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Geoffrey P. Colby, Bowen Jiang, Matthew T. Bender, Narlin B. Beaty, Erick M. Westbroek, Risheng Xu, Li-Mei Lin, Jessica K. Campos, Rafael J. Tamargo, Judy Huang, Alan R. Cohen, and Alexander L. Coon

Intracranial aneurysms in the pediatric population are rare entities. The authors recently treated a 9-month-old infant with a 19-mm recurrent, previously ruptured, and coil-embolized left middle cerebral artery (MCA) pseudoaneurysm, which was treated definitively with single-stage Pipeline-assisted coil embolization. The patient was 5 months old when she underwent resection of a left temporal Grade 1 desmoplastic infantile ganglioglioma at an outside institution, which was complicated by left MCA injury with a resultant 9-mm left M1 pseudoaneurysm. Within a month, the patient had two aneurysmal rupture events and underwent emergency craniectomy for decompression and evacuation of subdural hematoma. The pseudoaneurysm initially underwent coil embolization; however, follow-up MR angiography (MRA) revealed aneurysm recanalization with saccular enlargement to 19 mm. The patient underwent successful flow diversion–assisted coil embolization at 9 months of age. At 7 months after the procedure, follow-up MRA showed complete aneurysm occlusion without evidence of in-stent thrombosis or stenosis. Experience with flow diverters in the pediatric population is still in its early phases, with the youngest reported patient being 22 months old. In this paper the authors report the first case of such a technique in an infant, whom they believe to be the youngest patient to undergo cerebral flow diversion treatment.

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Sunil Manjila, Gagandeep Singh, Obinna Ndubuizu, Zoe Jones, Daniel P. Hsu, and Alan R. Cohen

The authors demonstrate the use of an endovascular plug in securing a carotid artery pseudoaneurysm in an emergent setting requiring craniotomy for a concurrent subdural empyema.

They describe the case of a 14-year-old boy with sinusitis and bifrontal subdural empyema who underwent transsphenoidal exploration at an outside hospital. An injury to the right cavernous segment of the ICA caused torrential epistaxis. Bleeding was successfully controlled by inflating a Foley balloon catheter within the sphenoid sinus, and the patient was transferred to the authors’ institution. Emergent angiography showed a dissection of the right cavernous carotid artery, with a large pseudoaneurysm projecting into the sphenoid sinus at the site of arterial injury. The right internal carotid artery was obliterated using pushable coils distally and an endovascular plug proximally. The endovascular plug enabled the authors to successfully exclude the pseudoaneurysm from the circulation. The patient subsequently underwent an emergent bifrontal craniotomy for evacuation of a left frontotemporal subdural empyema and exenteration of both frontal sinuses. He made a complete neurological recovery.

Endovascular large-vessel sacrifice, obviating the need for numerous coils and antiplatelet therapy, has a role in the setting of selected acute neurosurgical emergencies necessitating craniotomy. The endovascular plug is a useful adjunct in such circumstances as the device can be deployed rapidly, safely, and effectively.

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Roberta Rehder, Subash Lohani, and Alan R. Cohen

Donald Darrow Matson made seminal contributions to the field of pediatric neurosurgery. Born in 1913 in Fort Hamilton, New York, Matson was the youngest of four sons of an army colonel. He graduated from Cornell University and, years later, from Harvard Medical School. Matson selected Peter Bent Brigham Hospital for his neurosurgical training, which was interrupted during World War II. As a neurosurgeon, he worked close to the front lines under Brigadier General Elliot Cutler in Europe, earning a Bronze Star. Matson returned to Boston to become Franc Ingraham’s fellow and partner. He was a masterful surgeon and, with Ingraham, published Neurosurgery of Infancy and Childhood in 1954, the first pediatric neurosurgery textbook in the world. Upon Ingraham’s retirement, Matson became chairman of the department of neurosurgery at Boston Children’s Hospital and Peter Bent Brigham. In 1968, he became the inaugural Franc D. Ingraham Professor of Neurological Surgery at Harvard Medical School. Among his neurosurgical accomplishments, Matson served as President of the Harvey Cushing Society, later known as the American Association of Neurological Surgeons. He was unable to preside at the 1969 meeting that marked the 100th anniversary of Cushing’s birth, having contracted Creutzfeldt-Jakob disease. Matson died at the age of 55, surviving his mentor Ingraham by only 4 years.

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Alan R. Cohen, Timothy W. Vogel, and Hart G. W. Lidov

The authors describe an 8-year-old girl who underwent emergency posterior fossa surgery for resection of a large cerebellar astrocytoma in November 1939. The surgery was carried out by Franc D. Ingraham at Boston Children's Hospital a decade after he established the first pediatric neurosurgical service in the world at the same institution. Four years later the tumor recurred and the patient underwent repeat resection followed by external-beam radiation therapy. The pathological diagnosis by Sidney Farber was fibrillary astrocytoma.

