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Acute fatal hemorrhage from previously undiagnosed cerebral arteriovenous malformations in children: a single-center experience

Coleman P. Riordan, Darren B. Orbach, Edward R. Smith, and R. Michael Scott

OBJECTIVE

The most significant adverse outcome of intracranial hemorrhage from an arteriovenous malformation (AVM) is death. This study reviews a single-center experience with pediatric AVMs to quantify the incidence and characterize clinical and radiographic factors associated with sudden death from the hemorrhage of previously undiagnosed AVMs in children.

METHODS

A single-center database review of the period from 2006 to 2017 identified all patients with a first-time intracranial hemorrhage from a previously undiagnosed AVM. Clinical and radiographic data were collected and compared between patients who survived to hospital discharge and those who died at presentation.

RESULTS

A total of 57 patients (average age 10.8 years, range 0.1–19 years) presented with first-time intracranial hemorrhage from a previously undiagnosed AVM during the study period. Of this group, 7/57 (12%) patients (average age 11.5 years, range 6–16 years) suffered hemorrhages that led directly to their deaths. Compared to the cohort of patients who survived their hemorrhage, patients who died were 4 times more likely to have an AVM in the posterior fossa. No clear pattern of antecedent triggering activity (sports, trauma, etc.) was identified, and 3/7 (43%) experienced cardiac arrest in the prehospital setting. Surviving patients were ultimately treated with resection of the AVM in 42/50 (84%) of cases.

CONCLUSIONS

Children who present with hemorrhage from a previously undiagnosed intracranial AVM had a 12% chance of sudden death in our single-institution series of pediatric cerebrovascular cases. Clinical triggers of hemorrhage are unpredictable, but subsequent radiographic evidence of a posterior fossa AVM was present in 57% of fatal cases, and all fatal cases were in locations with high risk of potential herniation. These data support a proactive, aggressive approach toward definitive treatment of AVMs in children.

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The neurosurgeon as baseball fan and inventor: Walter Dandy and the batter’s helmet

Ryan Brewster, Wenya Linda Bi, Timothy R. Smith, William B. Gormley, Ian F. Dunn, and Edward R. Laws Jr.

Baseball maintains one of the highest impact injury rates in all athletics. A principal causative factor is the “beanball,” referring to a pitch thrown directly at a batter’s head. Frequent morbidities elicited demand for the development of protective gear development in the 20th century. In this setting, Dr. Walter Dandy was commissioned to design a “protective cap” in 1941. His invention became widely adopted by professional baseball and inspired subsequent generations of batting helmets. As a baseball aficionado since his youth, Walter Dandy identified a natural partnership between baseball and medical practice for the reduction of beaning-related brain injuries. This history further supports the unique position of neurosurgeons to leverage clinical insights, inform innovation, and expand service to society.

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Massive growth of a meningioma into the brachial plexus and thoracic cavity after intraspinal and supraclavicular resection

Case report and review of the literature

Edward R. Smith, Mark Ott, John Wain, David N. Louis, and E. Antonio Chiocca

✓ Extracranial meningiomas comprise approximately 2% of all meningiomas. Involvement of peripheral nerves by meningioma, either by a primary tumor or through secondary extension of an intraaxial lesion, is a much rarer entity; there have been only two reported primary brachial plexus meningiomas and one description of secondary involvement of the brachial plexus by extension of an intraaxial lesion. Although thoracic cavity meningiomas have been described in the literature, their pathogenesis is poorly understood. The authors present the case report of a 36-year-old man who was initially treated for a thoracic spinal meningioma that infiltrated the brachial plexus. After resection, progressive and massive growth with infiltration of the brachial plexus and pleural cavity occurred over a 5-year period despite radio- and chemotherapy. The case report is followed by a review of the literature of this rare entity.

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Complications after transsphenoidal surgery for patients with Cushing's disease and silent corticotroph adenomas

Timothy R. Smith, M. Maher Hulou, Kevin T. Huang, Breno Nery, Samuel Miranda de Moura, David J. Cote, and Edward R. Laws

OBJECT

The purpose of this study was to describe complications associated with the endonasal, transsphenoidal approach for the treatment of adrenocorticotropic hormone (ACTH)–positive staining tumors (Cushing's disease [CD] and silent corticotroph adenomas [SCAs]) performed by 1 surgeon at a high-volume academic medical center.

METHODS

Medical records from Brigham and Women's Hospital were retrospectively reviewed. Selected for study were 82 patients with CD who during April 2008–April 2014 had consecutively undergone transsphenoidal resection or who had subsequent pathological confirmation of ACTH-positive tumor staining. In addition to demographic, patient, tumor, and surgery characteristics, complications were evaluated. Complications of interest included syndrome of inappropriate antidiuretic hormone secretion, diabetes insipidus (DI), CSF leakage, carotid artery injury, epistaxis, meningitis, and vision changes.

