Laurie L. Ackerman

Cyst-ventricle stent as primary or salvage treatment for posterior fossa arachnoid cysts: Clinical article

OBJECT The optimal treatment of symptomatic posterior fossa arachnoid cysts is controversial. Current options include open or endoscopic resection, fenestration, or cyst-peritoneal shunt placement. There are potential drawbacks with all options. Previous authors have described stenting a cyst into the ventricular system for supratentorial lesions. The current authors have used a similar strategy for posterior fossa cysts. METHODS The authors performed a retrospective review of 79 consecutive patients (1993-2010) with surgically treated intracranial arachnoid cysts. RESULTS The authors identified 3 patients who underwent placement of a stent from a posterior fossa arachnoid cyst to a supratentorial ventricle. In 2 patients the stent construct consisted of a catheter placed into a posterior fossa arachnoid cyst and connecting to a lateral ventricle catheter. Both patients underwent stent placement as a salvage procedure after failure of open surgical fenestration. In the third patient a single-catheter cyst-ventricle stent was stereotactically placed. All 3 patients improved clinically. Two patients remained asymptomatic, with radiographic stability in a follow-up period of 1 and 5 years, respectively. The third patient experienced initial symptom resolution with a demonstrable reduction of intracystic pressure. However, he developed recurrent headaches after 2 years. CONCLUSIONS Posterior fossa cyst-ventricle stenting offers the benefits of ease of surgical technique and a low morbidity rate. It may also potentially reduce the incidence of shunt-related headaches by equalizing the pressure between the posterior fossa and the supratentorial compartments. While fenestration is considered the first-line therapy for most symptomatic arachnoid cysts, the authors consider cyst-ventricle stenting to be a valuable additional strategy in treating these rare and often difficult lesions.

Circumferential fixation with craniofacial miniplates for a cervical spine injury in a child: Case report

Unstable pediatric cervical spine injuries present significant challenges in terms of fixation. Given the smaller cervical vertebral bodies in the preschool-aged population, commercially available pediatric cervical fixation instrumentation may be unsuitable because of the inappropriately large size of the screws and plates. The authors describe a 2-year-old girl who sustained an unstable C6-7 distraction injury during a motor vehicle accident. Because of the small size of her vertebral bodies, standard cervical spine instrumentation was not feasible, and posterior wiring alone was believed to be insufficient because of the complete distraction of all 3 spinal columns. The patient was taken to the operating room where craniofacial plates with an inherent locking mechanism were used to circumferentially stabilize the cervical spine. Follow-up examination 6 months postoperatively demonstrated stable cervical spine alignment and fusion with no evidence of the failure of either the anterior or posterior hardware. The use of craniofacial miniplates with an intrinsic locking mechanism represents a superior alternative for both anterior and posterior cervical fixations when spinal instrumentation is needed in the pediatric age group.

Intracranial chordoma presenting as acute hemorrhage in a child: Case report and literature review.

Background: Chordomas are rare, slow-growing malignant neoplasms derived from remnants of the embryological notochord. Pediatric cases comprise only 5% of all chordomas, but more than half of the reported pediatric chordomas are intracranial. For patients of all ages, intracranial chordomas typically present with symptoms such as headaches and progressive neurological deficits occurring over several weeks to many years as they compress or invade local structures. There are only reports of these tumors presenting acutely with intracranial hemorrhage in adult patients. Case Description: A 10-year-old boy presented with acute onset of headache, emesis, and diplopia. Head computed tomography and magnetic resonance imaging of brain were suspicious for a hemorrhagic mass located in the left petroclival region, compressing the ventral pons. The mass was surgically resected and demonstrated acute intratumoral hemorrhage. Pathologic examination was consistent with chordoma. Conclusion: There are few previous reports of petroclival chordomas causing acute intracranial hemorrhage. To the authors’ knowledge, this is the first case of a petroclival chordoma presenting as acute intracranial hemorrhage in a pediatric patient. Although uncommon, it is important to consider chordoma when evaluating a patient of any age presenting with a hemorrhagic lesion of the clivus.

