The Use of the Three-Dimensional Printed Polyether Ether Ketone Implant in Secondary Craniosynostosis Revision.

BACKGROUND: Skull deformities may be seen in patients years after craniosynostosis correction. These deformities cause psychosocial distress in affected patients. In this series, the authors describe the use of patient specific polyether ether ketone (PEEK) implants for correction of skull deformities after cranial vault remodeling for craniosynostosis. METHODS: A chart review was conducted for 3 revision procedures performed by 1 plastic surgeon in collaboration with 1 neurosurgeon, both affiliated with Northwell Health. Preoperative computed tomography scans were used to design three-dimensional (3D) printed PEEK implants manufactured by KLS Martin. Implants were used to correct frontal and orbital asymmetry and skull deformities in each patient. Outcomes were assessed at 1 week, 1 month, and 3 months post-operation. RESULTS: Two males and 1 female, ages 13, 17, and 19, underwent revision cranioplasty or orbital rim reconstruction using a custom, single piece 3D printed PEEK implant. All 3 patients underwent cranial vault remodeling in infancy; 1 was treated for coronal craniosynostosis and 2 were treated for metopic craniosynostosis. Revision cranioplasty operative times were 90, 105, and 147 minutes, with estimated blood loss of 45 mL, 75 mL, and 150 mL, respectively. One patient went home on post op day 1 and 2 patients went home on post op day 2. All patients had an immediate improvement in structural integrity and cranial contour, and all patients were pleased with their aesthetic results. CONCLUSIONS: Custom 3D printed PEEK implants offer a single piece solution in revision cranioplasty surgery to correct skull deformities after cranial vault remodeling for craniosynostosis.

Virtual Planning for Exchange Cranioplasty in Cranial Vault Remodeling.

ABSTRACT: The use of virtual surgical planning and computer-aided design/computer-aided manufacturing has gained popularity in the surgical correction of craniosynostosis. This study expands the use of virtual surgical planning and computer-aided design/computer-aided manufacturing in cranial vault reconstruction by using these methods to reconstruct the anterior vault using a single endocortically-plated unit constructed from the posterior calvarium. This technique was designed to reduce the risk of undesirable contour deformities that can occur when multiple bone grafts are used to reconstruct the anterior vault and fronto-orbital rim. Six patients were included in this study, all of which had nonsyndromic craniosynostosis. Excellent aesthetic outcomes were obtained in all patients, without complication. Additionally, the placement of a single reconstructive unit constructed from the posterior calvarium was efficient, aesthetically pleasing, and minimized postoperative contour deformities secondary to bone gaps, resorption, and often palpable resorbable plates.

Pedicled nasoseptal flap reconstruction for craniopharyngiomas in pediatric patients.

PURPOSE: Though the use of the pedicled nasoseptal flap (NSF), a reconstructive technique used after endoscopic endonasal approaches (EEA) for resection of craniopharyngiomas, has been shown to reduce the occurrence of post-operative cerebrospinal fluid (CSF) leaks in adults, less is known about its use in pediatric populations, specifically in children under the age of 7. The goal of this retrospective cohort study is to determine the viability of the pedicled NSF for pediatric patients. METHODS: Retrospective review of 12 pediatric patients (ages 2-16) undergoing 13 NSF reconstructions after resection of craniopharyngiomas. Radioanatomic analysis of computed tomography (CT) scans was utilized to classify the pneumatization of the sphenoid sinus depending on the thickness of the sphenoid bone margin. Intercarotid distances were measured from magnetic resonance imaging (MRI) scans to assess the feasibility of this reconstruction technique in pediatric patients. RESULTS: At the time of surgery, all patients were noted to have adequate NSF length and width. No post-operative high-flow CSF leaks were found within the group. Lack of pneumatization of the sphenoid sinus and narrow intercarotid distances in the youngest of patients did not lead to negative clinical outcomes. CONCLUSIONS: Based on our results and experience, the pedicled nasoseptal flap is a viable reconstructive option after EEA in the pediatric population, including even the youngest of patients. In these patients, a narrowed window between the intercarotid arteries and the lack of pneumatization of the sphenoid sinus present a challenge that can be overcome by using stereotactic navigation and advanced endoscopic techniques.