The young girl is currently a healthy, functional 82-year-old woman. The authors believe that this 74-year follow-up represents one of the longest in history, if not the longest, of a patient undergoing resection of a brain tumor. A recent MRI study shows postoperative changes with no evidence of residual or recurrent tumor. The original block tissue specimen had been preserved. It was restained and examined, revealing the pathological diagnosis to be juvenile pilocytic astrocytoma.

The case is analyzed in the context of Ingraham's powerful and lasting impact on the field of pediatric neurosurgery.

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Farideh Nejat, Pari Zarrini, and Mostafa El Khashab

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Sunil Manjila, Erin Miller, Amad Awadallah, Shunichi Murakami, Mark L. Cohen, and Alan R. Cohen

True ossification within benign brain tumors is rare, and the molecular mechanism for this process is poorly understood. The authors report a case of ossified choroid plexus papilloma (CPP) and analyze it to help elucidate the underlying molecular basis of osteogenesis in benign brain tumors. A 21-year-old man presented with headache and depression that progressed over years. Computed tomography, MRI, and angiography demonstrated a large heavily calcified fourth ventricular tumor with a vascular blush and no hydrocephalus. The tumor was resected and was found to be an ossified CPP. Immunohistochemical staining for VEGF, Sox2, BMP-2, osterix, osteopontin, and osteocalcin was performed in an attempt to elucidate the mechanism of bone formation. The tumor was extensively ossified with mature bone trabeculae. Immunostaining for VEGF was positive. Additional staining showed the presence of osteocalcin in this ossified tumor but not in samples of nonossified CPPs collected from other patients. Staining for osterix and osteopontin was equivocally positive in the ossified CPP but also in the nonossified CPPs examined. The presence of osteocalcin in the ossified CPP demonstrates that there is true bone formation rather than simple calcification. Its appearance within cells around the trabeculae suggests the presence of osteoblasts. The presence of osterix suggests that a pluripotent cell, or one that is already partially differentiated, may be differentiated into an osteoblast through this pathway. This represents the first systematic immunohistochemical analysis of osteogenesis within choroid plexus tumors.

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Timothy W. Vogel, Biji Bahuleyan, Shenandoah Robinson, and Alan R. Cohen

Object

Hydrocephalus remains a major public health problem. Conventional treatment has relied on extracranial shunting of CSF to another systemic site, but this approach is associated with a high rate of complications. Endoscopic third ventriculostomy (ETV) is a novel treatment for select forms of hydrocephalus that can eliminate the need for implantation of a lifelong ventricular shunt system. However, the indications for ETV are contested and its long-term effectiveness is not well established.

Methods

The authors selected 100 consecutive patients who underwent ETV for hydrocephalus beginning in 1994. Patients were enrolled and treated at a single institution by a single surgeon. The primary outcome was success of ETV, with success defined as no need for subsequent surgery for hydrocephalus.

Results

Ninety-five patients satisfied the inclusion criteria. The mean follow-up period was 5.1 years (median 4.7 years) with follow-up data available for as long as 17 years. Patients commonly presented with headache (85%), ataxia (34%), emesis (29%), and changes in vision (27%). The success rate for ETV was 75%. Twenty-one patients (22%) in the series had malfunctioning shunts preoperatively and 13 (62%) were successfully treated with ETV. Preoperative inferior bowing of the third ventricle floor on MRI was significantly associated with ETV success (p < 0.05).

Conclusions

Endoscopic third ventriculostomy is an effective and durable treatment for select patients with hydrocephalus. When successful, the procedure eliminates the lifelong complications associated with implanted ventricular shunts.

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Subash Lohani and Alan R. Cohen

In 1929, Franc D. Ingraham, Harvey Cushing's protégé, established the first pediatric neurosurgical unit in the world at Boston Children's Hospital and dedicated his career to the neurosurgical care of children. He trained with both Cushing and Dandy and spent 1 year working in Oxford with Sherrington, who considered Ingraham to be the finest operative surgeon ever to work in his laboratory. Ingraham was instrumental in developing novel treatments, which he compiled in his classic book, Neurosurgery of Infancy and Childhood. Although he was modest and shy, Ingraham loved to entertain children with magic and enjoyed photography in and out of the operating room. Unfortunately, his career was plagued by personal illness, and he died young in 1965 at the age of 67. Despite his prolific 36-year neurosurgical career, Ingraham remained an associate professor at Harvard at his retirement. To recognize his remarkable contributions, Harvard established an endowed chair in his name in 1967. Ingraham was a pioneer and a leader in the development of pediatric neurosurgery by virtue of his imagination, intelligence, and ability to lead and inspire others. Cushing has come to be regarded as the founder of neurosurgery. It is fair to conclude that Ingraham, his disciple, is the founder of pediatric neurosurgery.

