S tatuS epilepticus (SE), defined as seizures lasting 30 minutes or longer, is a common life-thre... more S tatuS epilepticus (SE), defined as seizures lasting 30 minutes or longer, is a common life-threatening medical emergency in adults and children and is associated with high morbidity and mortality. More than 150,000 new cases occur annually in the US, which result in 22,000-42,000 deaths. 7,8,15 Refractory status epilepticus (RSE) is associated with 12.9% neurological morbidity after the first episode of SE and 29.2% morbidity after recurrent SE. 13,25 The management of SE is typically staged with medical treatment gradually escalated to bring seizures under control. 29 Most patients with the disorder respond to firstline treatment with phenytoin, barbiturates, or levetiracetam. High-dose benzodiazepines, such as midazolam, pentobarbital, or propofol, are typically introduced when seizures are uncontrolled by the first-line medications. 30 Intravenous propofol, lidocaine, valproate, ketamine, and even electroconvulsive therapy have been used to suppress SE; 14,28,32 however, a small number of cases are refractory to all medical treatment, or respond only briefly and continue in SE for prolonged periods of time, sometimes for weeks or months. 17 By this point in the course of the disease, patients are often critically ill, requiring intensive medical treatment and cardiorespiratory support. Prolonged coma and respiratory problems account for more than 50% of deaths. 13 Refractory status epilepticus, defined as SE unresponsive to benzodiazepines and intravenous anticonvulsants, including phenytoin or levetiracetam, occurs in 30% of patients with SE. 17 Those affected typically have a focal electrophysiological onset, yet neurosurgical intervention is rarely performed. Studies on the surgical treatment of RSE are therefore limited to single case reports or small series involving cortical resection, callosal sectioning, multiple subpial transections (MSTs), hemispherectomy, and vagus nerve stimulation. 1,3,4,9-12,16,17,19,20,24 There is no consensus on how or when to surgically manage this medical emergency.
To highlight the rising number and nature of pediatric head injuries secondary to falling televis... more To highlight the rising number and nature of pediatric head injuries secondary to falling television sets and correlate this with the increasing number of home TVs sold per year. National statistics for the past 10 years for the number of head traumas from falling television sets in children less than the age of 18 were obtained from the US Consumer Product Safety Commission. Statistics for the number of TV sales were obtained from the Consumer Electronic US Sales Report for the same time period. A retrospective chart review overlapping the same period of time was performed at our local institution. National statistics show a steady rise in the number of pediatric head injuries, from 31 in 1992 to 117 in 2001. The number of TVs sold by year increased from 20,384,000 to 23,776,000 in a 10-year period from 1990 to 2000. Six of the 7 patients in our series (average age 22 months, range 18-36 months) suffered a skull fracture; 2 of those 6 were basilar skull fractures. The average length of hospital stay was 5 days (range 1-13 days). The number of pediatric head injuries from falling televisions has been steadily increasing. A similar, yet more modest rise in the number of televisions manufactured and sold per year has also been noted. From our series, the most common type of pediatric head injury sustained from falling television sets was skull fracture, seen mostly in children less than 2 years of age.
The results of 32 patients who underwent transcranial monobloc frontofacial advancement by the se... more The results of 32 patients who underwent transcranial monobloc frontofacial advancement by the senior author (Wolfe) are examined. Fifteen of the patients were Crouzon's and 17 were Apert's, 14 of whom also underwent simultaneous facial bipartition. The ages ranged from 7 months to 14 years. Although a transcranial facial advancement carries with it substantial risks, with careful consideration of airway control, the anterior cranial base dura, and the retrofrontal dead space, the procedure is recommended for carefully selected patients. The indications and contraindications for the procedure in various age groups are given.
OBJECT Pediatric brain tumors may be associated with medically intractable epilepsy for which sur... more OBJECT Pediatric brain tumors may be associated with medically intractable epilepsy for which surgery is indicated. The authors sought to evaluate the efficacy of epilepsy surgery for seizure control in pediatric patients with brain tumors. METHODS The authors performed a retrospective review of consecutive patients undergoing resective epilepsy surgery related to pediatric brain tumors at Miami Children's Hospital between June 1986 and June 2014. Time-to-event analysis for seizure recurrence was performed; an "event" was defined as any seizures that occurred following resective epilepsy surgery, not including seizures and auras in the 1st postoperative week. The authors analyzed several preoperative variables to determine their suitability to predict seizure recurrence following surgery. RESULTS Eighty-four patients (47 males) with a mean age (± standard deviation) of 8.7 ± 5.5 years (range 0.5-21.6 years) were included. The study included 39 (46%) patients with gliom...
