✓ The authors report the case of a newborn presenting at birth with macrocephaly and a large pineal region hemorrhagic cyst without neurological deficit. No neurosurgical intervention was performed, and subsequent imaging studies demonstrated complete involution of the cyst.
Anitha Nimmagadda, David I. Sandberg and John Ragheb
Parthasarathi Chamiraju, Sanjiv Bhatia, David I. Sandberg and John Ragheb
The aim of this study was to determine the role of endoscopic third ventriculostomy and choroid plexus cauterization (ETV/CPC) in the management of posthemorrhagic hydrocephalus of prematurity (PHHP) and to analyze which factors affect patient outcomes.
This study retrospectively reviewed medical records of 27 premature infants with intraventricular hemorrhage (IVH) and hydrocephalus treated with ETV and CPC from 2008 to 2011. All patients were evaluated using MRI before the procedure to verify the anatomical feasibility of ETV/CPC. Endoscopic treatment included third ventriculostomy, septostomy, and bilateral CPC. After ETV/CPC, all patients underwent follow-up for a period of 6–40 months (mean 16.2 months). The procedure was considered a failure if the patient subsequently required a shunt. The following factors were analyzed to determine a relationship to patient outcomes: gestational age at birth, corrected age and weight at surgery, timing of surgery after birth, grade of IVH, the status of the prepontine cistern and cerebral aqueduct on MRI, need for a ventricular access device prior to the endoscopic procedure, and scarring of the prepontine cistern noted at surgery.
Seventeen (63%) of 27 patients required a shunt after ETV/CPC, and 10 patients did not require further CSF diversion. Several factors studied were associated with a higher rate of ETV/CPC failure: Grade IV hemorrhage, weight 3 kg or less and age younger than 3 months at the time of surgery, need for reservoir placement, and presence of a normal cerebral aqueduct. Two factors were found to be statistically significant: the patient's corrected gestational age of less than 0 weeks at surgery and a narrow prepontine cistern on MRI. The majority (83%) of ETV/CPC failures occurred in the first 3 months after the procedure. None of the patients had a complication directly related to the procedure.
Endoscopic third ventriculostomy/CPC is a safe initial procedure for hydrocephalus in premature infants with IVH and hydrocephalus, obviating the need for a shunt in selected patients. Even though the success rate is low (37%), the lower rate of complications in comparison with shunt treatment may justify this procedure in the initial management of hydrocephalus. As several of the studied factors have shown influence on the outcome, patient selection based on these observations might increase the success rate.
Anna Lo Presti, Alexander G. Weil and John Ragheb
Stacey Quintero Wolfe, Sanjiv Bhatia, Barth Green and John Ragheb
✓The authors report on a 17-year-old boy with cervical myelopathy from dilated epidural veins due to cerebrospinal fluid (CSF) overdrainage. The patient had a long-standing subdural–peritoneal shunt and presented with incapacitating spastic tetraparesis. Magnetic resonance imaging revealed significant cervical spinal cord compression from a markedly dilated epidural venous plexus. The shunt was externalized so that CSF flow dynamics could be assessed, and the patient was found to have low intracranial pressure (ICP). The patient was gradually acclimated to higher ICPs, and a new shunt was placed with an antisiphon device and a programmable valve set at the higher pressure. Postoperatively the child experienced significant clinical improvement, and reduction of spinal cord compression was evident on images. Compensatory engorgement of the epidural venous plexus due to long-term shunt usage should be considered in the differential diagnosis when cervical myelopathy due to a dilated epidural venous plexus is present.
Anomalous venous drainage preventing safe posterior fossa decompression in patients with Chiari malformation Type I and multisutural craniosynostosis
Report of two cases and review of the literature
David I. Sandberg, Ramon Navarro, Josep Blanch and John Ragheb
✓ The authors report on two children in whom an anomalous posterior fossa venous drainage pattern prevented safe posterior fossa decompression. Both patients had Chiari malformation Type I, multisutural craniosynostosis, and crowded posterior fossa structures. Both patients had been treated with ventriculoperitoneal shunts for hydrocephalus. Pfeiffer syndrome had been diagnosed in one of the patients, and the other was suspected to have osteogenesis imperfecta. Although both patients were believed to have symptoms resulting from brainstem compression, posterior fossa decompression was not offered due to profound venous anomalies noted on imaging studies that greatly increased the expected risks associated with surgery. These cases are presented to alert neurosurgeons to carefully evaluate the posterior fossa venous anatomy prior to considering posterior fossa decompression with or without occipitocervical fusion or calvarial vault remodeling procedures in patients with multisutural craniosynostosis.
