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1.
Neurocrit Care ; 35(1): 87-102, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33205356

RESUMO

BACKGROUND: Elevated intracranial pressure due to cerebral edema is associated with very poor survival in patients with acute liver failure (ALF). Placing an intracranial pressure monitor (ICPm) aids in management of intracranial hypertension, but is associated with potentially fatal hemorrhagic complications related to the severe coagulopathy associated with ALF. METHODS: An institutional Acute Liver Failure Clinical Protocol (ALF-CP) was created to correct ALF coagulopathy prior to placing parenchymal ICP monitoring bolts. We aimed to investigate the frequency, severity, and clinical significance of hemorrhagic complications associated with ICPm bolt placement in the setting of an ALF-CP. All assessed patients were managed with the ALF-CP and had rigorous radiologic follow-up allowing assessment of the occurrence and chronology of hemorrhagic complications. We also aimed to compare our outcomes to other studies that were identified through a comprehensive review of the literature. RESULTS: Fourteen ALF patients were included in our analysis. There was no symptomatic hemorrhage after ICP monitor placement though four patients were found to have minor intraparenchymal asymptomatic hemorrhages after liver transplant when the ICP monitor had been removed, making the rate of radiographically identified clinically asymptomatic hemorrhage 28.6%. These results compare favorably to those found in a comprehensive review of the literature which revealed rates as high as 17.5% for symptomatic hemorrhages and 30.4% for asymptomatic hemorrhage. CONCLUSION: This study suggests that an intraparenchymal ICPm can be placed safely in tertiary referral centers which utilize a protocol such as the ALF-CP that aggressively corrects coagulopathy. The ALF-CP led to advantageous outcomes for ICPm placement with a 0% rate of symptomatic and low rate of asymptomatic hemorrhagic complications, which compares well to results reported in other series. A strict ICPm placement protocol in this setting facilitates management of ALF patients with cerebral edema during the wait time to transplantation or spontaneous recovery.


Assuntos
Edema Encefálico , Hipertensão Intracraniana , Falência Hepática Aguda , Edema Encefálico/etiologia , Humanos , Hipertensão Intracraniana/etiologia , Hipertensão Intracraniana/terapia , Pressão Intracraniana , Falência Hepática Aguda/terapia , Monitorização Fisiológica , Literatura de Revisão como Assunto
2.
Mol Diagn Ther ; 24(3): 327-338, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32274701

RESUMO

BACKGROUND: The presence of mutations in the isocitrate dehydrogenase 1 and 2 genes (IDH1/2) in glioma tumors is correlated with good prognosis upon standard-of-care treatment. Therefore, information on whether the glioma tumor has IDH1/2 mutations could be used in the correct diagnosis and management of glial tumors. The two most common techniques used to detect IDH1/2 mutations, immunohistochemistry (IHC) and Sanger sequencing, are prone to missing these mutations, especially if the tumor cells that carry the mutations constitute a small minority of the tumor itself. OBJECTIVES: We developed and validated a rapid method (3-mismatch-amplification refractory mutation system [3m-ARMS]) that can be used for pre-, intra- and postoperative detection of the most common IDH1/2 mutations in glial tumors with high specificity and sensitivity. We also conducted a comprehensive IDH1/2 mutation analysis in 236 glial tumor samples comparing 3m-ARMS, IHC and Sanger sequencing. METHODS: 3m-ARMS was optimized and validated for the specific and sensitive detection of the most common IDH1 and IDH2 mutations. We then analyzed 236 glial tumor samples for the presence of IDH1/2 mutations using 3m-ARMS, Sanger sequencing and IHC techniques. We then analyzed and compared the results, evaluating the diagnostic and screening potential of 3m-ARMS. RESULTS: Comparison of the three techniques used in the mutation analysis showed that 3m-ARMS-based IDH1/2 mutation detection was superior to IHC and Sanger sequencing-based IDH1/2 mutation detection in terms of accuracy, specificity and sensitivity, especially for tumor samples in which only a small minority of the cell population carried the mutation. 3m-ARMS could detect the presence of femtogram levels of IDH1/2 mutant DNA in DNA samples in which the mutant DNA-to-wild-type DNA ratio was as low as 1:100,000. CONCLUSION: Sanger sequencing and IHC-based methods have shortcomings when detecting mutations in glial tumors so can miss IDH1/2 mutations in glial tumors when used alone without proper modifications. 3m-ARMS-based mutation detection is fast and simple with potential for use as a diagnostic test for the majority of hot spot mutations in IDH1/2 genes. It can detect IDH1/2 mutations within an hour so can be adapted for intraoperative diagnosis.