Fibrous dysplasia is an abnormal growth of bone that can lead to severe facial disfigurement. A d... more Fibrous dysplasia is an abnormal growth of bone that can lead to severe facial disfigurement. A dreaded outcome is compression of the optic nerve, leading to blindness. Controversy has surrounded the role of optic nerve unroofing for circumferential involvement of the optic canal. At present, many neurosurgeons unroof the nerve therapeutically in the setting of optic nerve dysfunction. Prophylactic unroofing (i.e., unroofing the nerve prior to the development of visual symptoms) has been previously proposed, although reported outcomes have been mixed. The authors present their long-term results of patients who have undergone optic nerve unroofing. From 1975 to 2012, patients with fibrous dysplasia were investigated. Their age, demographics, operative procedure, optic nerve involvement (radiologically and clinically), and long-term outcomes and complications were recorded. Over 37 years, the senior author (S.A.W.) operated on 32 patients with fibrous dysplasia. Average follow-up was 5 years. Nine patients underwent optic nerve unroofing. Two patients had bilateral unroofing. Three patients who underwent therapeutic optic nerve unroofing ultimately went on to complete vision loss. The remaining seven patients who underwent prophylactic unroofing had no immediate postoperative visual compromise. Therapeutic optic nerve unroofing is advocated in fibrous dysplasia patients with continuous deterioration of vision. However, the authors believe prophylactic unroofing is safe, and it should be performed not necessarily as a primary surgical procedure, but as a procedure along with excision of fibrous dysplasia in the anterior skull base during the same operation performed for orbitocranial deformity. Therapeutic, IV.
Desmoplastic fibromas are rare bone tumors that have been reported in the adult skull but rarely ... more Desmoplastic fibromas are rare bone tumors that have been reported in the adult skull but rarely in that of children. Although desmoplastic fibromas of the pediatric skull are uncommon, their similarity to benign skull lesions and their locally aggressive nature make them an important part of the differential diagnosis. Local recurrence is common after curettage alone but complete resection appears to be curative. Close follow up of incompletely resected lesions is essential. The authors detail three cases of pediatric desmoplastic fibromas of the skull and discuss diagnosis and treatment.
This article reviews the scientific evidence behind the diagnostic tools available for the approp... more This article reviews the scientific evidence behind the diagnostic tools available for the appropriate workup and management of patients with occult spinal dysraphism (OSD). The diagnostic tools include the use of detailed history and physical examination, plain films, ultrasound, MR imaging, and neurophysiologic tests. In addition, the article discusses the epidemiology of the most common causes of OSD in children, which will allow physicians caring for children to develop a pretest probability of disease and make a more educated decision as to when additional diagnostic testing is required.
Ten pediatric medulloblastoma patients were analyzed for DNA content, cell cycle, expression of d... more Ten pediatric medulloblastoma patients were analyzed for DNA content, cell cycle, expression of drug resistance, apoptosis, cell proliferation, and N-myc genes to determine their prognostic significance. Medulloblastoma patients with progressive disease had fourth ventricle foraminal extension and larger tumors in the imaging studies. Patients with aneuploid tumors responded well to treatment regimens as compared with those with diploid tumors. Cell cycle analysis showed that the patients with progressive disease had a high S-phase fraction in the tumor cell population as compared with patients with favorable response to treatment. The correlation coefficients between Bcl-2 and MRP, Bcl-2 and Bax, p53 and p21, as well as Ki67 and PCNA were positive and significant, indicating their possible coregulated expression. The relationship between these markers indicates their relative and cumulative effect on cellular drug resistance, apoptosis, and/or cell proliferation in pediatric medulloblastomas.
To prospectively evaluate effect of functional magnetic resonance (MR) imaging on diagnostic work... more To prospectively evaluate effect of functional magnetic resonance (MR) imaging on diagnostic work-up and treatment planning in patients with seizure disorders who are candidates for surgical treatment. Institutional review board approval was obtained; informed consent was obtained either from the patient or the parent or guardian in all patients. This study was conducted with Health Insurance Portability and Accountability Act compliance. Sixty consecutively enrolled patients (33 males, 27 females; mean age, 15.8 years +/- 8.7 [standard deviation]; range, 6.8-44.2 years) were prospectively examined. Forty-five (75%) patients were right handed, nine (15%) were left handed, and six (10%) had indeterminate hand dominance. Prospective questionnaires were used to evaluate diagnostic work-up, counseling, and treatment plans of the seizure team before and after functional MR imaging. Confidence level scales were used to determine effect of functional MR imaging on diagnostic and therapeutic thinking. Paired t test and 95% confidence interval analyses were performed. In 53 patients, language mapping was performed; in 33, motor mapping; and in seven, visual mapping. The study revealed change in anatomic location or lateralization of language-receptive (Wernicke) (28% of patients) and language-expressive (Broca) (21% of patients) areas. Statistically significant increases were found in confidence levels after functional MR imaging in regard to motor and visual cortical function evaluation. In 35 (58%) of 60 patients, the seizure team thought that functional MR imaging results altered patient and family counseling. In 38 (63%) of 60 patients, functional MR imaging results helped to avoid further studies, including Wada test. In 31 (52%) and 25 (42%) of 60 patients, intraoperative mapping and surgical plans, respectively, were altered because of functional MR imaging results. In five (8%) patients, two-stage surgery with extra-operative direct electrical stimulation mapping was averted, and resection was accomplished in one stage. In four (7%) patients, extent of surgical resection was altered because eloquent areas were identified close to seizure focus. Functional MR imaging results influenced diagnostic and therapeutic decision making of the seizure team; results indicated language dominance changed, confidence level in identification of critical brain function areas increased, patient and family counseling were altered, and intraoperative mapping and surgical approach were altered.