Michael Ragheb, Ashish H. Shah, Sarah Jernigan, Tulay Koru-Sengul and John Ragheb
Hydrocephalus is recognized as a common disabling pediatric disease afflicting infants and children disproportionately in the developing world, where access to neurosurgical care is limited and risk of perinatal infection is high. This surgical case series describes the Project Medishare Hydrocephalus Specialty Surgery (PMHSS) program experience treating hydrocephalus in Haiti between 2008 and 2015.
The authors conducted a retrospective review of all cases involving children treated for hydrocephalus within the PMHSS program in Port-au-Prince, Haiti, from 2008 through 2015. All relevant epidemiological information of children treated were prospectively collected including relevant demographics, birth history, hydrocephalus etiology, head circumference, and operative notes. All appropriate associations and statistical tests were performed using univariate and multivariate logistic regression analyses.
Among the 401 children treated within PMHSS, postinfectious hydrocephalus (PIH) accounted for 39.4% (n = 158) of cases based on clinical, radiographic, and endoscopic findings. The majority of children with hydrocephalus in Haiti were male (54.8%, n = 197), born in the rainy season (59.7%, n = 233), and born in a coastal/inland location (43.3%, n = 61). The most common surgical intervention was endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) (45.7%, n = 175). Multivariate logistic regression analysis yielded coastal birth location (OR 3.76, 95% CI 1.16–12.18) as a statistically significant predictor of PIH. Increasing head circumference (adjusted OR 1.06, 95% CI 0.99–1.13) demonstrated a slight trend toward significance with the incidence of PIH.
This information will provide the foundation for future clinical and public health studies to better understand hydrocephalus in Haiti. The 39.4% prevalence of PIH falls within observed rates in Africa as does the apparently higher prevalence for those born during the rainy season. Although PIH was the most frequent etiology seen in almost all birth locations, the potential relationship with geography noted in this series will be the focus of further research in an effort to understand the link between climate and PIH in Haiti. The ultimate goal will be to develop an appropriate public health strategy to reduce the burden of PIH on the children of Haiti.
Sanjiv Bhatia, Faiz Ahmad, Ian Miller, John Ragheb, Glenn Morrison, Prasanna Jayakar and Michael Duchowny
Refractory status epilepticus (RSE) is a life-threatening neurological emergency associated with high morbidity and mortality. Affected patients often require prolonged intensive care and can suffer multiple complications. Surgical intervention to control RSE is rarely used but can obviate the risks of prolonged seizures and intensive care treatment. Authors of the present study analyzed their experience with the surgical management of patients suffering from RSE.
The Epilepsy Surgery Database at Miami Children's Hospital was reviewed for patients who had undergone surgery for RSE. Clinical presentation, electrophysiological profile, radiological data, surgical details, and postoperative course were evaluated.
Between 1990 and 2012, 15 patients underwent surgery for uncontrolled seizures despite high-dose medical suppressive therapy. The mean preoperative duration of status epilepticus was 8 weeks. Ictal SPECT and FDG-PET imaging in conjunction with intraoperative electrophysiological studies helped to outline the extent of resection. Surgical intervention controlled seizures in all patients and facilitated the transition out of intensive care. Adverse events related to a prolonged intensive care unit stay included sepsis and respiratory complications. Four patients had worsened neurological function, developing hemiparesis and dysphasia. There was no operative mortality.
Surgical intervention can successfully control refractory partial status epilepticus, prevent associated morbidity, and decrease intensive care unit stay. Ictal SPECT and PET are valuable in guiding resection.
Alexander G. Weil, Andrew L. Middleton, Toba N. Niazi, John Ragheb and Sanjiv Bhatia
Operations on tumors of the posteromedial temporal (PMT) region, that is, on those arising from the posterior parahippocampal, fusiform, and lingual gyri, are challenging to perform because of the deep-seated location of these tumors between critical cisternal neurovascular structures and the adjacent temporal and occipital cortexes. Traditional surgical approaches require temporal or occipital transgression, retraction, or venous sacrifice. These approaches may result in unintended complications that should be avoided. To avoid these complications, the supracerebellar-transtentorial (SCTT) approach to this region has been used as an effective alternative treatment in adult patients. The SCTT approach uses a sitting position that offers a direct route to the posterior fusiform and lingual gyri of the temporal lobe. The authors report the feasibility, safety, and efficacy of this approach, using a modified lateral park-bench position in a small cohort of pediatric patients.