RESULTS

Of the 82 patients, 68 (82.9%) had CD and 14 (17.1%) had SCAs; 55 patients were female and 27 were male. Most common (n = 62 patients, 82.7%) were microadenomas, followed by macroadenomas (n = 13, 14.7%). A total of 31 (37.8%) patients underwent reoperation. Median follow-up time was 12.0 months (range 3–69 months). The most common diagnosis was ACTH-secreting (n = 68, 82.9%), followed by silent tumors/adenomas (n = 14, 17.1%). ACTH hyperplasia was found in 8 patients (9.8%). Of the 74 patients who had verified tumors, 12 (16.2%) had tumors with atypical features.

The overall (CD and SCA) rate of minor complications was 35.4%; the rate of major complications was 8.5% (n = 7). All permanent morbidity was associated with DI (n = 5, 6.1%). In 16 CD patients (23.5%), transient DI developed. Transient DI was more likely to develop in CD patients who had undergone a second operation (37.9%) than in those who had undergone a first operation only (12.8%, p < 0.05). Permanent DI developed in 4 CD patients (5.9%) and 1 SCA patient (7.1%). For 1 CD patient, intraoperative carotid artery injury required endovascular sacrifice of the injured artery, but the patient remained neurologically intact. For another CD patient, aseptic meningitis developed and was treated effectively with corticosteroids. One CD patient experienced major postoperative epistaxis requiring another operative procedure to achieve hemostasis. For 2 CD patients, development of sinus mucoceles was managed conservatively. For 1 SCA patient, an abdominal wound dehisced at the fat graft site. No patients experienced postoperative CSF leakage, visual impairment, or deep vein thrombosis.

CONCLUSIONS

Transsphenoidal surgery is the treatment of choice for patients with CD and other ACTH-positive staining tumors. Recent advances in endoscopic technology and increasing surgeon comfort with this technology are making transsphenoidal procedures safer, faster, and more effective. Serious complications are uncommon and can be managed successfully.

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Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome

Edward R. Smith, Craig D. McClain, Matthew Heeney, and R. Michael Scott

Object

Many children with sickle cell anemia (SCA) also have clinical and radiographic findings of an arteriopathy suggestive of moyamoya syndrome. These patients may continue to experience strokes despite optimal medical management. The authors wished to define features of moyamoya syndrome associated with SCA and determine the results of surgical revascularization in these patients at early and late follow-up.

Methods

The authors reviewed the clinical and radiographic records of all patients with moyamoya syndrome and SCA who underwent cerebral revascularization surgery using a standardized surgical procedure—pial synangiosis—from 1985 to 2008.

Results

Twelve patients had SCA and moyamoya syndrome. Six patients were female and 6 were male. The average patient age at surgery was 11.3 years (range 3–22 years). All patients presented with ischemic symptoms, 11 (92%) with previous transient ischemic attacks, and 10 (83%) with completed strokes. Eleven patients (92%) had radiographic evidence of previous stroke at presentation. None presented with hemorrhage. Surgical treatment included pial synangiosis in all patients. Complications included 1 perioperative stroke, 1 wound infection, and 1 perioperative pneumonia. The average length of hospital stay was 5.7 days (including a 24-hour preoperative admission for hydration) and average blood loss was 92.5 ml/hemisphere (in a total of 19 hemispheres). Clinical and radiographic follow-up with an average of 49 months (range 9–144 months) demonstrated no worsening in neurological status in any patient. No clinical or radiographic evidence of new infarcts was observed in any patient at late follow-up, despite disease progression in 13 (68%) of 19 hemispheres.

Conclusions

The clinical and radiographic features of moyamoya syndrome associated with SCA appear comparable to primary moyamoya disease. Successful treatment of these patients requires multidisciplinary care involving hematologists, anesthesiologists, and neurosurgeons. Operative treatment of moyamoya syndrome using pial synangiosis appears to be safe and confers long-lasting protection against further stroke in this population, and provides an alternative for failure of optimal medical therapy in patients. This study underscores the potential merit of screening patients with SCA for moyamoya syndrome.

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Incidence, clinical features, and treatment of familial moyamoya in pediatric patients: a single-institution series

Jonathan Gaillard, Jennifer Klein, Daniel Duran, Armide Storey, R. Michael Scott, Kristopher Kahle, and Edward R. Smith

OBJECTIVE

Limited data exist on familial moyamoya in children. The purpose of this study was to characterize presentation and outcomes of pediatric moyamoya patients who have relatives diagnosed with moyamoya.