This case report describes a 15-month-old female who developed diffuse cerebral vasospasm after r... more This case report describes a 15-month-old female who developed diffuse cerebral vasospasm after resection of a cerebellopontine angle primitive neuroectodermal tumor. The patient developed an acute dense left hemiparesis 16 days postoperatively with partial right ptosis. Initial magnetic resonance imaging and diffusion study were unremarkable, though a magnetic resonance angiography 1 day later demonstrated severe intracranial vasospasm of both carotid and vertebral arteries. The vasospasm was confirmed with cerebral angiography. The patient progressed to bihemispheric infarcts with laminar necrosis despite combination therapy with anticoagulation, pharmacological hypertension, hypervolemia, and nimodipine. The clinical course, radiographic, and pathological findings are presented.
To highlight the rising number and nature of pediatric head injuries secondary to falling televis... more To highlight the rising number and nature of pediatric head injuries secondary to falling television sets and correlate this with the increasing number of home TVs sold per year. National statistics for the past 10 years for the number of head traumas from falling television sets in children less than the age of 18 were obtained from the US Consumer Product Safety Commission. Statistics for the number of TV sales were obtained from the Consumer Electronic US Sales Report for the same time period. A retrospective chart review overlapping the same period of time was performed at our local institution. National statistics show a steady rise in the number of pediatric head injuries, from 31 in 1992 to 117 in 2001. The number of TVs sold by year increased from 20,384,000 to 23,776,000 in a 10-year period from 1990 to 2000. Six of the 7 patients in our series (average age 22 months, range 18-36 months) suffered a skull fracture; 2 of those 6 were basilar skull fractures. The average length of hospital stay was 5 days (range 1-13 days). The number of pediatric head injuries from falling televisions has been steadily increasing. A similar, yet more modest rise in the number of televisions manufactured and sold per year has also been noted. From our series, the most common type of pediatric head injury sustained from falling television sets was skull fracture, seen mostly in children less than 2 years of age.
A cerebrospinal fluid (CSF) shunt is the primary treatment for most etiologies of hydrocephalus i... more A cerebrospinal fluid (CSF) shunt is the primary treatment for most etiologies of hydrocephalus in the pediatric population. Malfunction of the shunt may present with unique symptoms and signs. This retrospective review investigates the presenting signs and symptoms of pediatric patients with shunt malfunction. Clinical One-hundred-and-thirty CSF diversion procedures were performed at two affiliated pediatric hospitals over a 2-year period. Seventy consecutive cases of CSF shunt revision were reviewed. These 70 operations were performed on 65 patients. Their medical records and radiographic studies were reviewed, and supplemented with a telephone interview to obtain a minimum of 3 months follow-up. The 65 patients' age ranged from 3 months to 16 years. The original etiology of the hydrocephalus was Chiari II malformation in 17, idiopathic in 15 and intraventricular hemorrhage in 10, neoplasm in 8 patients and meningitis in 5 patients. The most frequent presenting symptoms were headache (39 admissions), nausea/vomiting (28) and drowsiness (21). Seven Chiari patients (41%) presented with neck pain, 2 (12%) presented with lower cranial nerve palsy, and 2 (12%) presented with symptomatic syrinx, complaints not reported by non-Chiari patients (p < 0.01, chi2 analysis). Four myelodysplastic patients presented with a new-onset or recurrent seizure episode, which was significantly more frequent than in nonmyelodysplastic patients (p < 0.05, chi2 analysis). On examination, increased head circumference was noted in 17 patients. Parinaud's syndrome was noted more prominently in patients with a history of intracranial neoplasm (4 of 8 cases) than in patients with nonneoplastic diseases (2 of 62 cases; p < 0.05, chi2 analysis). Other interesting presenting signs were pseudocyst (2), syringomyelia (2), hemiparesis (2) and Parkinson-like rigidity (2). Pediatric shunt malfunction generally presents with headache, nausea/vomiting, altered mental status, increased head circumference and bulging fontanelle. Other less frequent but unique presenting signs and symptoms, such as neck pain, syringomyelia and lower cranial nerve palsy in the myelodysplastic population, and Parinaud's syndrome in patients with a history of intracranial neoplasm are frequently associated with shunt malfunction and should prompt a radiographic workup.
Lymphangiomatosis of the bone is rare. The axial as well as appendicular skeleton may be affected... more Lymphangiomatosis of the bone is rare. The axial as well as appendicular skeleton may be affected. Neurosurgical consultation may be called for several reasons: (1) lesions involving the calvarium and/or spine; (2) nondiagnostic biopsies from more accessible and less morbid locations, and (3) persistent CSF leak and/or recurrent meningitis. Thus, it is important for the neurosurgeon to be familiar with this disease entity and consider it in the differential diagnoses of multifocal lytic lesions of the axial skeleton. We present a case report of a 4-year-old girl with lymphangiomatosis of the skull base and upper cervical spine with concurrent Chiari I malformation and briefly review the literature.