Outcomes following endoscopic endonasal resection of sellar and supresellar lesions in pediatric patients.

PURPOSE: The endoscopic endonasal approach (EEA) is a credible surgical alternative for the resection of sellar and suprasellar lesions such as pituitary adenomas, craniopharyngiomas, and Rathke cleft cysts. However, its application to pediatric patients poses several unique challenges that have not yet been well evaluated. The authors evaluate the safety, efficacy, and outcomes associated with the use of the EEA for treatment of these pathologic entities in pediatric patients. METHODS: Retrospective review of 30 patients between the ages of two and 24 who underwent endoscopic endonasal resection of sellar or suprasellar lesions between January 2010 and December 2015. Endocrinological and ophthalmological outcomes, as well as extent of resection and complications were all evaluated. RESULTS: Gross total resection was achieved in eight of the nine pituitary adenomas, nine of the 12 craniopharyngiomas, and six of the nine Rathke cleft cysts. Of the 30 patients, 22 remained disease free at last follow-up. A total of six patients developed hypopituitarism and five developed diabetes insipidus. Eleven patients experienced improved vision, sixteen experienced no change, and one patient experienced visual worsening. Postoperative cerebrospinal fluid leak was seen in a single case and later resolved, vasospasm/stroke was experienced by 10% of patients, and new obesity was recorded in 10% of patients. There were no perioperative deaths. CONCLUSIONS: Endoscopic endonasal resection is a safe and effective surgical alternative for the management of sellar and suprasellar pathologies in pediatric populations with excellent outcomes, minimal complications, and a low risk of morbidity.

Hydrocephalus associated with childhood nonaccidental head trauma.

OBJECTIVE The incidence of posttraumatic ventriculomegaly (PTV) and shunt-dependent hydrocephalus after nonaccidental head trauma (NAHT) is unknown. In the present study, the authors assessed the timing of PTV development, the relationship between PTV and decompressive craniectomy (DC), and whether PTV necessitated placement of a permanent shunt. Also, NAHT/PTV cases were categorized into a temporal profile of delay in admission and evaluated for association with outcomes at discharge. METHODS The authors retrospectively reviewed the cases of patients diagnosed with NAHT throughout a 10-year period. Cases in which sequential CT scans had been obtained (n = 28) were evaluated for Evans' index to determine the earliest time ventricular dilation was observed. Discharge outcomes were assessed using the King's Outcome Scale for Childhood Head Injury score. RESULTS Thirty-nine percent (11 of 28) of the patients developed PTV. A low admission Glasgow Coma Scale (GCS) score predicted early PTV presentation (within < 3 days) versus a high GCS score (> 1 week). A majority of PTV/NAHT patients presented with a subdural hematoma (both convexity and interhemispheric) and ischemic stroke, but subarachnoid hemorrhage was significantly associated with PTV/NAHT (p = 0.011). Of 6 patients undergoing a DC for intractable intracranial pressure, 4 (67%) developed PTV (p = 0.0366). These patients tended to present with lower GCS scores and develop ventriculomegaly early. Only 2 patients developed hydrocephalus requiring shunt placement. CONCLUSIONS PTV presents early after NAHT, particularly after a DC has been performed. However, the authors found that only a few PTV/NAHT patients developed shunt-dependent hydrocephalus.

Delay in Arrival to Care in Perpetrator-Identified Nonaccidental Head Trauma: Observations and Outcomes.

