Surgical Treatment of Spinal Dural Arteriovenous Fistulae: A Consecutive Series of 154 Patients

Abstract BACKGROUND: Embolization of spinal dural arteriovenous fistulae (SDVAFs) has emerged as an alternative to surgery. However, surgical disconnection is a simple and effective procedure. OBJECTIVE: To review results and complications of surgical treatment of 154 consecutive SDAVFs. METHODS: The records of 154 consecutive patients with SDAVFs were retrospectively reviewed. RESULTS: There were 120 males and 34 females (male/female ratio 3.5:1, mean age 63.6 years). The SDAVFs were located at the thoracic level in 92 patients and at the lumbar and sacral spine levels in 45 and 15 patients, respectively. The most common presenting symptoms were motor dysfunction (65 patients), sensory loss (31 patients), and paresthesias without sensory loss (13 patients). The mean interval from symptom onset to definitive diagnosis was 24.7 months (median 12 months). Surgery resulted in complete exclusion of the fistula at first attempt in 146 patients (95%). There were no deaths or major neurological complications related to the surgery. Six percent of patients experienced subjective or objective worsening of preoperative symptoms and signs by the time of discharge that persisted at follow-up. Other surgical complications consisted of wound infection in 2 patients and deep venous thrombosis in 3. Eight patients were lost to follow-up; 141 patients (96.6%) experienced improvement (120 patients, 82.2%) or stability (21 patients, 14.4%) of motor function at last follow-up compared with their preoperative status. Other symptoms such as numbness, sphincter dysfunction, and dysesthesias/neuropathic pain improved in 51.5%, 45%, and 32.6%, respectively. CONCLUSION: Surgical obliteration of SDAVFs is safe and very effective. Prognosis of motor function is favorable after surgical treatment.

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MNG-08 VOLUMETRIC STUDIES IN ASYMPTOMATIC MENINGIOMAS: SLOWDOWN CASES AND GROWTH ARREST CASES

Neuro-Oncology Advances ◽

10.1093/noajnl/vdz039.164 ◽

2019 ◽

Vol 1 (Supplement_2) ◽

pp. ii36-ii36

Author(s):

Ryuichi Hirayama ◽

Tomoyoshi Nakagawa ◽

Toru Umehara ◽

Chisato Yokota ◽

Noriyuki Kijima ◽

...

Keyword(s):

Surgical Treatment ◽

Growth Arrest ◽

Surgical Excision ◽

Volume Measurement ◽

Female Ratio ◽

Volume Increase ◽

Volume Curve ◽

Increase Rate ◽

Mri Scans

Abstract BACKGROUND The opportunity to follow up for asymptomatic meningiomas has increased. We have reported the risk of volume increase by individual continuous volume measurement of asymptomatic meningiomas. However, We have not reached fully understanding about natural history of meningiomas. Among cases are followed up over time, there are some cases that the volume increase rates slows down or almost stops are observed. METHODS We enrolled consecutive adult patients of asymptomatic meningiomas who follow-up for 2 years or more and 3 or more MRI scans. We performed sequential volumetric measurements on 95 patients (105 lesions) who met the criteria. We classified these transient volume curve of each lesion into three groups “Growing”, “Slowdown”, and “Growth arrest” for analysis. RESULTS The average age at the first visit was 62.8 years, the average follow-up period was 61.8 months, and the male-female ratio was 20:75 (male: female). There were 67 cases (73 lesions: 70.9%) that were in increasing trend, and 19 cases of those were received resection. Eleven cases (12 lesions: 11.7%) showed a tendency of “slow down” the increase rate, and one patient who became symptomatic led to surgical excision. In 18 cases (18 lesions: 17.4%) in which almost no volume change was observed during the observation period, no cases resulted in surgical treatment. CONCLUSIONS Among the meningiomas cases that have been followed for a long time, there are not a few those increase rate of tumor volume slows or does not change. Furthermore, most of these cases did not result in surgical treatment. The presence of these “Slowdown” and “Growth arrest” cases at a certain rate may have suggested the possibility of a Gompertz curve model as the natural course of meningiomas.