Assuntos
Análise Mutacional de DNA , Glioma/diagnóstico , Glioma/genética , Isocitrato Desidrogenase/genética , Mutação , Alelos , Biomarcadores Tumorais , Análise Mutacional de DNA/métodos , Humanos , Imuno-Histoquímica , Isocitrato Desidrogenase/metabolismo , Reação em Cadeia da Polimerase/métodos , Sensibilidade e Especificidade
3.
Neurosurg Rev ; 43(3): 835-846, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30334173

RESUMO

Effective communication is critical in healthcare facilitation. Our aim is to illustrate the impact of digital communication tools in the field of neurosurgery based on the cumulative recently published reports to show an evidence-based review of both benefits and limitations. We performed a systematic review of records published from January 2003 to March 2018. A specific set of keywords such as "digital" and "communication" were used on PubMed database to conduct a thorough online search. 13 articles, out of 52, were comprehensively studied after complying with our inclusion and exclusion criteria. Many of the reviewed studies reported several applications of digital health communication tools in neurosurgery including 46% (6/13) in the Emergency Room and 23% (3/13) in the Operating Room. 38.5% (5/13) were applied in teaching hospitals. Reviewed studies were divided into two groups according to their applications (interventional (3/13) and non-interventional (10/13)). In the Emergency Room, digital health tools facilitated timely diagnosis and management, while in the operating room it permitted revolutionary robotic surgery. It showed potential for "no-risk learning" at academic institutions. While the fruitful impacts were convincing of the digital communication tools' ability to enhance healthcare in neurosurgery, proper adherence to regulations against data loss and theft, two potential complications of digital tool misuse, must be maintained. Additionally, both time efficiency and the necessity of waiting for better implementation of communication tools proved to be obstacles to consistent digital tool integration.


Assuntos
Comunicação em Saúde , Neurocirurgia/tendências , Telecomunicações , Humanos
4.
J Neurosurg Sci ; 64(5): 446-451, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29115099

RESUMO

BACKGROUND: Spinal meningiomas are benign, well-circumscribed and slowly-growing intradural tumors that compress the spinal cord. Hereby, a retrospective review of 61 spinal meningioma cases evaluated in terms of demographic, clinical, pathological and radiological features to predict the early postoperative functional outcomes. METHODS: Patients' records and MRI images of all the histologically confirmed spinal meningioma cases that underwent surgical resection at two university hospitals from January 2005 to June 2016 were retrospectively reviewed. Demographic data, clinical findings, radiological features and pathology reports were reviewed. Univariate and multivariate logistic regression analyses were used to evaluate the impact of each factor on the early 3-month post-surgical functional outcome. A receiver operating characteristic (ROC) curve was used to predict the power of the model. RESULTS: Sixty-one cases of spinal meningiomas were operated: 13 males (21%) and 48 females (79%). Mean age was 60.5 years and ranged from 24 to 92 years. Patients presented with back pain (57%), motor deficits (47.5%), sensory deficits (18%) and sphincteric dysfunction (11.5%). One case (1.6%) showed an additional extradural growth. There were 40 thoracic, nine cervical, five cervicothoracic, five thoracolumbar, and two lumbar cases. Only four cases (6.6%) showed atypical pathological features (WHO grade II). At 3-month follow-up, 46 patients (75.4%) had either functionally improved or remained stable. Fifteen patients (24.6%) had "worse" functional outcome. Three variables showed statistically significant odds ratio for improved outcomes (OR): pre-surgical motor deficit (OR=5; P=0.005); presurgical sensory disturbance (OR=3.5; P=0.026); pre-surgical myelopathy (OR=3.5; P=0.026). Multivariate analysis showed increased OR for cross-sectional ratio, pre-surgical myelopathy, pre-surgical radiculopathy and non-cervical location of tumor (1.59, 3.46, 3.2, 1.63/3.56, respectively). Although none has reached statistical significance (P>0.05), the receiver operating characteristic (ROC) curve showed an area under the curve (AUC) of 0.74. CONCLUSIONS: The independent predictors of the early postoperative functional outcomes of spinal meningioma resections may include pre-surgical motor deficit, sensory deficit and myelopathy.


