervical spinal hemangioma at the age of 13 years old, when she was diagnosed as having Cobb syndrome. On admission, numerous angiomas covering from the right jaw to right thorasic part were seen, and a soft tumor extending to under the umbilicus was palpated. Abdominal ultrasonography, CT scan, and magnetic resonance imaging revealed a multilocular cystic tumor 14cm in diameter adjacent to the right kidney. It was diagnosed as retroperitoneal giant lymphangioma. At operation, a sharply demarcated, yellowish white cystic tumor was present which was successfully removed after communication with retroperitoneal lymphatic vessel under the left renal vein was confirmed. Histopathological diagnosis was lymphangioma. Cobb syndrome consists of a vascular skin nevus associated with an angioma in spinal cord and only about 40 cases were reported in the literature. This case had hemangiomas in other than the same derma-tomes as spinal hemangioma, and the abdominal lymphangioma might be considered as a finding of Cobb syn-drome.

Paediatric Spinal Arteriovenous Malformations: Angioarchitecture and Endovascular Treatment

This is a retrospective review of the clinical records and imaging of 14 children with spinal arteriovenous malformations referred to the neurointerventional service at our institution. The lesions are categorized by anatomic location into subpial (5 cases), epidural (3 cases), and paraspinal (6 cases). There were no dural arteriovenous fistulas in this group. The subpial lesions include both the intramedullary arteriovenous malformations (2 cases) and the perimedullary arteriovenous fistulas (3 cases). Two of the patients with perimedullary fistulas were first cousins and both had Rendu-Osler-Weber syndrome. The six paraspinallesions were vertebral-vertebral fistulas with five of these located at the first cervical metamere. Eleven cases (79%) were arteriovenous fistulas and three cases (21 %) were arteriovenous malformations with a nidus. There were nine (82%) high flow arteriovenous fistulae and two (18%) low flow arteriovenous fistulae. The ages range from seven months to 15 years, with a mean age of seven years. There were nine males and five females. Clinical presentations included: bruit alone (6 patients), progressive scoliosis (1 patient), pain (2 patients), neurologic deficit (4 patients) and one case of Cobb's syndrome. Management included: no treatment (1 patient), endovascular embolisation (10 patients) and surgery (3 patients). Of the patients who underwent endovascular treatment all were treated from the arterial side. Two patients were treated by N-butyl cyanoacrylate (NBCA) alone, two with NBCA and coils, one with balloons alone, three with balloons and coils and two with coils alone. In the endovascular treatment group, nine fistulae were completely obliterated (all high flow fistulae) and one patient had partial treatment (a spinal cord arteriovenous malformation). There were no complications from endovascular treatment.

Cobb Syndrome: New Case and Historical Review

DOI: http://dx.doi.org/10.5915/30-1-16290Cobb syndrome is a combination of a skin nevus and a spinal angioma in the same segmental distribution. Although this syndrome originally was thought to be rare, its prevalence has been reported to be as high as 20% of patients with spinal angiomas in some series. We report on a case and provide an historical review of the literature to emphasize the importance of recognizing Cobb syndrome, which can lead to early diagnosis and treatment of spinal angiomas, helping to improve the prognosis.

Cobb's Syndrome: A Case Report

Cobb's syndrome is characterized by spinal and vertebral hemangiomas with a skin nevus at the same metameric level. We report a case of Cobb's syndrome in a young female patient with radiologic findings including simple X-ray , magnetic resonance imaging and angiography.

Classification and therapeutic modalities of spinal vascular malformations in 80 patients.

To set up a more detailed classification system for spinal vascular malformations and to discuss the relationship between the classification and therapeutic alternatives. Case series from the tertiary care center were studied. Spinal vascular malformations in 80 patients were reviewed and classified into intramedullary arteriovenous malformations (AVMs) (juvenile and glomus AVMs), intradural arteriovenous fistulas (AVFs) (Type I-III), dural AVFs, paravertebral AVMs, and Cobb's syndrome. Therapeutic alternatives to each type of spinal vascular malformations, including surgery, endovascular embolization, and a combination of both, as well as the short-term outcomes, were analyzed. Of the 80 patients, 38 (47.5%) had intramedullary AVMs (23 juvenile and 15 glomus AVMs), 27 (33.8%) had intradural AVFs (6 Type I, 14 Type II, and 7 Type III), 8 (10%) had dural AVFs, and 7 (8.8%) had other malformations. Most of the juvenile AVMs and the Types II and III AVFs were treated by endovascular embolization. Other lesions were treated by surgery or preoperative embolization plus surgery. Among the 76 patients whom we treated, early clinical improvement was achieved in 66 (86.8%). Six (7.9%) remained stable, and four (5.3%) became worse. Our classification system offers only limited guidance for the treatment of spinal vascular malformations. Successful treatment in each individual spinal vascular malformation requires correct understanding of the lesion's anatomic location and its angioarchitecture, as well as the limitations of both surgery and endovascular embolization.