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Surgical Treatment of Childhood Inflammatory Myofibroblastic Tumors

European Journal of Pediatric Surgery ◽

10.1055/s-0036-1593380 ◽

2016 ◽

Vol 27 (04) ◽

pp. 319-323 ◽

Cited By ~ 7

Author(s):

Beril Talim ◽

İbrahim Karnak ◽

Saniye Ekinci ◽

Fatih Andiran ◽

Arbay Çiftçi ◽

...

Keyword(s):

Surgical Treatment ◽

Lower Lobe ◽

Diagnostic Methods ◽

Incomplete Resection ◽

Total Resection ◽

Presenting Symptoms ◽

Inflammatory Myofibroblastic Tumors ◽

The Mean

Aim A retrospective study was performed to evaluate the clinical features, diagnostic methods, and treatment alternatives of childhood inflammatory myofibroblastic tumors (IMTs). Patients and Methods Patients who underwent surgical treatment for IMT between 2000 and 2015 were evaluated for age, sex, presenting symptoms, physical examination findings, diagnostic methods, treatment modalities, histopathologic findings, and results of surgical treatment during long-term follow-up. Results Eleven patients who underwent surgical treatment were included in the study. Male:female ratio was 7:4 and the mean age of the patients was 6.09 years (1–10 years). Presenting symptoms were respiratory difficulty, cough (n = 7, 63.3%), abdominal pain, vomiting (n = 2, 18.8%), loss of body weight (n = 1, 9.09%), palpable mass (n = 1, 9.09%), and rectal bleeding (n = 1, 9.09%). Ultrasonography (n = 4, 36.3%) and computed tomography (n = 9, 81.1%) were used for diagnosis. Localizations of tumors were lungs (n = 5, 45.4%), mediastinum (n = 2, 18.1%), spleen (n = 1, 9.09%), neck (n = 1, 9.09%), colon (n = 1, 9.09%), and rectum (n = 1, 9.09%). The mean size of mass was 6.6 cm (2–12 cm) and six patients were diagnosed with preoperative biopsy. Lung lobectomy (right lower lobe; n = 3, right middle and lower lobe; n = 2), total resection of mass with adjacent bowel (n = 2), partial splenectomy (n = 1), total resection of neck mass (n = 1), and incomplete resection (n = 2) were the choice of surgical treatment. Incomplete resection was performed in masses closely adjacent to atrium and mediastinal structures. In histopathologic evaluation, surgical margins were free of tumor in four cases, positive in six cases, and were not reported in one case. Anaplastic lymphoma kinase (ALK) positivity was detected in six cases, negative in two cases, and was not evaluated in three cases. Two cases who had residual mass with positive ALK received chemotherapy. Mean follow-up time was 68.2 months (5 months to 12 years). During follow-up, there was no recurrence or distant metastasis. Ten patients survived and one patient was lost to follow-up. Conclusion IMT is a rare tumor of childhood with a spectrum of clinical findings because of variable localization. Surgical treatment is the first choice of treatment. Patients with residual mass and ALK positivity may require medical treatment. In our series, long-term survival of patients was favorable in patients with total resection.

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Cervical Laminoplasty as a Management Option for Patients With Cervical Spondylotic Myelopathy

Neurosurgery ◽

10.1227/01.neu.0000371981.83022.b1 ◽

2010 ◽

Vol 67 (2) ◽

pp. 272-277 ◽

Cited By ~ 16

Author(s):

Anthony L. Petraglia ◽

Vasisht Srinivasan ◽

Michelle Coriddi ◽

M. Gordon Whitbeck ◽

James T. Maxwell ◽

...