Pleomorphic xanthoastrocytomas (PXAs) are rare primary central nervous system neoplasms of childh... more Pleomorphic xanthoastrocytomas (PXAs) are rare primary central nervous system neoplasms of childhood and early adulthood. While PXAs are intimately associated with the leptomeninges, there has never been a description of an extradural-extracranial manifestation of PXA until now. We describe a case of PXA where the patient presented with a calvarial lesion. We also review the differential diagnoses of lytic skull lesions in addition to PXA.
The seizure outcome and neurological outcome in children who undergo reoperation for failed epile... more The seizure outcome and neurological outcome in children who undergo reoperation for failed epilepsy surgery have not been well documented. This retrospective study evaluated 20 children who underwent a second resective surgery for recurrent seizures. Four categories of patients were identified: (1) extension of the initial resection was performed in 8 patients; (2) 5 patients underwent lobectomy or corticectomy in a region remote from the original surgical site; (3) multilobar resection which may have included further resection of the initial procedure was accomplished in 4 patients; (4) hemispherectomy was performed in 3 patients. Patients with reoperation in the same lobe as the first procedure (group 1) had a 62% seizure-free rate, while 44% of patients in groups 2 and 3 were free from seizures at follow-up evaluation. Patients undergoing hemispherectomy had a 67% seizure-free rate. Significant unexpected neurological deficits occurred in 3 patients who underwent multilobar resection at reoperation. Complications included motor and language deficits. Reoperation for intractable partial epilepsy is beneficial in selected children. Patients who require multilobar resections may have higher risk of postoperative neurological deficit than those patients with reoperation in one lobe. These factors may be useful in counseling parents of children considering reoperation for recurrent epilepsy.
This study was undertaken to assess the utility of digital camera-derived intraoperative images i... more This study was undertaken to assess the utility of digital camera-derived intraoperative images in the planning of neurosurgery for children with epilepsy. A hand-held digital camera was used to capture the exposed surgical field at the time of craniotomy for 11 children with medically intractable seizure disorders. Intraoperative somatosensory evoked potential recordings of phase reversals and direct cortical stimulation were used to map areas of eloquent brain tissue. Digital camera images were obtained to mark regions of functional brain tissue with respect to cortical surface landmarks and subdural grid placement. The digital camera images were then immediately downloaded, in the operating room, to a laptop computer, which was placed next to the electroencephalographic recording device. Using computer software, the epileptologist highlighted the primary and secondary zones of epileptogenesis, as well as the functional brain areas identified during the monitoring period, on the digital camera images on the computer screen. A neurosurgical map was thus created to aid the neurosurgeon and the epileptologist with the proposed cortical resections and multiple subpial transections. With the images obtained using the digital camera, the epilepsy team was able to observe the contacts of the grid electrodes with the brain during the procedure. Color printouts of the images served as references during the period of invasive monitoring. Zones of primary and secondary epileptogenesis, as well as areas of functional brain tissue, were identified and plotted on the digital camera images. Other benefits of the digital camera-derived images included the ability to accurately reposition the grids or letters marking eloquent brain tissue if they were inadvertently shifted during the procedure, the ease with which the images could be obtained and manipulated, the ability to assess postresection epileptiform activity of the surrounding brain tissue with images obtained while an electrocorticographic array was in place, the ability to provide the entire epilepsy team with updated information on the neurosurgical field while minimizing movement in the operating room, and facilitation, with neurosurgical maps, of discussions with the patients and their families concerning proposed cortical resections. Digital camera images have become essential components for the planning of cortical resections for children with intractable epilepsy at our institution. We envision widespread application of this technology to other neurosurgical fields.
ABSTRACT OBJECTIVE Invasive urological procedures, commonly performed on patients with myelodyspl... more ABSTRACT OBJECTIVE Invasive urological procedures, commonly performed on patients with myelodysplasia, may contribute to the occurrence of cerebrospinal fluid shunt complications. Shunt complications that occurred after urological procedures in children with myelodysplasia were studied. METHODS Shunt complications occurring after urological procedures were examined in 29 patients. Differences between patients with or without complications were studied. Complications were analyzed according to the location of abnormality, the treatment, and the timing after shunt and urological surgery. RESULTS The 1-year incidence of shunt complications after a urological procedure had been performed was 31% (overall incidence, 41.4%). Shunt complications were observed only after intraperitoneal urological procedures. Most complications were distal, occurring more than 1 year after the preceding shunt surgery. The patients in the shunt complications group had significantly more intraperitoneal urological procedures (3.2 versus 0.8, P = 0.004) and previous shunt revisions (2.0 versus 0.9, P = 0.015) than had the group without complications. As compared with the group of patients with extraperitoneal complications, the intraperitoneal group experienced significantly more infections (4 of 9 versus 0 of 10, P = 0.014) requiring more complex treatment, and their complications occurred significantly earlier in the follow-up period after the urological procedure had been performed (7.2 versus 27.3 mo, P = 0.006). The patients in the group with extraperitoneal complications experienced significantly more mechanical shunt malfunctions than did the intraperitoneal group (9 of 10 versus 4 of 9, P = 0.016), which required simple shunt revisions. CONCLUSION Patients with spina bifida and shunted hydrocephalus may have an increased risk of developing intraperitoneal shunt complications after intraperitoneal urological procedures have been performed. These intraperitoneal shunt complications usually occur a few months after urological surgery and require complex treatment.