Assuntos
Neoplasias Meníngeas , Meningioma , Neoplasias da Medula Espinal , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Feminino , Humanos , Masculino , Neoplasias Meníngeas/cirurgia , Meningioma/cirurgia , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos , Estudos Retrospectivos , Neoplasias da Medula Espinal/cirurgia , Resultado do Tratamento , Adulto Jovem
5.
Asian Spine J ; 13(6): 1036-1046, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31422644

RESUMO

Antidepressant drugs can be advantageous in treating psychiatric and non-psychiatric illnesses, including spinal disorders. However, spine surgeons remain unfamiliar with the advantages and disadvantages of the use of antidepressant drugs as a part of the medical management of diseases of the spine. Our review article describes a systematic method using the PubMed/Medline database with a specific set of keywords to identify such benefits and drawbacks based on 17 original relevant articles published between January 2000 and February 2018; this provides the community of spine surgeons with available cumulative evidence contained within two tables illustrating both observational (10 studies; three cross-sectional, three case-control, and four cohort studies) and interventional (seven randomized clinical trials) studies. While tricyclic antidepressants (e.g., amitriptyline) and duloxetine can be effective in the treatment of neuropathic pain caused by root compression, venlafaxine may be more appropriate for patients with spinal cord injury presenting with depression and/or nociceptive pain. Despite the potential associated consequences of a prolonged hospital stay, higher cost, and controversial reports regarding the lowering of bone mineral density in the elderly, antidepressants may improve patient satisfaction and quality of life following surgery, and reduce postoperative pain and risk of delirium. The preoperative treatment of preexisting psychiatric diseases, such as anxiety and depression, can improve outcomes for patients with spinal cord injury-related disabilities; however, a preoperative platelet function assay is advocated prior to major spine surgical procedures to protect against significant intraoperative blood loss, as serotonergic antidepressants (e.g., selective serotonin reuptake inhibitors) and bupropion can increase the likelihood of bleeding intraoperatively due to drug-induced platelet dysfunction. This comprehensive review of this evolving topic can assist spine surgeons in better understanding the benefits and risks of antidepressant drugs to optimize outcomes and avoid potential hazards in a spine surgical setting.

6.
Turk Neurosurg ; 29(2): 289-296, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30843598

RESUMO

AIM: To better understand the indications and the impact of antidepressant drugs on the field of neurosurgery in terms of risks and benefits in Turkey. MATERIAL AND METHODS: A national survey was conducted among neurosurgeons in current practice in Turkey to report routine prescription trends of antidepressant drugs. Gender, neurosurgical subspecialty, city, type of practice, years of experience, antidepressant agent, indications and reasons not to prescribe were ascertained. A brief review of literature was done to show clinical and research evidence. RESULTS: A total of 336 neurosurgeons took the survey; 53% of them prescribe antidepressants routinely, whereas 47% of them do not. Of the 72% non-prescribers prefer to refer their patients to a psychiatrist and 22% of them believe that antidepressants have no role in neurosurgery. Vascular and pediatric neurosurgeons as well as neurosurgeons at research hospitals showed the lowest prescription proportion, while general and brain tumor neurosurgeons and ones in private practice showed the highest prescription proportion. Surgeons with more than 20 year-experience had the lowest prescription proportion (43%). Type of practice and years of experience showed a statistically significant association with the prescription of antidepressant drugs across (p=0.002 and p=0.025, respectively). CONCLUSION: Type of practice and years of experience showed a statistically significant association with the proportion of antidepressants' prescribers in Turkish neurosurgery practice at the time of this survey. Non-prescriber neurosurgeons need to recognize the indications, whereas prescribers have to understand the risks of using antidepressants.


Assuntos
Antidepressivos/uso terapêutico , Neurocirurgiões/estatística & dados numéricos , Padrões de Prática Médica/estatística & dados numéricos , Feminino , Humanos , Masculino , Neurocirurgia , Inquéritos e Questionários , Turquia
7.
Oper Neurosurg (Hagerstown) ; 16(5): 607-613, 2019 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-30169686