Keyword(s):

Cervical Spondylotic Myelopathy ◽

Medical Center ◽

Demographic Data ◽

Adult Population ◽

Postoperative Outcome ◽

Outcome Data ◽

Consecutive Series ◽

Cervical Laminoplasty ◽

Presenting Symptoms

Abstract BACKGROUND Cervical spondylotic myelopathy (CSM) is one of the leading causes of spinal cord dysfunction in the adult population. Laminoplasty is an effective decompressive procedure for the treatment of CSM. OBJECTIVE We present our experience with 40 patients who underwent cervical laminoplasty using titanium miniplates for CSM. METHODS We performed a retrospective review of the medical records of a consecutive series of patients with CSM treated with laminoplasty at the University of Rochester Medical Center or Rochester General Hospital. We documented patient demographic data, presenting symptoms, and postoperative outcome. Data are also presented regarding the general cost of constructs for a hypothetical 3-level fusion. RESULTS Forty patients underwent cervical laminoplasty; all were available for follow-up. The mean number of levels was 4. All patients were myelopathic, and 17 (42.5%) had signs of radiculopathy preoperatively. Preoperatively, 62.5% of patients had a Nurick grade of 2 or worse. The average follow-up was 31.3 months. The median length of stay was 48 hours. On clinical evaluation, 36 of 40 patients demonstrated an improvement in their myelopathic symptoms; 4 were unchanged. Postoperative kyphosis did not develop in any patients. CONCLUSION The management of CSM for each of its etiologies remains controversial. As demonstrated in our series, laminoplasty is a cost-effective, decompressive procedure for the treatment of CSM, providing a less destabilizing alternative to laminectomy while preserving mobility. Cervical laminoplasty should be considered in the management of multilevel spondylosis because of its ease of exposure, ability to decompress, effective preservation of motion, maintenance of spinal stability, and overall cost.

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Case of a Giant Prolactinoma Measuring Greater Than 10 CM in a Post-Menopausal Woman

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.1173 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A575-A575

Author(s):

Nasvin Imamudeen ◽

Aiman Zafar ◽

Shankar Bettadahalli

Keyword(s):

Medical Therapy ◽

Prolactin Level ◽

Night Vision ◽

Serum Prolactin ◽

Menopausal Woman ◽

Female Ratio ◽

Presenting Symptoms ◽

The Right ◽

The Brain

Abstract Prolactinomas are the most common type of pituitary adenomas. Macroprolactinoma is the name used for tumors when their size exceeds 1 cm and giant prolactinomas are those that exceeds 4 cm. Females are more commonly reported to have microprolactinomas with female-to-male ratio of 20:1, on the contrary macroprolactinomas are usually diagnosed in men aged 20-50 years with a reported male-to-female ratio of 9:1. We are presenting the case of a 63-year-old female with history of cataracts and hypertension who presented with complaints of progressive decrease in vision in both of her eyes (right greater than left) for several months and declining night vision without headache. Her only other symptoms were tiredness and cold intolerance. A detailed visual exam led to the findings of bitemporal hemianopsia and possible right optic neuropathy. Her neurological exam otherwise was intact. Subsequently contrast magnetic resonance imaging (MRI) of the brain showed a large sellar and suprasellar mass measuring greater than 10 cm x 6 cm in size invading right cavernous sinus, surrounding vessels and compressing optic chiasm with extension into the right temporal lobe, left frontal lobe and subfalcine shift to the right. Laboratory studies indicated elevated prolactin level of 4932 ng/mL (2.8 - 29.2 ng/mL) consistent with prolactinoma. Pituitary hormone function testing revealed suppressed gonadotropins, subnormal ACTH stimulation test and central hypothyroidism. She was started on medical therapy with cabergoline. At one month follow-up, serum prolactin level significantly decreased to 136.2 ng/mL but the size of the mass did not significantly decrease on follow up brain MRI. Patient currently wants to try medical management alone. Conclusion: Macroprolactinomas measuring >4 cm are rare, accounting for only 1–5% of all prolactinomas and are more commonly seen in men, however they can be seen in postmenopausal women as well. Headaches and visual field disturbances are common presenting symptoms due to compressive effect and warrant further investigation with MRI of the brain. Medical therapy alone can sometimes suffice and surgery is a second line option as it confers morbidity risks.