BACKGROUND: Children who sustained nonaccidental head trauma (NAHT) are at severe risk for mortality within the first 24 hours after presentation. OBJECTIVE: Extent of delay in seeking medical attention may be related to patient outcome. METHODS: A 10-year, single-institution, retrospective review of 48 cases treated at a large tertiary Children's Hospital reported to the New York State Central Registrar by the child protection team was conducted. The perpetrator was identified in 28 cases on the basis of confession or conviction. The medical and legal records allowed for identification of time of injury and the interval between injury and arrival to the hospital; this information was categorized as follows: <6 hours (without delay); 6-12 hours (moderate delay); and >12 hours (severe delay). The King's Outcome Scale for Childhood Head Injury (KOSCHI) score was recorded for each case. RESULTS: All children were 3 years of age or younger (2.1-34 months) and predominantly male (68%; 19/28). On arrival, 61% of patients (17/28) presented with moderate or severe delay. A low arrival Glasgow Coma Scale (GCS) score (P < 0.0001) and extracranial injuries (P < 0.0061) correlated with worse clinical patient outcomes. Patients with an arrival GCS score <7 predominantly arrived without delay or with moderate delay. Patients presenting without delay or with severe delay were more likely to have a higher KOSCHI outcome score on discharge (P < 0.0426). Four of the 6 patients who died presented after moderate delay. CONCLUSION: Patients presenting to medical care 6-12 hours after NAHT (moderate delay) appeared to have worse outcomes than those presenting earlier or later.

Choroid plexus papilloma and Pierpont syndrome.

The authors describe the case of a child who presented with hydrocephalus and phenotypic features characteristic of a multiple congenital anomalies/mental retardation syndrome. Dysmorphic facies, medial plantar lipomatosis, and developmental delay were observed in this case and are identical to documented findings of Pierpont syndrome diagnosed in 3 boys. This is the fourth case reported to date and is the first documented case of an oncological process- an intraventricular atypical choroid plexus papilloma tumor-found in association with Pierpont syndrome. Syndromes associated with choroid plexus papilloma are reviewed.

Role of Rac1-regulated signaling in medulloblastoma invasion. Laboratory investigation.

OBJECT: Medulloblastomas are the most common malignant brain tumors in children. These tumors are highly invasive, and patients harboring these lesions are frequently diagnosed with distant spread. In this study, the authors investigated the role of Rac1, a member of the Rho family of small guanosine triphosphatases, in medulloblastoma invasion. METHODS: Three established medulloblastoma cell lines were used: DAOY, UW-228, and ONS-76. Specific depletion of Rac1 protein was accomplished by transient transfection of small interfering RNA. Cell invasion through extracellular matrix (Matrigel) was quantified using a transwell migration assay. Mitogen activated protein kinase activation was determined using phospho-MAP kinase-specific antibodies, and inhibition of MAP kinase pathways was achieved by specific small molecule inhibitors. Localization of Rac1 and its expression levels were determined by immunohistochemical analysis using a Rac1-specific antibody, and Rac1 activation was qualitatively assessed by Rac1 plasma membrane association. RESULTS: Small interfering RNA-mediated depletion of Rac1 strongly inhibited medulloblastoma cell invasion. Although depletion of Rac1 inhibited the proliferation of UW-228 cells, and of ONS-76 cells to a lesser extent, it stimulated the proliferation of DAOY cells. Depletion of Rac1 also inhibited the activation of the ERK and JNK MAP kinase pathways, and inhibition of either pathway diminished invasion and proliferation. Immunohistochemical analysis demonstrated that the Rac1 protein was overexpressed in all medulloblastoma tumors examined, and indicated that Rac1 was hyperactive in 6 of 25 tumors. CONCLUSIONS: The authors' data show that Rac1 is necessary for the invasive behavior of medulloblastoma cells in vitro, and plays a variable role in medulloblastoma cell proliferation. In addition, these results indicate that Rac1 stimulates medulloblastoma invasion by activating the ERK and JNK pathways. The authors suggest that Rac1 and signaling elements controlled by this guanosine triphosphatase may serve as novel targets for therapeutic intervention in malignant medulloblastomas.

Seizure propagation in a patient with musicogenic epilepsy.

Musicogenic epilepsy (ME) is a condition in which seizures are triggered by music. We describe a novel investigation of the mechanisms of ME using noninvasive (ictal 2-flurodeoxyglucose (2-FDG) positron emission tomography (PET)) and invasive (subdural arrays and depth electrodes) methodology in one patient with ME. Ictal PET defined onsets in the right mesial temporal lobe. This was confirmed by intracranial electrode recordings, where there was onset in the right mesial temporal lobe, followed by sequential spread to the lateral temporal cortex, Heschel's gyrus, insula, and frontal lobes. Right temporal lobectomy resulted in Engel class 1 outcome.