METHODS

The authors performed a single-institution retrospective analysis of a case series including all surgically treated children with moyamoya with first- or second-degree relatives with moyamoya. Clinical and radiographic characteristics were analyzed, along with surgical outcomes.

RESULTS

A total of 537 patients underwent surgery for moyamoya during the study period. Eighteen of those patients (3.4% of the total series) had moyamoya and a family history of moyamoya and were included in this study. Of these 18 patients, 14 were non-twin siblings, and the remaining 4 represented 2 pairs of identical (affected) twins.

The presentation was predominantly ischemic (72%), but 4 patients (33%) were asymptomatic when they were found to have moyamoya. Bilateral disease was present in 13 patients (72%). Radiographic stroke prevalence (67%), Suzuki grade (3.3), and angiographic findings were comparable to findings in nonfamilial moyamoya patients. Thirty revascularization procedures were performed, with a 3.3% operative stroke rate per hemisphere and no new strokes in an average follow-up period of 4.5 years.

CONCLUSIONS

In a North American surgical series, familial moyamoya existed in 3.4% of cases, and was distinguished by manifesting in a broad range of ethnic groups, with a higher proportion of male patients and increased rates of asymptomatic and unilateral disease in comparison to nonfamilial moyamoya. Screening indications remain controversial and the current data are used to suggest guidelines. Surgical therapy is warranted, effective, and durable in these patients, but patients should be carefully selected.

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Treatment of moyamoya disease in the adult population with pial synangiosis

Clinical article

Ning Lin, Joshua P. Aronson, Sunil Manjila, Edward R. Smith, and R. Michael Scott

Object

Surgical treatment of moyamoya disease in the adult population commonly uses direct revascularization, the superficial temporal artery (STA) to middle cerebral artery (MCA) bypass (STA-MCA). Pial synangiosis, a method of indirect revascularization, has been used in adult patients with moyamoya when STA-MCA bypass was not technically feasible. Although the effectiveness of pial synangiosis has been well described in children, only limited reports have examined its role in adult patients with moyamoya disease. In this study the authors report on their experience with pial synangiosis revascularization for this population.

Methods

The authors reviewed the clinical and radiographic records of all adult patients (≥ 18 years of age) with moyamoya disease who underwent cerebral revascularization surgery using pial synangiosis at a single institution.

Results

From 1985 to 2010, 66 procedures (6 unilateral, 30 bilateral) were performed on 36 adult patients with moyamoya disease. The mean age at surgery was 28.3 years, and 30 patients were female. Twenty-eight patients (77.8%) presented with transient ischemic attacks (TIAs), 24 (66.7%) with stroke, and 3 (8.3%) with hemorrhage. Preoperative Suzuki stage was III or higher in 50 hemispheres (75.8%) and 3 patients had undergone prior treatments to the affected hemisphere before pial synangiosis surgery. Clinical follow-up was available for an average of 5.8 years (range 0.6–14.1 years), with 26 patients (72.2%) followed for longer than 2 years. Postoperative angiography was available for 24 patients and 46 revascularized hemispheres, and 39 (84.8%) of the 46 hemispheres demonstrated good collateral formation (Matsushima Grade A or B). Postoperative complications included 3 strokes, 5 TIAs, and 2 seizures, and there was no hemorrhage during the follow-up period. One patient required additional revascularization surgery 8 months after pial synangiosis.

Conclusions

Pial synangiosis is a safe and durable method of cerebral revascularization in adult patients with moyamoya and can be considered as a potential treatment option for moyamoya disease in adults.

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Cavernous malformations of the brain after treatment for acute lymphocytic leukemia: presentation and long-term follow-up

Clinical article

Amit Singla, Jill E. Brace O'Neill, Edward Smith, and R. Michael Scott

Object

The authors undertook this study to determine the clinical course and long-term outcomes in pediatric patients who developed cavernous malformations of the brain following treatment for acute lymphocytic leukemia (ALL).

Methods

They reviewed the senior author's database of surgically treated cavernous malformations of the brain to identify those patients whose cavernous malformations developed after cranial radiation during treatment for ALL. The medical records of these patients were reviewed to determine their clinical presentation, radiological findings, and outcome at long-term follow-up.

Results

Five patients fulfilled the specified criteria over a 23-year period. At the time of ALL diagnosis, they were all 4–5 years old. The cerebral cavernous malformations developed 2–8 years after cranial radiation, and 4 of the 5 patients presented with neurological symptoms, which ranged from focal deficits to seizures. Two patients required a second craniotomy, one from lesion recurrence possibly due to incomplete resection, and another for a second cavernous malformation, which developed at another site 6 years after the initial malformation was excised. Long-term follow-up of 2, 10, 11, 11, and 17 years has revealed no additional lesion development or recurrence.