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NEUROSURGICAL TREATMENT FOR ANEURYSM REMNANTS OR RECURRENCES AFTER COIL OCCLUSION

Neurosurgery ◽

10.1227/01.neu.0000325499.82876.6d ◽

2008 ◽

Vol 63 (4) ◽

pp. 684-692 ◽

Cited By ~ 30

Author(s):

Jean-Paul Lejeune ◽

Laurent Thines ◽

Christian Taschner ◽

Philippe Bourgeois ◽

Hilde Henon ◽

...

Keyword(s):

Surgical Treatment ◽

Glasgow Outcome Scale ◽

Postoperative Outcome ◽

Consecutive Series ◽

Anterior Circulation ◽

Microsurgical Treatment ◽

Coil Occlusion ◽

Neurosurgical Treatment ◽

Temporary Occlusion

ABSTRACT OBJECTIVE Neurosurgical management of residual aneurysms (RA) after coiling remains a challenging issue. We present a consecutive series of 21 patients who underwent microsurgical treatment of a previously coiled aneurysm. METHODS We retrospectively reviewed a consecutive series of 21 patients who underwent operations for an RA after coiling between 1997 and 2007. Postcoiling follow-up imaging included brain magnetic resonance angiography and digital subtraction angiography. The decision for surgical treatment was made when an RA was significant and unsuitable for re-embolization. Data related to the RA and to the surgical technique were analyzed. Postoperative outcome was evaluated with the Glasgow Outcome Scale. RESULTS Twenty aneurysms were initially ruptured. Twelve had undergone complete coil occlusion, whereas 6 had a residual neck, 2 had a residual lobule, and 1 had a residual sac. The aneurysms were in the anterior circulation in 18 cases and in the posterior circulation in 3 cases. Twenty RAs were excluded with the apposition of 1 clip beneath the coils, 2 required a temporary occlusion, 2 required extraction of the coils, and 1 presented with an operative rupture. All aneurysms, except 2 that had their residual neck wrapped, were completely occluded. The postoperative Glasgow Outcome Scale score was unchanged in 90% of patients, and 2 patients sustained a moderate disability. CONCLUSION Microsurgical treatment of RA after endovascular treatment is effective, provided that patients are selected appropriately. The surgical treatment of recanalized aneurysms after coiling is challenging but can result in a good outcome with low morbidity and no mortality.

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A TWENTY-FIVE YEAR FOLLOW-UP STUDY ON THE HYPERKINETIC CHILD WITH MINIMAL BRAIN DYSFUNCTION

PEDIATRICS ◽

10.1542/peds.39.3.393 ◽

1967 ◽

Vol 39 (3) ◽

pp. 393-399

Author(s):

Miriam M. Menkes ◽

Jane S. Rowe ◽

John H. Menkes

Keyword(s):

Brain Dysfunction ◽

Motor Dysfunction ◽

Minimal Brain Dysfunction ◽

Speech Impairment ◽

Presenting Symptoms ◽

Follow Up Study ◽

Short Attention Span ◽

Hyperkinetic Child ◽

Visual Motor

Eighteen patients who had been evaluated at a child psychiatry clinic a mean of 24 years ago were selected for follow-up by the following criteria: the presenting symptoms had been hyperactivity and short attention span, none had seizures, all had an I.Q. over 70, and all had indications of neurologic abnormalities such as poor coordination, visual motor dysfunction, or speech impairment. Eleven of the 18 were reexamined fully and an interview or information was obtained about three more. Neurologic examination showed definite abnormalities in eight subjects and suspicious findings in one, but it was normal in two. Hyperactivity was still present in three subjects aged 22 to 23 years and had disappeared between the ages of 8 and 21 in the others. Four subjects were institutionalized as psychotic, two others were retarded and supported by their families, and eight were self-supporting. Of the latter, four have spent some time in institutions. A major prognositc factor was the I.Q. obtained during initial evaluation; all but one of the subjects who were self-supporting had scored an I.Q. above 90.