Resting tremor secondary to a pineal cyst: case report and review of the literature.

BACKGROUND: Patients with pineal lesions typically present with the classical signs of increased intracranial pressure (headache, nausea, vomiting) and/or Parinaud's syndrome. Rare symptomatology of secondary parkinsonism attributed to pineal lesions has been previously reported in the literature. We describe an unusual case of a pineal cyst in a patient with the presenting sign of a resting tremor. CASE DESCRIPTION: We report an 18-year-old Caucasian female who presented with a 1-month history of a new-onset progressive, unilateral low-frequency right-hand resting tremor with associated headache, nausea, vomiting, and excessive diarrhea. Magnetic resonance imaging demonstrated an atypical appearance with enhancement of a mildly prominent pineal gland, possibly representing a pineal cyst. The patient did not exhibit radiographic signs of hydrocephalus. Based upon the radiographic appearance, one could not exclude with absolute certainty the presence of a malignancy. The patient ultimately underwent a bilateral suboccipital craniotomy with gross total resection of the lesion. Postoperatively, the patient exhibited immediate resolution of her preoperative resting tremor and continues to be symptom free at 1 year. CONCLUSIONS: A new-onset, resting tremor and/or other secondary parkinsonism symptoms should raise clinical suspicions of pineal lesions. Treatment can be guided based on tissue type and the presence or absence of hydrocephalus. We observed that complete surgical resection of the lesion provided the best treatment option for the total resolution of symptoms attributed to the disturbance of the microvasculature surrounding the nigro-striatal-pallidal system.

Case report: spontaneous intracranial hypotension in association with the presence of a false localizing C1-C2 cerebrospinal fluid leak.

BACKGROUND: Spontaneous intracranial hypotension is an increasingly recognized cause of new-onset, daily, persistent headaches. Although these headaches are similar to post-lumbar puncture headaches, characteristic differences include intracranial pachymeningeal enhancement, subdural fluid collections, and downward displacement of the brain. The identification of upper cervical epidural fluid collections as a false localizing sign in patients with SIH has provided significant insight into the selection of management options. CASE DESCRIPTION: We review a case of a 57-year-old woman who presented to our institution with progressive orthostatic headaches relieved by recumbency. The patient had no recent history of lumbar puncture, spinal, or intracranial procedure. The patient isolated the onset of symptoms to 3 weeks prior, when she was lifting heavy items in her home, and was diagnosed with SIH. Subsequently, she was found to have a C1-C2 epidural fluid collection. After much diagnostic consideration and review of the literature, the collection was defined as a false localizing sign; and the patient eventually underwent a lumbar EBP with complete resolution of her symptoms. CONCLUSIONS: Upper cervical fluid collections in patients with SIH often represent a false localizing sign. Conservative management should be instituted; and if unsuccessful, a lumbar EBP should be performed. It is important to note that C1-C2 fluid accumulations may not provide the actual leak site in patients presenting with SIH. This understanding prevents therapy from being unintentionally directed at C1-C2.

Trigonocephaly: surgical considerations and long term evaluation.

Trigonocephaly accounts for approximately 10% of all craniosynostosis. Severe trigonocephaly results in a triangular-shaped forehead, superior-lateral orbital depression, hypotelorism, and compensatory occipital-parietal calvarial changes. Radiographic findings include ovoid orbits with parallel medial borders, thickened keel-shaped frontal bone, small ethmoid sinuses, and a short anterior cranial fossa with pitched sphenoid wings. Our experience with 50 infantile cases of severe nonsyndromic trigonocephaly patients treated from 1987 to 2005 is clinically reviewed to assess long-term growth based on a standardized operative technique. The average age of the patients at surgery was 6 months and the mean follow-up was 12 years. Our complication rate was 2%; the reoperative rate was 12%. The use of resorbable rigid plate fixation combined with alloplastic augmentation has improved the cosmetic outcome in patients treated since 1996 and reduced the reoperative rate.

Chiari malformation and odontoid panus causing craniovertebral stenosis in a child with Crouzon's syndrome.