S tatuS epilepticus (SE), defined as seizures lasting 30 minutes or longer, is a common life-thre... more S tatuS epilepticus (SE), defined as seizures lasting 30 minutes or longer, is a common life-threatening medical emergency in adults and children and is associated with high morbidity and mortality. More than 150,000 new cases occur annually in the US, which result in 22,000-42,000 deaths. 7,8,15 Refractory status epilepticus (RSE) is associated with 12.9% neurological morbidity after the first episode of SE and 29.2% morbidity after recurrent SE. 13,25 The management of SE is typically staged with medical treatment gradually escalated to bring seizures under control. 29 Most patients with the disorder respond to firstline treatment with phenytoin, barbiturates, or levetiracetam. High-dose benzodiazepines, such as midazolam, pentobarbital, or propofol, are typically introduced when seizures are uncontrolled by the first-line medications. 30 Intravenous propofol, lidocaine, valproate, ketamine, and even electroconvulsive therapy have been used to suppress SE; 14,28,32 however, a small number of cases are refractory to all medical treatment, or respond only briefly and continue in SE for prolonged periods of time, sometimes for weeks or months. 17 By this point in the course of the disease, patients are often critically ill, requiring intensive medical treatment and cardiorespiratory support. Prolonged coma and respiratory problems account for more than 50% of deaths. 13 Refractory status epilepticus, defined as SE unresponsive to benzodiazepines and intravenous anticonvulsants, including phenytoin or levetiracetam, occurs in 30% of patients with SE. 17 Those affected typically have a focal electrophysiological onset, yet neurosurgical intervention is rarely performed. Studies on the surgical treatment of RSE are therefore limited to single case reports or small series involving cortical resection, callosal sectioning, multiple subpial transections (MSTs), hemispherectomy, and vagus nerve stimulation. 1,3,4,9-12,16,17,19,20,24 There is no consensus on how or when to surgically manage this medical emergency.
To highlight the rising number and nature of pediatric head injuries secondary to falling televis... more To highlight the rising number and nature of pediatric head injuries secondary to falling television sets and correlate this with the increasing number of home TVs sold per year. National statistics for the past 10 years for the number of head traumas from falling television sets in children less than the age of 18 were obtained from the US Consumer Product Safety Commission. Statistics for the number of TV sales were obtained from the Consumer Electronic US Sales Report for the same time period. A retrospective chart review overlapping the same period of time was performed at our local institution. National statistics show a steady rise in the number of pediatric head injuries, from 31 in 1992 to 117 in 2001. The number of TVs sold by year increased from 20,384,000 to 23,776,000 in a 10-year period from 1990 to 2000. Six of the 7 patients in our series (average age 22 months, range 18-36 months) suffered a skull fracture; 2 of those 6 were basilar skull fractures. The average length of hospital stay was 5 days (range 1-13 days). The number of pediatric head injuries from falling televisions has been steadily increasing. A similar, yet more modest rise in the number of televisions manufactured and sold per year has also been noted. From our series, the most common type of pediatric head injury sustained from falling television sets was skull fracture, seen mostly in children less than 2 years of age.
The results of 32 patients who underwent transcranial monobloc frontofacial advancement by the se... more The results of 32 patients who underwent transcranial monobloc frontofacial advancement by the senior author (Wolfe) are examined. Fifteen of the patients were Crouzon's and 17 were Apert's, 14 of whom also underwent simultaneous facial bipartition. The ages ranged from 7 months to 14 years. Although a transcranial facial advancement carries with it substantial risks, with careful consideration of airway control, the anterior cranial base dura, and the retrofrontal dead space, the procedure is recommended for carefully selected patients. The indications and contraindications for the procedure in various age groups are given.
OBJECT Pediatric brain tumors may be associated with medically intractable epilepsy for which sur... more OBJECT Pediatric brain tumors may be associated with medically intractable epilepsy for which surgery is indicated. The authors sought to evaluate the efficacy of epilepsy surgery for seizure control in pediatric patients with brain tumors. METHODS The authors performed a retrospective review of consecutive patients undergoing resective epilepsy surgery related to pediatric brain tumors at Miami Children's Hospital between June 1986 and June 2014. Time-to-event analysis for seizure recurrence was performed; an "event" was defined as any seizures that occurred following resective epilepsy surgery, not including seizures and auras in the 1st postoperative week. The authors analyzed several preoperative variables to determine their suitability to predict seizure recurrence following surgery. RESULTS Eighty-four patients (47 males) with a mean age (± standard deviation) of 8.7 ± 5.5 years (range 0.5-21.6 years) were included. The study included 39 (46%) patients with gliom...