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Prevalence and natural history of arachnoid cysts in adults

Journal of Neurosurgery ◽

10.3171/2012.10.jns12548 ◽

2013 ◽

Vol 118 (2) ◽

pp. 222-231 ◽

Cited By ~ 97

Author(s):

Wajd N. Al-Holou ◽

Samuel Terman ◽

Craig Kilburg ◽

Hugh J. L. Garton ◽

Karin M. Muraszko ◽

...

Keyword(s):

Natural History ◽

Brain Mri ◽

Arachnoid Cysts ◽

Consecutive Series ◽

Middle Fossa ◽

Presenting Symptoms ◽

History Analysis ◽

Number Of Patients ◽

History Of

Object Arachnoid cysts are a frequent finding on intracranial imaging. The prevalence and natural history of these cysts in adults are not well defined. Methods We retrospectively reviewed the electronic medical records of a consecutive series of adults who underwent brain MRI over a 12-year interval to identify those with arachnoid cysts. The MRI studies were reviewed to confirm the diagnosis. For those patients with arachnoid cysts, we evaluated presenting symptoms, cyst size, and cyst location. Patients with more than 6 months' clinical and imaging follow-up were included in a natural history analysis. Results A total of 48,417 patients underwent brain MRI over the study period. Arachnoid cysts were identified in 661 patients (1.4%). Men had a higher prevalence than women (p < 0.0001). Multiple arachnoid cysts occurred in 30 patients. The most common locations were middle fossa (34%), retrocerebellar (33%), and convexity (14%). Middle fossa cysts were predominantly left-sided (70%, p < 0.001). Thirty-five patients were considered symptomatic and 24 underwent surgical treatment. Sellar and suprasellar cysts were more likely to be considered symptomatic (p < 0.0001). Middle fossa cysts were less likely to be considered symptomatic (p = 0.01. The criteria for natural history analysis were met in 203 patients with a total of 213 cysts. After a mean follow-up of 3.8 ± 2.8 years (for this subgroup), 5 cysts (2.3%) increased in size and 2 cysts decreased in size (0.9%). Only 2 patients developed new or worsening symptoms over the follow-up period. Conclusions Arachnoid cysts are a common incidental finding on intracranial imaging in all age groups. Although arachnoid cysts are symptomatic in a small number of patients, they are associated with a benign natural history for those presenting without symptoms.

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Suprainguinal retroperitoneal approach for the successful surgical treatment of meralgia paresthetica

Journal of Neurosurgery ◽

10.3171/2008.3.17471 ◽

2009 ◽

Vol 110 (4) ◽

pp. 768-774 ◽

Cited By ~ 11

Author(s):

Olaf Alberti ◽

Jürgen Wickboldt ◽

Ralf Becker

Keyword(s):

Surgical Treatment ◽

Complete Remission ◽

Cutaneous Nerve ◽

Lateral Femoral Cutaneous Nerve ◽

Retroperitoneal Approach ◽

Meralgia Paresthetica ◽

Female Ratio ◽

Femoral Cutaneous Nerve ◽

The Mean

Object Neurosurgical textbooks describe an infrainguinal approach as the standard or preferred option for the surgical treatment of meralgia paresthetica (MP), the most frequent entrapment neuropathy of the lower limb. However, inhomogeneous results led the authors to adopt a suprainguinal, retroperitoneal approach for decompression of the lateral femoral cutaneous nerve. In this paper the authors' aim was to study the outcome of patients harboring MP treated via this different surgical approach. Methods The outcome of 55 consecutive patients who underwent surgery for MP via the suprainguinal retroperitoneal approach during a 15-year period was ascertained through postal questionnaires (in 47 patients) and follow-up visits (in 8 patients). The male to female ratio was 1:0.67, and the mean patient age was 50 ± 12.9 years. The mean follow-up was 3.2 ± 3.3 years. Seven of the patients underwent bilateral surgery. Results Intraoperatively the lateral femoral cutaneous nerve was consistently found in close anatomical relationship to the anterior superior iliac spine, although some variations regarding the diameter, number of branches, and underlying pathological entity were observed. Eighty-seven percent of patients showed improvement (21 patients) or complete remission (27 patients) of painful dysesthesia in the anterolateral thigh, and 13% (7 patients) remained unchanged. In addition 82% had improvement (31 patients) or complete remission (14 patients) of hypesthesia, leaving 18% with unchanged (9 patients) or worsened (1 patient) hypesthesia. In the patient-evaluated group 66% (31 of 47) were completely satisfied with the outcome, 23% (11 of 47) were partially satisfied, and 11% (5 of 47) were not satisfied with the outcome. Two cases each of recurrence, seroma, wound infection, and 1 case of hematoma requiring revision were encountered as complications. Conclusions The suprainguinal retroperitoneal approach is a viable first-choice option for the surgical relief of MP.