RESUMO

BACKGROUND: The posterior cervical keyhole (KH) laminoforaminotomy has been described to involve the lateral portion of cervical laminae of the upper vertebra alone (small KH) or of both upper and lower vertebrae (large KH). OBJECTIVE: To microscopically compare the two keyhole techniques in terms of their ability to expose the corresponding cervical roots. METHODS: Ten cadaveric specimens were operated bilaterally from C3-4 to C6-7 level to expose a total of 80 nerve roots. The large KH was applied to the left side, the small KH to the right side. The maximal length of exposed nerve roots was measured under microscope. The virtual optimal KH surface area was determined using digital software. Each root was inspected for exposure of its root and axilla. RESULTS: The maximal exposed nerve root length on the large KH side was significantly larger than on the small KH side at C3-4, C5-6, and C6-7 levels (P = .031, P = .002, P = .003). No significance was reported for C4-5 (P = .06). We could expose right axillae in (3/40) and left axillae in (33/40; P < .001). Optimal keyhole surface areas were 37.9, 38.2, 38.7, and 46.2 mm2 in craniocaudal order. CONCLUSION: Large KH defects involving both upper and lower laminae and facets can expose the roots to greater extent than small KH defects at C3-4, C5-6, and C6-7 levels. Large KH defects may allow better exposure of nerve roots axillae than small KH defects.


Assuntos
Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Foraminotomia/métodos , Laminectomia/métodos , Raízes Nervosas Espinhais/diagnóstico por imagem , Raízes Nervosas Espinhais/cirurgia , Cadáver , Vértebras Cervicais/patologia , Humanos , Raízes Nervosas Espinhais/patologia
8.
World Neurosurg ; 111: 216-226, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29253696

RESUMO

BACKGROUND: The cervicothoracic junction is a challenging anatomic transition in spine surgery. It is commonly affected by different types of diseases that may significantly impair stability in this region. The seventh cervical vertebra (C7) is an atypical cervical vertebra with unique anatomic features compared to subaxial cervical spine (C3 to C6). C7 has relatively broader laminae, larger pedicles, smaller lateral masses, and a long nonbifid spinous process. These features allow a variety of surgical methods for performing posterior rigid instrumentation in the form of different types of screws, such as lateral mass screws, pedicle screws, transfacet screws, and intralaminar screws. Many biomechanical studies on cadavers have evaluated and compared different types of implants at C7. METHODS: We reviewed PubMed/Medline by using specific combinations of keywords to summarize previously published articles that examined C7 posterior rigid instrumentation thoroughly in an experimental fashion on patients or cadavers with additional descriptive radiologic parameters for evaluation of the optimum surgical technique for each type. RESULTS: A total of 44 articles were reported, including 22 articles that discussed anatomic considerations (entry points, sagittal and axial trajectories, and features of screws) and another 22 articles that discussed the relevant biomechanical testing at this transitional region if C7 was directly involved in terms of receiving posterior rigid implants. CONCLUSIONS: C7 can accommodate different types of screws, which can provide additional benefits and risks based on availability of bony purchase, awareness of surgical technique, biomechanics, and anatomic considerations.


Assuntos
Vértebras Cervicais/cirurgia , Fusão Vertebral/métodos , Vértebras Torácicas/cirurgia , Fenômenos Biomecânicos , Humanos
9.
Clin Neurol Neurosurg ; 162: 118-126, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-29031234

RESUMO

OBJECTIVES: 1) To provide neurosurgeons and radiologists with a new quantitative and anatomical method to describe spinal meningiomas (SM) consistently. 2) To provide a guide to the surgical approach needed and amount of bony resection required based on the proposed classification. 3) To report the distribution of our 58 cases of SM over different Stages and Subtypes in correlation to the surgical treatment needed for each case. 4) To briefly review the literature on the rare non-conventional surgical corridors to resect SM. PATIENTS AND METHODS: We reviewed the literature to report on previously published cohorts and classifications used to describe the location of the tumor inside the spinal canal. We reviewed the cases that were published prior showing non-conventional surgical approaches to resect spinal meningiomas. We proposed our classification system composed of Staging based on maximal cross-sectional surface area of tumor inside canal, Typing based on number of quadrants occupied by tumor and Subtyping based on location of the tumor bulk to spinal cord. Extradural and extra-spinal growth were also covered by our classification. We then applied it retrospectively on our 58 cases. RESULTS: 12 articles were published illustrating overlapping terms to describe spinal meningiomas. Another 7 articles were published reporting on 23 cases of anteriorly located spinal meningiomas treated with approaches other than laminectomies/laminoplasties. 4 Types, 9 Subtypes and 4 Stages were described in our Classification System. In our series of 58 patients, no midline anterior type was represented. Therefore, all our cases were treated by laminectomies or laminoplasties (with/without facetectomies) except a case with a paraspinal component where a costotransversectomy was needed. CONCLUSION: Spinal meningiomas can be radiologically described in a precise fashion. Selection of surgical corridor depends mainly on location of tumor bulk inside canal.