Fibrous dysplasia is an abnormal growth of bone that can lead to severe facial disfigurement. A d... more Fibrous dysplasia is an abnormal growth of bone that can lead to severe facial disfigurement. A dreaded outcome is compression of the optic nerve, leading to blindness. Controversy has surrounded the role of optic nerve unroofing for circumferential involvement of the optic canal. At present, many neurosurgeons unroof the nerve therapeutically in the setting of optic nerve dysfunction. Prophylactic unroofing (i.e., unroofing the nerve prior to the development of visual symptoms) has been previously proposed, although reported outcomes have been mixed. The authors present their long-term results of patients who have undergone optic nerve unroofing. From 1975 to 2012, patients with fibrous dysplasia were investigated. Their age, demographics, operative procedure, optic nerve involvement (radiologically and clinically), and long-term outcomes and complications were recorded. Over 37 years, the senior author (S.A.W.) operated on 32 patients with fibrous dysplasia. Average follow-up was 5 years. Nine patients underwent optic nerve unroofing. Two patients had bilateral unroofing. Three patients who underwent therapeutic optic nerve unroofing ultimately went on to complete vision loss. The remaining seven patients who underwent prophylactic unroofing had no immediate postoperative visual compromise. Therapeutic optic nerve unroofing is advocated in fibrous dysplasia patients with continuous deterioration of vision. However, the authors believe prophylactic unroofing is safe, and it should be performed not necessarily as a primary surgical procedure, but as a procedure along with excision of fibrous dysplasia in the anterior skull base during the same operation performed for orbitocranial deformity. Therapeutic, IV.
Desmoplastic fibromas are rare bone tumors that have been reported in the adult skull but rarely ... more Desmoplastic fibromas are rare bone tumors that have been reported in the adult skull but rarely in that of children. Although desmoplastic fibromas of the pediatric skull are uncommon, their similarity to benign skull lesions and their locally aggressive nature make them an important part of the differential diagnosis. Local recurrence is common after curettage alone but complete resection appears to be curative. Close follow up of incompletely resected lesions is essential. The authors detail three cases of pediatric desmoplastic fibromas of the skull and discuss diagnosis and treatment.
This article reviews the scientific evidence behind the diagnostic tools available for the approp... more This article reviews the scientific evidence behind the diagnostic tools available for the appropriate workup and management of patients with occult spinal dysraphism (OSD). The diagnostic tools include the use of detailed history and physical examination, plain films, ultrasound, MR imaging, and neurophysiologic tests. In addition, the article discusses the epidemiology of the most common causes of OSD in children, which will allow physicians caring for children to develop a pretest probability of disease and make a more educated decision as to when additional diagnostic testing is required.
Ten pediatric medulloblastoma patients were analyzed for DNA content, cell cycle, expression of d... more Ten pediatric medulloblastoma patients were analyzed for DNA content, cell cycle, expression of drug resistance, apoptosis, cell proliferation, and N-myc genes to determine their prognostic significance. Medulloblastoma patients with progressive disease had fourth ventricle foraminal extension and larger tumors in the imaging studies. Patients with aneuploid tumors responded well to treatment regimens as compared with those with diploid tumors. Cell cycle analysis showed that the patients with progressive disease had a high S-phase fraction in the tumor cell population as compared with patients with favorable response to treatment. The correlation coefficients between Bcl-2 and MRP, Bcl-2 and Bax, p53 and p21, as well as Ki67 and PCNA were positive and significant, indicating their possible coregulated expression. The relationship between these markers indicates their relative and cumulative effect on cellular drug resistance, apoptosis, and/or cell proliferation in pediatric medulloblastomas.
To prospectively evaluate effect of functional magnetic resonance (MR) imaging on diagnostic work... more To prospectively evaluate effect of functional magnetic resonance (MR) imaging on diagnostic work-up and treatment planning in patients with seizure disorders who are candidates for surgical treatment. Institutional review board approval was obtained; informed consent was obtained either from the patient or the parent or guardian in all patients. This study was conducted with Health Insurance Portability and Accountability Act compliance. Sixty consecutively enrolled patients (33 males, 27 females; mean age, 15.8 years +/- 8.7 [standard deviation]; range, 6.8-44.2 years) were prospectively examined. Forty-five (75%) patients were right handed, nine (15%) were left handed, and six (10%) had indeterminate hand dominance. Prospective questionnaires were used to evaluate diagnostic work-up, counseling, and treatment plans of the seizure team before and after functional MR imaging. Confidence level scales were used to determine effect of functional MR imaging on diagnostic and therapeutic thinking. Paired t test and 95% confidence interval analyses were performed. In 53 patients, language mapping was performed; in 33, motor mapping; and in seven, visual mapping. The study revealed change in anatomic location or lateralization of language-receptive (Wernicke) (28% of patients) and language-expressive (Broca) (21% of patients) areas. Statistically significant increases were found in confidence levels after functional MR imaging in regard to motor and visual cortical function evaluation. In 35 (58%) of 60 patients, the seizure team thought that functional MR imaging results altered patient and family counseling. In 38 (63%) of 60 patients, functional MR imaging results helped to avoid further studies, including Wada test. In 31 (52%) and 25 (42%) of 60 patients, intraoperative mapping and surgical plans, respectively, were altered because of functional MR imaging results. In five (8%) patients, two-stage surgery with extra-operative direct electrical stimulation mapping was averted, and resection was accomplished in one stage. In four (7%) patients, extent of surgical resection was altered because eloquent areas were identified close to seizure focus. Functional MR imaging results influenced diagnostic and therapeutic decision making of the seizure team; results indicated language dominance changed, confidence level in identification of critical brain function areas increased, patient and family counseling were altered, and intraoperative mapping and surgical approach were altered.