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Profile of Pediatric Chronic Myeloid Leukemia in the Era of Imatinib - a Study from a Developing Country

Blood ◽

10.1182/blood.v126.23.5599.5599 ◽

2015 ◽

Vol 126 (23) ◽

pp. 5599-5599

Author(s):

Rashmika Rajendran ◽

Catherene Cirumalar Bernard ◽

Febe Renjitha Suman ◽

Julius Xavier Scott ◽

Aruna Rajendran ◽

...

Keyword(s):

Chronic Myeloid Leukemia ◽

Interpersonal Relationships ◽

Myeloid Leukemia ◽

Bone Pain ◽

Treatment Modalities ◽

Molecular Response ◽

Female Ratio ◽

Presenting Symptoms ◽

Long Term Treatment

Abstract INTRODUCTION Chronic myeloid leukemia (CML) is very rare in children constituting 3% of pediatric leukemias and 10% of all CML cases. There is a lack of uniform guidelines, though the leukemic biology and clinicohematological features are the same as adults. Since the advent of imatinib, treatment modalities have evolved. However the effect of long term treatment on the child and family are areas to be studied, especially in developing countries. AIM To study the clinicohematologic profile, diagnostic and therapeutic methods, follow up measures, impact of the disease on the patient's education and the psychosocioeconomic compromise of the parents. MATERIALS AND METHODS Institutional ethics committee approval and informed consent from parents was obtained. All cases of pediatric CML were included. The patients and parents were interviewed and their medical records reviewed. The demography, clinical and hematological features at presentation, treatment, compliance, toxicity and follow up details were collected. The parents were provided with a validated questionnaire to assess the psychosocioeconomic impact. RESULTS In 2011 - 2014, 7 patients below 18 years of age were diagnosed to have CML. Incidence: 5.6 % of pediatric leukemias and 9 % of CML in our centre. 1 patient opted out of treatment; 6 are undergoing treatment and follow up. 3 (50%) were above 14 years of age, 2 (33%) between 10 and 14 years, 1 (17%) below 10 years. Male to female ratio is 2:1. The predominant presenting symptoms were abdominal pain, fever, weight loss and weakness. 4 patients (67%) complained of bone pain, pallor and throat pain. 4 patients (67%) had splenomegaly. All patients presented at the chronic phase of CML. 50% had WBC count less than 20,000/µl and 1 patient (17%) had hyperleukocytosis. FISH and karyotyping detected t(9;22) in all patients. RT-PCR for bcr-abl was done. All patients were started on upfront therapy with standard dose of T.Imatinib. None needed escalated therapy. Toxic symptoms noted were gastrointestinal intolerance, skin rashes, hyperpigmentation, bone pain, myalgia and cramps which were tolerable in 5 (83%) patients. 1 (17%) had to be hospitalized. The dosage was reduced and then gradually increased. 5 patients (83%) were compliant. RESPONSE TO TREATMENT AND FOLLOWUP 3 (50%) patients obtained full treatment response attaining complete molecular response (CMR) at 12-18 months, maintaining CMR at about 4 years of follow up. 2 (33%) patients are also responding well with major molecular response at 1 year (follow up 14 months) and complete cytogenetic response at 6 months (follow up 9 months). 1 patient showed treatment failure due to default follow up and loss of compliance. PSYCHOSOCIOECONOMIC EFFECT: 4 (66%) patients absented from school for more than 6 months and lost an academic year.2 patients who were above 14 years of age absented for a few months which did not affect their studies. 5 (83%) patients belonged to upper middle socioeconomic class; they travelled to the hospital by bus and met medical expenses by insurance and support from funding agencies and friends. 1 (17%) belonged to upper class who met expenses by himself. Of the parents of patients evaluated, 33% responded to feeling guilty. 16% felt guilt related to self, 33% felt guilt with regards to interpersonal relationships. 50% were depressed with 33% admitting to feeling gloomy, 33% feeling easily upset and 33% worried about the future. 50% showed difficulty in coping, 50% felt burdened, 50% felt their activities were affected. However, nobody turned to drugs or alcohol. 33% had problems in their interpersonal relationships; 17% had issues with family, 33% did not want to mingle with others. 17% felt stigmatized; 17% felt neglected, 17% felt their social life was disturbed. 33% felt economical constraints; 33% worried about making ends meet, 17% were required to sell/ mortgage assets. DISCUSSION AND CONCLUSION: In correlation with literature, pediatric CML is rare in our centre. Patients responded well with minimum toxicity to Imatinib when compliance was good. Schooling was affected by the disease. Parents showed signs of depression and difficulty in coping. The government should initiate strategies to provide psychosocioeconomic support to CML patients and family members so they may cope with the disease. Disclosures No relevant conflicts of interest to declare.