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John Weaver, Sunil Manjila, Biji Bahuleyan, Barbara A. Bangert, and Alan R. Cohen

Object

Rhombencephalosynapsis (RS) is a rare congenital posterior fossa malformation characterized by dorsal fusion of the cerebellar hemispheres, hypogenesis or agenesis of the vermis, and fusion of the dentate nuclei and superior cerebellar peduncles. The objective of this institutional study is to review the clinical conditions associated with RS and analyze the varied biological profile of this unique condition.

Methods

The study data were collected retrospectively from the medical records of patients at Rainbow Babies and Children's Hospital. After required institutional review board approval, the authors obtained information regarding the cases of RS reviewed by the Departments of Radiology, Genetics, and/or Pediatric Neurology. Medical charts were systematically reviewed, and 9 patients were analyzed in detail.

Results

The authors describe 6 cases of RS and 3 cases of partial RS. This case series demonstrates an association between RS and symptomatic hydrocephalus (7 of 9 patients) and RS and Chiari malformation (5 of 9 patients). Patients with symptomatic hydrocephalus underwent endoscopic third ventriculostomy or ventriculoperitoneal shunt insertion. One of the patients with an associated Chiari malformation underwent foramen magnum decompression.

Conclusions

The authors present a large case series of RS. Patients with RS often had hydrocephalus and/or a Chiari Type I or II hindbrain malformation. Neuroimaging findings of RS are presented along with hypotheses to explain the embryopathology of this unusual condition.

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Nima Alan, Sunil Manjila, Nori Minich, Nancy Bass, Alan R. Cohen, Michele Walsh, and Shenandoah Robinson

Object

Although survival for extremely low gestational age newborns (ELGANs) has improved in the past 3 decades, these infants remain prone to complications of prematurity, including intraventricular hemorrhage (IVH). The authors reviewed the outcomes for an entire cohort of ELGANs who suffered severe IVH at their institution during the past 12 years to gain a better understanding of the natural history of IVH and frequency of ventriculoperitoneal (VP) shunt placement in this population.

Methods

Data from the neonatal ICU (NICU) database, neurosurgery operative log, and medical records were used to identify and follow up all ELGANs who suffered a severe IVH between 1997 and 2008. Trends between Period 1 (1997–2001) and Period 2 (2004–2008) were analyzed using the Pearson chi-square test.

Results

Between 1997 and 2008, 1335 ELGANs were admitted to the NICU at the authors' institution within 3 days of birth, and 111 (8.3%) of these infants suffered a severe IVH. Survival to 2 years, incidence of severe IVH, neonatal risk factors (gestational age, birth weight, and incidence of necrotizing enterocolitis), ventriculomegaly on cranial ultrasonography, and use of serial lumbar punctures for symptomatic hydrocephalus were all stable. Infants from Period 2 had a significantly lower incidence of bronchopulmonary dysplasia and sepsis than infants from Period 1 (both p < 0.001). All ELGANs with severe IVH and ventriculomegaly underwent long-term follow-up to identify shunt status at late follow-up. Twenty-two ELGANs (20%) with severe IVH required a temporary ventriculosubgaleal (VSG) shunt. Three infants with VSG shunts showed spontaneous hydrocephalus resolution, and 2 infants died of unrelated causes during the neonatal admission. The temporary VSG shunt complication rate was 20% (12% infection and 8% malfunction). Sixteen percent of all ELGANs (18 of 111) with severe IVH eventually required permanent ventricular shunt insertion. Six (35%) of 17 infants with a permanent VP shunt required at least 1 permanent shunt revision during the 1st year. The proportion of ELGANs with severe IVH who required a temporary VSG (35%) or permanent VP shunt (30%) during Period 1 decreased by more than 60% in Period 2 (10% [p = 0.005] and 8.3% [p = 0.009], respectively).

Conclusions

The authors report for the first time a marked reduction over the past 12 years in the proportion of ELGANs with severe IVH who required surgical intervention for hydrocephalus. Using the NICU database, the authors were able to identify and follow all ELGANs with severe IVH and ventriculomegaly. They speculate that the reduction in ventricular shunt rate results from improved neonatal medical care, including reduced infection, improved bronchopulmonary dysplasia, and postnatal steroid avoidance, which may aid innate repair mechanisms. Multicenter prospective trials and detailed analyses of NICU parameters of neonatal well-being are needed to understand how perinatal factors influence the propensity to require ventricular shunting.