Risk of deep venous thrombosis in elective neurosurgical procedures: a prospective, Doppler ultrasound–based study in children 12 years of age or younger

OBJECTIVE The risk of venous thromboembolism (VTE) from deep venous thrombosis (DVT) is significant in neurosurgical patients. VTE is considered a leading cause of preventable hospital deaths and preventing DVT is a closely monitored quality metric, often tied to accreditation, hospital ratings, and reimbursement. Adult protocols include prophylaxis with anticoagulant medications. Childrens hospitals may adopt adult protocols, although the incidence of DVT and the risk or efficacy of treatment is not well defined. The incidence of DVT in children is likely less than in adults, although there is very little prospectively collected information. Most consider the risk of DVT to be extremely low in children 12 years of age or younger. However, this consideration is based on tradition and retrospective reviews of trauma databases. In this study, the authors prospectively evaluated pediatric patients undergoing a variety of elective neurosurgical procedures and performed Doppler ultrasound studies before and after surgery. METHODS A total of 100 patients were prospectively enrolled in this study. All of the patients were between the ages of 1 month and 12 years and were undergoing elective neurosurgical procedures. The 91 patients who completed the protocol received a bilateral lower-extremity Doppler ultrasound examination within 48 hours prior to surgery. Patients did not receive either medical or mechanical DVT prophylaxis during or after surgery. The ultrasound examination was repeated within 72 hours after surgery. An independent, board-certified radiologist evaluated all sonograms. We prospectively collected data, including potential risk factors, details of surgery, and details of the clinical course. All patients were followed clinically for at least 1 year. RESULTS There was no clinical or ultrasound evidence of DVT or VTE in any of the 91 patients. There was no clinical evidence of VTE in the 9 patients who did not complete the protocol. CONCLUSIONS In this prospective study, no DVTs were found in 91 patients evaluated by ultrasound and 9 patients followed clinically. While the study is underpowered to give a definitive incidence, the data suggest that the risk of DVT and VTE is very low in children undergoing elective neurosurgical procedures. Prophylactic protocols designed for adults may not apply to pediatric patients. Clinical trial registration no.: NCT02037607 (clinicaltrials.gov).

Unusual causes of papilledema: Two illustrative cases

Background: Neurosurgeons are frequently among the first physicians asked to evaluate patients with papilledema, and the patient is often referred with the implication that they may require shunting. After an initial evaluation to exclude potential neurosurgical emergencies, the physician should carefully consider various etiologies of papilledema to prevent unnecessary neurosurgical operations. Case Description: The authors report two illustrative cases of unusual causes of papilledema: Anemia and leukemic infiltration of the central nervous system. In each case, a complete blood count provided clues for the diagnosis. A review of the literature is also included. Conclusions: Both patients responded to medical management/treatment of the underlying disease and did not require neurosurgical operative intervention. Papilledema may be caused by other etiologies besides increased intracranial pressure. The authors present two unusual cases leading to papilledema and provide an outline for the workup of these conditions.

Severe hydronephrosis caused by an infected intra-abdominal fluid collection in a patient with a ventriculoperitoneal shunt

CaseThe authors report a case of a 10-year-old boy with myelodysplasia and shunted hydrocephalus who developed acute severe hydronephrosis presumed secondary to a large abdominal cerebrospinal fluid (CSF) collection.MethodThe patient had recently begun having trouble catheterizing his Monti channel, and had been diagnosed and treated for an Escherichia Coli urinary tract infection. He presented with abdominal pain, and an abdominal CT demonstrated severe hydronephrosis and a large abdominal fluid collection presumed to be a CSFoma. The shunt was externalized, and fluid consistent with CSF was removed by aspiration of the shunt catheter at the time of surgery and by interventional radiology placement of a pigtail drain into the remaining fluid collection. Endoscopic evaluation of the Monti channel revealed evidence of healed perforation. A suprapubic catheter was placed and the Monti channel remained catheterized. The shunt was eventually converted to a ventriculoatrial shunt. A postoperative renal ultrasound later demonstrated marked improvement in the hydronephrosis and mild caliectasis. The suprapubic catheter and the indwelling Monti catheter were removed. The patient resumed catheterization of the Monti channel.ResultFollow-up ultrasounds have shown stable, mild hydronephrosis, consistent with the patient’s baseline.ConclusionsPractitioners should have a high index of suspicion for potential shunt contamination and CSFoma development in patients with Monti channels and a history of difficult catheterization.

Pediatric Pontine Cavernous Malformations: The Presigmoid, Posterior Petrosal Approach

BACKGROUND Brainstem cavernous malformations (cavernomas) in children have a high risk of hemorrhage and neurological deterioration. This risk is magnified if the child has a genetic predisposition for cavernoma formation. The surgical management is challenging and carries a significant risk of morbidity. OBJECTIVE To describe the feasibility of a posterior petrosal approach to brainstem cavernomas in a pediatric population. METHODS A single institution operative experience with this technique was reviewed; 2 cases were identified and are technically described here with supportive figures and illustrations, as well as a focused literature review. RESULTS Two pediatric cases with multiple symptomatic hemorrhages from large expanding pontine cavernomas were identified. Both cavernomas were resected through a presigmoid posterior petrosal approach. While this approach is well described in the adult literature for ventral brainstem lesions, its description for the treatment of pontine cavernomas in the pediatric populations is scarce. CONCLUSION This study demonstrates the utility and feasibility of the posterior petrosal approach in two pediatric patients at different points in cranial base development.

Decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry

OBJECTIVEThe purpose of this study was to determine the rate of decompression for Chiari malformation type II in individuals with myelomeningocele in the National Spina Bifida Patient Registry (NSBPR). In addition, the authors explored the variation in rates of Chiari II decompression across NSBPR institutions, examined the relationship between Chiari II decompression and functional lesion level of the myelomeningocele, age, and need for tracheostomy, and they evaluated for temporal trends in rates of Chiari II decompression.METHODSThe authors queried the NSBPR to identify all individuals with myelomeningocele between 2009 and 2015. Among these patients, they identified individuals who had undergone at least 1 Chiari II decompression as well as those who had undergone tracheostomy. For each participating NSBPR institution, the authors calculated the proportion of patients enrolled at that site who underwent Chiari II decompression. Logistic regression was performed to analyze the relationship between Chiari II decompression, functional lesion level, age at decompression, and history of tracheostomy.RESULTSOf 4448 individuals with myelomeningocele identified from 26 institutions, 407 (9.15%) had undergone at least 1 Chiari II decompression. Fifty-one patients had undergone tracheostomy. Logistic regression demonstrated a statistically significant relationship between Chiari II decompression and functional lesion level of the myelomeningocele, with a more rostral lesion level associated with a higher likelihood of posterior fossa decompression. Similarly, children born before 2005 and those with history of tracheostomy had a significantly higher likelihood of Chiari II decompression. There was no association between functional lesion level and need for tracheostomy. However, among those children who underwent Chiari II decompression, the likelihood of also undergoing tracheostomy increased significantly with younger age at decompression.CONCLUSIONSThe rate of Chiari II decompression in patients with myelomeningocele in the NSBPR is consistent with that in previously published literature. There is a significant relationship between Chiari II decompression and functional lesion level of the myelomeningocele, which has not previously been reported. Younger children who undergo Chiari II decompression are more likely to have undergone tracheostomy. There appears to be a shift away from Chiari II decompression, as children born before 2005 were more likely to undergo Chiari II decompression than those born in 2005 or later.

Loss of mechanosensitive sclerostin may accelerate cranial bone growth and regeneration

OBJECTIVE Cranial defects can result from trauma, infection, congenital malformations, and iatrogenic causes and represent a surgical challenge. The current standard of care is cranioplasty, with either autologous or allogeneic material. In either case, the intrinsic vascularity of the surrounding tissues allows for bone healing. The objective of this study was to determine if mechanotransductive gene manipulation would yield non-weight-bearing bone regeneration in a critical size calvarial defect in mice. METHODS A mouse model of Sost deletion in Sost knockout (KO) mice was created in which the osteocytes do not express sclerostin. A critical size calvarial defect (4 mm in diameter) was surgically created in the parietal bone in 8-week-old wild-type (n = 8) and Sost KO (n = 8) male mice. The defects were left undisturbed (no implant or scaffold) to simulate a traumatic calvariectomy model. Eight weeks later, the animals were examined at necropsy by planimetry, histological analysis of new bone growth, and micro-CT scanning of bone thickness. RESULTS Defects created in wild-type mice did not fill with bone over the study period of 2 months. Genetic downregulation of sclerostin yielded animals that were able to regenerate 40% of the initial critical size defect area 8 weeks after surgery. A thin layer of bone covered a significant portion of the original defect in all Sost KO animals. A statistically significant increase in bone volume (p < 0.05) was measured in Sost KO mice using radiodensitometric analysis. Immunohistochemical analysis also confirmed that this bone regeneration occurred through the Wnt pathway and originated from the edge of the defect; BMP signaling did not appear to be affected by sclerostin. CONCLUSIONS Mechanical loading is an important mechanism of bone formation in the cranial skeleton and is poorly understood. This is partially due to the fact that it is difficult to load bone in the craniomaxillofacial skeleton. This study suggests that modulation of the Wnt pathway, as is able to be done with monoclonal antibodies, is a potentially efficacious method for bone regeneration that requires further study.