Crouzon's disease is a well-known disorder affecting multiple organ systems, specifically a craniofacial disorder with highly variable penetrance and severity of deformity. Crouzon's patients typically have anomalies of the skull base leading to gross distortion of the cranium and in some cases the cervicocranium. We present a 5-year-old girl with Crouzon's disease who suffered from an acquired Chiari I malformation after insertion of a ventriculoperitoneal shunt and a coexistent ventral odontoid panus. Both these lesions were causing cervicomedullary compression. The literature is controversial on the surgical management of anterior and posterior compression at the craniocervical junction. We review the literature on surgical options for decompression at the craniocervical junction and offer our surgical case as a treatment option for patients in this rare clinical situation.

Alloplastic reconstruction of large cranio-orbital defects: a comparative evaluation.

Norian CRS, Bone Cement (Synthes CMF), and Mimix (Lorenz) have been used to reconstruct large cranio-orbital defects in 85 patients. Resorbable mesh (Macropore), used in combination in selected patients, obviates dura pulsations that have been postulated to cause fragmentation of alloplastic material. Norian is composed of monocalcium phosphate, monohydrate, alpha-tricalcium phosphate, and calcium carbonate. Admixture with NaPO4 creates dahllite, which has a higher carbonate content (4%--6%) than hydroxyapatite (0%). CRS is soluble at low pH, facilitating its resorption and replacement by bone. In contradistinction, Mimix is converted to aqueous solution at 37 degrees C, supports fibrovascular ingrowth and bony interdigitation at the implant-material surface. Forty-five adults (mean age=42 years) and 40 children (mean age=8 years) were evaluated with respect to etiology of defect, size, location, gram usage of alloplast material, type of alloplast, postoperative clinical course, and complications. A minimum of 3-year follow-up is available; 22 adult patients additionally underwent resorbable mesh reconstruction. There were 7 (8%) complications, including infection, extrusion, a sterile loculated fluid collection and fragmentation. No difference in complication rate was noted between biomaterials. Two additional patients exhibited resorption (Norian), necessitating reaugmentation. Alloplastic replacement of cranio-orbital defects has recently advanced dramatically. Bivalved cranial bone grafting with its attached morbidity and sequelae can be avoided. Resorbable mesh allows for the placement of alloplast material in larger defects while avoiding dura pulsation causing alloplast fragmentation. In avoiding titanium type reconstruction, it obviates any interference with radiologic diagnosis and radiotherapeutic modalities. Long-term results are needed to assess bone growth within alloplast and to study bone growth in alloplastic reconstructed pediatric patients.

Precocious puberty associated with a pineal cyst: is it disinhibition of the hypothalamic-pituitary axis?

Accelerated development of secondary sexual characteristics or sexual precocity is a well-known entity. Most authors recognize two groups of patients, those described as having central precocious puberty (CPP) and those with precocious pseudopuberty. CPP results from premature activation of the hypothalamic-pituitary-gonadal axis and pseudopuberty is caused by lesions that secrete gonadotropin-like substances or hormones. The onset of CPP is usually before age 8 in females and age 9 in males; however, there is contention that the age of onset is much earlier and also differs depending on the patients' race. Previously reported causes of CPP include intracranial neoplasm, infection, trauma, hydrocephalus and Angelman's syndrome. Pineal cysts are usually asymptomatic incidental findings, but have been associated with CPP. We present an interesting case of a patient with CPP and an associated pineal cyst. We review the literature on the pathogenesis of CPP and associated pineal cyst, the neuroendocrine relationship between the pineal gland and puberty and the neurosurgical role in these cases.

Craniovertebral instability with spinal cord compression in a 17-month-old boy with Sly syndrome (mucopolysaccharidosis type VII): a surgical dilemma.