This case report describes a 15-month-old female who developed diffuse cerebral vasospasm after r... more This case report describes a 15-month-old female who developed diffuse cerebral vasospasm after resection of a cerebellopontine angle primitive neuroectodermal tumor. The patient developed an acute dense left hemiparesis 16 days postoperatively with partial right ptosis. Initial magnetic resonance imaging and diffusion study were unremarkable, though a magnetic resonance angiography 1 day later demonstrated severe intracranial vasospasm of both carotid and vertebral arteries. The vasospasm was confirmed with cerebral angiography. The patient progressed to bihemispheric infarcts with laminar necrosis despite combination therapy with anticoagulation, pharmacological hypertension, hypervolemia, and nimodipine. The clinical course, radiographic, and pathological findings are presented.
To highlight the rising number and nature of pediatric head injuries secondary to falling televis... more To highlight the rising number and nature of pediatric head injuries secondary to falling television sets and correlate this with the increasing number of home TVs sold per year. National statistics for the past 10 years for the number of head traumas from falling television sets in children less than the age of 18 were obtained from the US Consumer Product Safety Commission. Statistics for the number of TV sales were obtained from the Consumer Electronic US Sales Report for the same time period. A retrospective chart review overlapping the same period of time was performed at our local institution. National statistics show a steady rise in the number of pediatric head injuries, from 31 in 1992 to 117 in 2001. The number of TVs sold by year increased from 20,384,000 to 23,776,000 in a 10-year period from 1990 to 2000. Six of the 7 patients in our series (average age 22 months, range 18-36 months) suffered a skull fracture; 2 of those 6 were basilar skull fractures. The average length of hospital stay was 5 days (range 1-13 days). The number of pediatric head injuries from falling televisions has been steadily increasing. A similar, yet more modest rise in the number of televisions manufactured and sold per year has also been noted. From our series, the most common type of pediatric head injury sustained from falling television sets was skull fracture, seen mostly in children less than 2 years of age.
A cerebrospinal fluid (CSF) shunt is the primary treatment for most etiologies of hydrocephalus i... more A cerebrospinal fluid (CSF) shunt is the primary treatment for most etiologies of hydrocephalus in the pediatric population. Malfunction of the shunt may present with unique symptoms and signs. This retrospective review investigates the presenting signs and symptoms of pediatric patients with shunt malfunction. Clinical One-hundred-and-thirty CSF diversion procedures were performed at two affiliated pediatric hospitals over a 2-year period. Seventy consecutive cases of CSF shunt revision were reviewed. These 70 operations were performed on 65 patients. Their medical records and radiographic studies were reviewed, and supplemented with a telephone interview to obtain a minimum of 3 months follow-up. The 65 patients' age ranged from 3 months to 16 years. The original etiology of the hydrocephalus was Chiari II malformation in 17, idiopathic in 15 and intraventricular hemorrhage in 10, neoplasm in 8 patients and meningitis in 5 patients. The most frequent presenting symptoms were headache (39 admissions), nausea/vomiting (28) and drowsiness (21). Seven Chiari patients (41%) presented with neck pain, 2 (12%) presented with lower cranial nerve palsy, and 2 (12%) presented with symptomatic syrinx, complaints not reported by non-Chiari patients (p < 0.01, chi2 analysis). Four myelodysplastic patients presented with a new-onset or recurrent seizure episode, which was significantly more frequent than in nonmyelodysplastic patients (p < 0.05, chi2 analysis). On examination, increased head circumference was noted in 17 patients. Parinaud's syndrome was noted more prominently in patients with a history of intracranial neoplasm (4 of 8 cases) than in patients with nonneoplastic diseases (2 of 62 cases; p < 0.05, chi2 analysis). Other interesting presenting signs were pseudocyst (2), syringomyelia (2), hemiparesis (2) and Parkinson-like rigidity (2). Pediatric shunt malfunction generally presents with headache, nausea/vomiting, altered mental status, increased head circumference and bulging fontanelle. Other less frequent but unique presenting signs and symptoms, such as neck pain, syringomyelia and lower cranial nerve palsy in the myelodysplastic population, and Parinaud's syndrome in patients with a history of intracranial neoplasm are frequently associated with shunt malfunction and should prompt a radiographic workup.
Lymphangiomatosis of the bone is rare. The axial as well as appendicular skeleton may be affected... more Lymphangiomatosis of the bone is rare. The axial as well as appendicular skeleton may be affected. Neurosurgical consultation may be called for several reasons: (1) lesions involving the calvarium and/or spine; (2) nondiagnostic biopsies from more accessible and less morbid locations, and (3) persistent CSF leak and/or recurrent meningitis. Thus, it is important for the neurosurgeon to be familiar with this disease entity and consider it in the differential diagnoses of multifocal lytic lesions of the axial skeleton. We present a case report of a 4-year-old girl with lymphangiomatosis of the skull base and upper cervical spine with concurrent Chiari I malformation and briefly review the literature.
Pleomorphic xanthoastrocytomas (PXAs) are rare primary central nervous system neoplasms of childh... more Pleomorphic xanthoastrocytomas (PXAs) are rare primary central nervous system neoplasms of childhood and early adulthood. While PXAs are intimately associated with the leptomeninges, there has never been a description of an extradural-extracranial manifestation of PXA until now. We describe a case of PXA where the patient presented with a calvarial lesion. We also review the differential diagnoses of lytic skull lesions in addition to PXA.