Assuntos
Neoplasias Meníngeas/classificação , Neoplasias Meníngeas/patologia , Meningioma/classificação , Meningioma/patologia , Medula Espinal/patologia , Humanos , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/cirurgia , Meningioma/diagnóstico por imagem , Meningioma/cirurgia , Medula Espinal/diagnóstico por imagem , Medula Espinal/cirurgia
10.
World Neurosurg ; 105: 1032.e7-1032.e11, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28559065

RESUMO

BACKGROUND: Pituitary sarcomas are rare clinical entities most often encountered as secondary neoplasms representing late sequelae of radiation therapy to the sellar region. Primary pituitary fibrosarcomas (PPFS), in contrast, are exceptionally rare tumors with very few cases described in the literature thus far. Herein, we present a case of PFFS and describe it in the context of the existing literature. CASE DESCRIPTION: A 39-year-old woman presented with 2 months of headaches and rapidly progressive vision loss. She was found to have a 2.7-cm pituitary mass and initially underwent transnasal transsphenoidal resection from which pathology confirmed a pituitary adenoma. Eight months after surgery, she represented with vision changes and a recurrent tumor that postoperatively was found to be a primary fibrosarcoma with embedded adenoma remnants. On post-hoc examination of her original pathologic specimen, a prominent fibrous tissue component was suspected to be the source of her fibrosarcoma. CONCLUSIONS: This is a rare case of PFFS that highlights the need for close surveillance in affected patients. Through this case, we review the existing literature and discuss both surgical management and aggressive adjuvant therapy of this rare disease entity.


Assuntos
Adenoma/patologia , Descompressão Cirúrgica/métodos , Fibrossarcoma/secundário , Fibrossarcoma/cirurgia , Neoplasias Hipofisárias/cirurgia , Adenoma/diagnóstico por imagem , Adenoma/cirurgia , Adulto , Diagnóstico Diferencial , Feminino , Fibrossarcoma/diagnóstico por imagem , Gadolínio/farmacocinética , Humanos , Imageamento por Ressonância Magnética , Hipófise/diagnóstico por imagem , Hipófise/metabolismo , Hipófise/patologia , Neoplasias Hipofisárias/diagnóstico por imagem
11.
Eur Spine J ; 25(12): 4171-4180, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27388018

RESUMO

PURPOSE: Vertebral artery loop is a congenital or acquired anomaly. Vertebral artery loops are incidentally diagnosed during evaluation of neck problems and trauma. We aimed to present the incidence of VA loops using magnetic resonance angiography in consecutive patients and discuss epidemiological data including the gender, age, location, signs and symptoms, treatment approaches and outcomes of VA loops via analyzing literature. METHODS: In the first leg of our two-legged study, consecutive patients were evaluated using magnetic resonance angiography to detect any medial loop of vertebral arteries. The study period was from October 2015 to March 2016. In the second leg, academic databases about medial loop of vertebral artery were screened. Case reports, case series, abstracts and references of relevant literature were searched manually to avoid any missing cases. RESULTS: We evaluated 239 consecutive patients using magnetic resonance angiography. Twenty-one patients were excluded from the study due to inadequate image acquisition, aplastic vertebral artery and/or concomitant vertebral artery stenosis. Medial loop of V2 vertebral artery was observed in 13 patients (5.9 %): 9 with left, 2 with right and 2 with bilateral medial V2 loop. Patients with medial V2 loop were significantly older than patients with straight vertebral arteries (70.30 vs. 62.36, p = 0.028). In the literature analysis, VA loops were more commonly observed at V2 segment (90.5 %). Vertebral artery loops were mostly diagnosed at the 5th and 6th decades of life predominantly in females. The most common signs and symptoms were radiculopathy and/or neck pain, and signs and symptoms of vertebrobasilar insufficiency. CONCLUSIONS: Concise pre-operative evaluation of the vertebral arteries is essential to avoid the injury of undiagnosed VA loops during surgery, which might result in catastrophic circumstances. Further evaluation of the vertebral arteries using MR angiography is required, especially in elder age, before cervical spine surgeries.