The authors carried out a retrospective case review of 5 consecutive patients undergoing a paramedian SCTT approach between 2009 and 2014 at the authors' institution.
The SCTT approach in the park-bench position was used in 3 boys and 2 girls with a mean age of 7.8 years (range 13 months to 16 years). All patients presented with a seizure disorder related to a tumor in a PMT region involving the parahippocampal and fusiform gyri of the left (n = 3) or right (n = 2) temporal lobe. No procedure-related complications were observed. Gross-total resection and control of seizures were achieved in all cases. Tumor classes and types included 1 Grade II astrocytoma, 1 pleomorphic xanthoastrocytoma, 1 ganglioglioma, and 2 glioneural tumors. None of the tumors had recurred by the mean follow-up of 22 months (range 1–48 months). Outcomes of epileptic seizures were excellent, with seizure symptoms in all 5 patients scoring in Engel Class IA.
The SCTT approach represents a viable option when resecting tumors in this region, providing a reasonable working corridor and low morbidity. The authors' experience in a cohort of pediatric patients demonstrates that complete resection of the lesions in this location is feasible and is safe when involving an approach that involves using a park-bench lateral positioning.
Guillermo De Angulo, Sushmita Nair, Vivian Lee, Ziad Khatib, John Ragheb and David I. Sandberg
Solitary eosinophilic granuloma (EG) of the calvaria is most commonly treated with surgical excision. The authors hypothesize that many solitary EGs will resolve without intervention, and observation may be a reasonable option. This study was undertaken to investigate that hypothesis.
The authors reviewed their institutional records and identified 14 cases of solitary calvarial EG. In 6 cases the patients underwent resection based on family and/or neurosurgeon preferences. A strategy of nonoperative management (purposeful observation) was chosen for the other 8 cases. The authors report the clinical course and imaging results in these 8 cases.
One of the 8 patients underwent surgery 2 months after presentation because of slight enlargement of the lesion and increasing pain. After a median follow-up period of 1 year (range 6–19 months), none of the other patients had required surgery. Five of these 7 patients had pain at presentation. Pain resolved completely in all 5. The remaining 2 remained asymptomatic. Complete resolution of pain was reported in the 5 patients who had pain at presentation. There was complete clinical resolution of the palpable soft-tissue lesion in all 7 cases. Complete radiographic resolution of the lesion was observed in 5 cases and near-complete resolution in the remaining 2.
Observation is a safe and reasonable approach in the management of solitary calvarial EG and may prevent unnecessary surgical interventions.
Aizik Wolf, Lion Levi, Stuart Mirvis, John Ragheb, Stephen Huhn, Daniele Rigamonti and Walker L. Robinson
✓ Fifty-two patients with acute traumatic bilateral locked facets were treated at one trauma center during a 3½-year period (July, 1987, to December, 1990). The patients presented with complete motor quadriplegia (34 cases), incomplete myelopathy (13 cases), or intact long-tract function (five cases). The injuries occurred at C2–3 (one patient, with intact function), C4–5 (12 patients), C5–6 (16 patients), C6–7 (19 patients), and C7—T1 (four patients).
Immediate traction (with increasing weight and serial x-ray studies) and/or induction of general anesthesia and muscle relaxation reduced the dislocation in 40 patients, but 12 needed prompt operative reduction as their injuries failed to reduce within 4 hours. Stabilization was indicated for all patients, but three did not undergo surgery: two elderly patients with complete injuries (one refused surgery and one died), and one patient with multiple injuries (fusion was achieved by halo-vest immobilization for 3 months). Of the 49 patients treated operatively, 23 (44.2%) underwent surgery on the day of injury and 26 on a delayed basis (mean 8.7 days postinjury). Surgical treatment included fusion of the posterior facet to a spinous process (44 cases), an anterior Caspar plate technique (three cases), and both procedures (two cases). Of these 49 patients, three (6.1%) with complete injuries died due to an adult respiratory distress syndrome.
Improvement of cord function, judged by functional grade change, was observed at discharge in 15 patients (31.9%) and in 15 (71.4%) of the 21 patients with a 1-year follow-up period. Of the 34 patients with complete myelopathy on admission, three are ambulatory after 1 year, and 13 others have gained function in at least one nerve root. It is concluded that prompt reduction (nonoperative or surgical) and internal stabilization facilitate recovery even in neurologically compromised patients, and that early operative intervention is a wiser option than conservative management. This report also documents a higher incidence of this injury without deficit (five of the 52 cases) than reported in other series.