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Chorea in the clinical presentation of moyamoya disease: results of surgical revascularization and a proposed clinicopathological correlation

Journal of Neurosurgery Pediatrics ◽

10.3171/2012.11.peds12199 ◽

2013 ◽

Vol 11 (3) ◽

pp. 313-319 ◽

Cited By ~ 15

Author(s):

Edward S. Ahn ◽

R. Michael Scott ◽

Richard L. Robertson ◽

Edward R. Smith

Keyword(s):

Surgical Treatment ◽

Basal Ganglia ◽

Moyamoya Disease ◽

Clinical Presentation ◽

Disease Stage ◽

Consecutive Series ◽

Unilateral Disease ◽

Collateral Vessels ◽

Revascularization Surgery

Object Chorea is a movement disorder characterized by brief, irregular, involuntary contractions that appear to flow from 1 muscle to another. There are a limited number of reports in the literature that have linked moyamoya disease and chorea. The authors describe their experience in treating moyamoya disease in patients in whom chorea developed as part of the clinical presentation. Methods The authors conducted a retrospective review of a consecutive series of 316 children who underwent pial synangiosis revascularization for moyamoya disease at the Boston Children's Hospital. Results Of 316 surgically treated patients with moyamoya disease, 10 (3.2%; 6 boys and 4 girls) had chorea as a part of their presentation. The average age at surgical treatment was 9.9 years (range 3.8–17.9 years). All patients had evidence of hypertrophied lenticulostriate collateral vessels through the basal ganglia on preoperative angiography and/or MRI on affected sides. Two patients had cystic lesions in the basal ganglia. Nine patients underwent bilateral craniotomies for pial synangiosis, and 1 patient underwent a single craniotomy for unilateral disease. Follow-up was available in 9 patients (average 50.1 months). The mean duration of chorea was 1.36 years (range 2 days to 4 years), with resolution of symptoms in all patients. One patient developed chorea 3 years after surgical treatment, 4 patients had transient chorea that resolved prior to surgery, and 5 patients experienced resolution of the chorea after surgery (average 13 months). Conclusions The authors describe children with moyamoya disease and chorea as part of their clinical presentation. The data suggest that involvement of the basal ganglia by the hypertrophied collateral vessels contributes to the development of chorea, which can wax or wane depending on disease stage or involution of the vessels after revascularization surgery. In most patients, however, the chorea improves or disappears about 1 year after presentation.

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