Assuntos
Vértebras Cervicais/cirurgia , Lesões do Sistema Vascular/prevenção & controle , Artéria Vertebral/anormalidades , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Incidência , Angiografia por Ressonância Magnética , Masculino , Erros Médicos/prevenção & controle , Pessoa de Meia-Idade , Cervicalgia/etiologia , Radiculopatia/etiologia , Estudos Retrospectivos , Fatores Sexuais , Malformações Vasculares/complicações , Malformações Vasculares/diagnóstico por imagem , Malformações Vasculares/epidemiologia , Artéria Vertebral/diagnóstico por imagem , Insuficiência Vertebrobasilar/etiologia , Adulto Jovem
12.
J Craniofac Surg ; 27(5): e459-61, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27315314

RESUMO

A combination of trauma and a missed inflammatory response (nasal operation) concomitant with hydrocephalus and tumor in secondary encephalocele has not been described in the English literature yet. A 38-year-old man was admitted to the clinic with rhinorrhea that started 3 months ago. In his medical history, nothing abnormal was present except a nasal operation performed 1 year ago. Brain magnetic resonance imaging depicted left frontal encephalocele concomitant with obstructive hydrocephalus caused by an epidermoid cyst originated from the pineal region. A 2-staged surgery was planned. In the first stage, a ventriculoperitoneal shunt insertion was conveyed successfully. In the second-stage surgery, the herniated brain tissue was excised, and the frontal sinus was cleansed with serum saline combined with antibiotic. The bony defect and the dura defect were repaired. The patient's presenting complaint recovered fully, and he was discharged to home in a well condition. Acquired encephalocele is a rare entity. In case of rhinorrhea and encephalocele, even in the presence of prior history of nasal surgery, intracranial evaluation should be conveyed to exclude the presence of hydrocephalus and/or tumor. The cranial defect should be repaired to prevent future infections and brain tissue damage.


Assuntos
Cistos do Sistema Nervoso Central/complicações , Encefalocele/diagnóstico , Cisto Epidérmico/complicações , Glândula Pineal , Adulto , Cistos do Sistema Nervoso Central/diagnóstico , Encefalocele/complicações , Cisto Epidérmico/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Masculino , Tomografia Computadorizada por Raios X
13.
BMC Surg ; 15: 24, 2015 Mar 11.
Artigo em Inglês | MEDLINE | ID: mdl-25880917

RESUMO

BACKGROUND: Gorham's disease is a rare osteolytic disorder characterized by progressive resorption of bone and replacement of osseous matrix by a proliferative non-neoplastic vascular or lymphatic tissue. A standardized treatment protocol has not yet been defined due to the unpredictable natural history of the disease and variable clinical presentations. No single treatment has proven to be superior in arresting the course of the disease. Trials have included surgery, radiation and medical therapies using drugs such as calcium salts, vitamin D supplements and hormones. We report on our advantageous experience in the management of this osteolyic disorder in a case when it affected only the skull vault. A brief review of pertinent literature about Gorham's disease with skull involvement is provided. CASE PRESENTATION: A 25-year-old Caucasian male presented with a skull depression over the left fronto-temporal region. He noticed progressive enlargement of the skull defect associated with local pain and mild headache. Physical examination revealed a tender palpable depression of the fronto-temporal convexity. Conventional X-ray of the skull showed widespread loss of bone substance. Subsequent CT scans showed features of patchy erosions indicative of an underlying osteolysis. MRI also revealed marginal enhancement at the site of the defect. The patient was in need of a pathological diagnosis as well as complex reconstruction of the afflicted area. A density graded CT scan was done to determine the variable degrees of osteolysis and a custom made allograft was designed for cranioplasty preoperatively to allow for a single step excisional craniectomy with synchronous skull repair. Gorham's disease was diagnosed based on histopathological examination. No neurological deficit or wound complications were reported postoperatively. Over a two-year follow up period, the patient had no evidence of local recurrence or other systemic involvement. CONCLUSIONS: A single step excisional craniectomy and cranioplasty can be an effective treatment for patients with Gorham's disease affecting the skull vault only. Preoperative planning by a density graded CT aids to design a synthetic bone flap and is beneficial in skull reconstruction. Systemic involvement is variable in this patient's population.


Assuntos
Osteólise Essencial/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Crânio/cirurgia , Adulto , Humanos , Imageamento por Ressonância Magnética , Masculino , Osteólise Essencial/diagnóstico , Crânio/diagnóstico por imagem , Tomografia Computadorizada por Raios X
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