STUDY DESIGN: A case study with review of surgical technique in craniovertebral stabilization for young children with mucopolysaccharidosis. OBJECTIVES: To describe an interesting patient with a rare metabolic disorder and review surgical technique for craniovertebral instability in this rare patient population. SUMMARY OF BACKGROUND DATA: Craniovertebral instability has been reported in patients with mucopolysaccharidosis and poses a problem for spinal surgery because of the inherent metabolic disorder and age at presentation. We present the first case of craniovertebral instability and spinal cord compression occurring in Sly syndrome (mucopolysaccharidosis type VII) who is the youngest patient afflicted with this metabolic disorder to undergo craniovertebral stabilization. METHODS: A 17-month-old boy presented with inability to support his head, decreasing muscle strength in all extremities, distended abdomen, and shortness of breath. The patient was found to have a dilated cardiomyopathy, hepatosplenomegaly, abnormal hepatobiliary function, corneal clouding, and a questionable tracheal anomaly. Genetic testing provided a diagnosis of Sly syndrome, mucopolysaccharidosis type VII. Magnetic resonance imaging revealed focal stenosis with significant spinal cord compression at the craniovertebral junction. Neurologic examination revealed normal muscle volume with strength 3/5 in all extremities and significant weakness in the neck muscles with instability at the craniovertebral junction. RESULTS: On a concerted preoperative medical clearance by pediatric intensive care, pediatric neuroanesthesia, pediatric cardiology, pediatric gastroenterology, and pediatric neurosurgery, the patient underwent occipital to C3 decompression and fusion with autogenous rib grafts. The patient was placed in a prefitted halo-vest after surgery and was neurologically intact. CONCLUSIONS: This case demonstrates the heterogeneity of cervical spine deformities among the mucopolysaccharidosis syndromes and confirms the propensity for deposition of glycosaminoglycans at the craniovertebral junction. Further studies should investigate the etiology for this propensity of glycosaminoglycan deposition at the craniovertebral junction. We think that this case demonstrates that, with appropriate preoperative planning, these patients can undergo successful posterior cervical arthrodesis despite their age or metabolic defects.

New method of pediatric cranioplasty for skull defect utilizing polylactic acid absorbable plates and carbonated apatite bone cement.

Cranial defect repair in the pediatric population requires a variety of special considerations. The pediatric skull has a dynamic nature that prohibits the use of rigid fixation, which is commonly applied in the adult population. A technique using a combination of polylactic acid plates and carbonated apatite bone cement has been devised by our group. Skull defects of varying sizes were repaired in 34 pediatric patients. Patients were examined on postoperative day 3 and at 3 months via three-dimensional computed tomography scans. Patients have been followed up to 60 months after surgery without complications or failures to date. This method benefits the pediatric patients undergoing cranioplasty by minimizing the insertion of long-term foreign bodies and allows the possibility for transformation of this construct into viable tissue.

Rapid neurological deterioration associated with minor head trauma in chronic hydrocephalus.

CASE REPORT: An 8-year-old developmentally normal boy (status: post third ventriculostomy and resection of posterior fossa low-grade glioma 4 years earlier and with known history of ventriculomegaly/arrested hydrocephalus) presented to the emergency room with vomiting and lethargy after a minor head trauma. Computed tomography scan of the head revealed no acute changes since previous studies. However, the patient's neurological status rapidly declined in the emergency room, where an emergency ventriculostomy demonstrated increased intracranial pressure. The patient's clinical condition improved over 24 h: he underwent placement of a ventriculoperitoneal shunt without complications and was discharged intact. DISCUSSION: The pathogenesis of rapid neurological decline associated with minor head trauma in chronic hydrocephalus is reviewed.

The role of surgical placement and pump orientation in intrathecal pump system failure: a technical report.

Intrathecal pump catheter complications are the most common cause of failure in drug delivery. A previous report has documented that intra-abdominal positioning of the intrathecal pump may predispose the pump-catheter neck to premature catheter breakdown and leakage. Based on this report, we reviewed over 100 intrathecal pump cases to determine the frequency of malpositioning and its role in the pathogenesis of catheter failure. We found three specific cases where a 'fulcrum effect' occurred due to intra-abdominal positioning of the pump predisposing the catheter to breakdown. This study demonstrates that intra-abdominal placement of the pump can predispose the catheter to failure/breakdown and that surgeons should attempt to place the pump catheter neck in a superiomedial position, distant from any bony prominences, to prevent the 'fulcrum effect' on the pump-catheter neck junction and reducing the likelihood of either internal or external compressive forces.