The seizure outcome and neurological outcome in children who undergo reoperation for failed epile... more The seizure outcome and neurological outcome in children who undergo reoperation for failed epilepsy surgery have not been well documented. This retrospective study evaluated 20 children who underwent a second resective surgery for recurrent seizures. Four categories of patients were identified: (1) extension of the initial resection was performed in 8 patients; (2) 5 patients underwent lobectomy or corticectomy in a region remote from the original surgical site; (3) multilobar resection which may have included further resection of the initial procedure was accomplished in 4 patients; (4) hemispherectomy was performed in 3 patients. Patients with reoperation in the same lobe as the first procedure (group 1) had a 62% seizure-free rate, while 44% of patients in groups 2 and 3 were free from seizures at follow-up evaluation. Patients undergoing hemispherectomy had a 67% seizure-free rate. Significant unexpected neurological deficits occurred in 3 patients who underwent multilobar resection at reoperation. Complications included motor and language deficits. Reoperation for intractable partial epilepsy is beneficial in selected children. Patients who require multilobar resections may have higher risk of postoperative neurological deficit than those patients with reoperation in one lobe. These factors may be useful in counseling parents of children considering reoperation for recurrent epilepsy.
This study was undertaken to assess the utility of digital camera-derived intraoperative images i... more This study was undertaken to assess the utility of digital camera-derived intraoperative images in the planning of neurosurgery for children with epilepsy. A hand-held digital camera was used to capture the exposed surgical field at the time of craniotomy for 11 children with medically intractable seizure disorders. Intraoperative somatosensory evoked potential recordings of phase reversals and direct cortical stimulation were used to map areas of eloquent brain tissue. Digital camera images were obtained to mark regions of functional brain tissue with respect to cortical surface landmarks and subdural grid placement. The digital camera images were then immediately downloaded, in the operating room, to a laptop computer, which was placed next to the electroencephalographic recording device. Using computer software, the epileptologist highlighted the primary and secondary zones of epileptogenesis, as well as the functional brain areas identified during the monitoring period, on the digital camera images on the computer screen. A neurosurgical map was thus created to aid the neurosurgeon and the epileptologist with the proposed cortical resections and multiple subpial transections. With the images obtained using the digital camera, the epilepsy team was able to observe the contacts of the grid electrodes with the brain during the procedure. Color printouts of the images served as references during the period of invasive monitoring. Zones of primary and secondary epileptogenesis, as well as areas of functional brain tissue, were identified and plotted on the digital camera images. Other benefits of the digital camera-derived images included the ability to accurately reposition the grids or letters marking eloquent brain tissue if they were inadvertently shifted during the procedure, the ease with which the images could be obtained and manipulated, the ability to assess postresection epileptiform activity of the surrounding brain tissue with images obtained while an electrocorticographic array was in place, the ability to provide the entire epilepsy team with updated information on the neurosurgical field while minimizing movement in the operating room, and facilitation, with neurosurgical maps, of discussions with the patients and their families concerning proposed cortical resections. Digital camera images have become essential components for the planning of cortical resections for children with intractable epilepsy at our institution. We envision widespread application of this technology to other neurosurgical fields.
ABSTRACT OBJECTIVE Invasive urological procedures, commonly performed on patients with myelodyspl... more ABSTRACT OBJECTIVE Invasive urological procedures, commonly performed on patients with myelodysplasia, may contribute to the occurrence of cerebrospinal fluid shunt complications. Shunt complications that occurred after urological procedures in children with myelodysplasia were studied. METHODS Shunt complications occurring after urological procedures were examined in 29 patients. Differences between patients with or without complications were studied. Complications were analyzed according to the location of abnormality, the treatment, and the timing after shunt and urological surgery. RESULTS The 1-year incidence of shunt complications after a urological procedure had been performed was 31% (overall incidence, 41.4%). Shunt complications were observed only after intraperitoneal urological procedures. Most complications were distal, occurring more than 1 year after the preceding shunt surgery. The patients in the shunt complications group had significantly more intraperitoneal urological procedures (3.2 versus 0.8, P = 0.004) and previous shunt revisions (2.0 versus 0.9, P = 0.015) than had the group without complications. As compared with the group of patients with extraperitoneal complications, the intraperitoneal group experienced significantly more infections (4 of 9 versus 0 of 10, P = 0.014) requiring more complex treatment, and their complications occurred significantly earlier in the follow-up period after the urological procedure had been performed (7.2 versus 27.3 mo, P = 0.006). The patients in the group with extraperitoneal complications experienced significantly more mechanical shunt malfunctions than did the intraperitoneal group (9 of 10 versus 4 of 9, P = 0.016), which required simple shunt revisions. CONCLUSION Patients with spina bifida and shunted hydrocephalus may have an increased risk of developing intraperitoneal shunt complications after intraperitoneal urological procedures have been performed. These intraperitoneal shunt complications usually occur a few months after urological surgery and require complex treatment.
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Papers by Glenn Morrison