Conclusions

Symptomatic cavernous malformations of the brain may develop several years after chemotherapy and cranial radiation treatment for ALL, and the clinical course of these cavernous malformations may be more aggressive than that of the typical post-radiation lesions seen in other conditions. Long-term clinical and imaging monitoring is recommended for children who have undergone treatment for ALL. Craniotomy for excision of the malformations appears to convey long-term protection from repeat hemorrhage and accumulating neurological deficits.

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Pial synangiosis in patients with moyamoya younger than 2 years of age

Clinical article

Eric M. Jackson, Ning Lin, Sunil Manjila, R. Michael Scott, and Edward R. Smith

Object

Patients with moyamoya who are younger than 2 years of age represent a therapeutic challenge because of their frequent neurological instability and concomitant anesthetic risks. The authors report their experience with pial synangiosis revascularization in this population.

Methods

The authors reviewed the clinical and radiographic records of all patients with moyamoya in a consecutive series of patients under 2 years of age, who underwent cerebral revascularization surgery using pial synangiosis at a single institution.

Results

During a 12-year period (1994–2005), 34 procedures (bilateral in 15 patients, unilateral in 4) were performed in 19 patients younger than 2 years (out of a total of 456 procedures in 240 patients). Eighteen of these patients presented with either stroke or transient ischemic attack. The average age of the 19 patients at first surgery was 1.4 years (range 6 months–1.9 years). Unanticipated staged operations occurred in 3 patients, due to persistent electroencephalographic changes during the initial surgery in 2 cases and due to brain swelling during the procedure requiring ventriculostomy in the other. There were 2 perioperative strokes; both patients had postoperative seizures but made clinical recoveries. The average follow-up was 7 years (range 1–14 years). Long term, at follow-up, 13 patients (68%) were clinically independent for their age, with 8 (42%) having no significant deficit. Late complications included subdural hygroma evacuation (1), additional revascularization procedures performed years later for frontal lobe ischemia (2), late infarction (1), and asymptomatic ischemic change on routine follow-up MRI studies (1). All patients who had both pre- and postoperative angiography demonstrated progression of disease.

Conclusions

Despite the challenges inherent to this population, the majority of children with moyamoya under the age of 2 years have a good long-term prognosis. The data from this study support the use of pial synangiosis as a safe, effective, and durable method for treatment of moyamoya for most children in this potentially high-risk population.

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Microsurgical treatment of arteriovenous malformations in pediatric patients: the Boston Children's Hospital experience

Bradley A. Gross, Armide Storey, Darren B. Orbach, R. Michael Scott, and Edward R. Smith

OBJECT

Outcomes of microsurgical treatment of arteriovenous malformations (AVMs) in children are infrequently reported across large cohorts.

METHODS

The authors undertook a retrospective review of departmental and hospital databases to obtain the medical data of all patients up to 18 years of age who were diagnosed with cerebral AVMs. Demographic and AVM angioarchitectural characteristics were analyzed, and for the patients who underwent surgery, the authors also analyzed the estimated intraoperative blood loss, postoperative angiographically confirmed obliteration rates, and neurological complications and outcomes classified according to the modified Rankin Scale (mRS).

RESULTS

Of 117 children with cerebral AVMs, 94 underwent microsurgical resection (80%). Twenty (21%) of these 94 patients underwent adjunctive preoperative embolization. The overall postoperative angiographically confirmed obliteration rate was 94%. As part of a new protocol, the last 50 patients in this series underwent immediate perioperative angiography, improving the subsequent obliteration rate from 86% to 100% (p = 0.01). No other factors, such as a hemorrhagic AVM, size of the AVM, location, drainage, or Spetzler-Martin grade, had a statistically significant impact on the obliteration rate. Perioperative neurological deficits occurred in 17% of the patients, but the vast majority of these (77%) were predictable visual field cuts. Arteriovenous malformations that were hemorrhagic or located in noneloquent regions were each associated with lower rates of postoperative neurological complications (p = 0.05 and 0.002, respectively). In total, 94% of the children had good functional outcomes (mRS Scores 0–2), and these outcomes were significantly influenced by the mRS score on presentation before surgery (p = 0.01). A review of 1- and 5-year follow-up data indicated an overall annual hemorrhage rate of 0.3% and a recurrence rate of 0.9%.

CONCLUSIONS

Microsurgical resection of AVMs in children is associated with high rates of angiographically confirmed obliteration and low rates of significant neurological complications. Implementation of a protocol using perioperative angiography in this series led to complete radiographically confirmed obliteration of all AVMs, with low annual repeat hemorrhage and recurrence rates.