Prenatal diagnosis of meningomyelocele resolves as a mature cystic teratoma in the thoracolumbar region.

A mature cystic teratoma is a mass with heterogeneous appearance, consisting of adult tissue with two or three layers: endoderm, mesoderm, and ectoderm. It is a rare, benign transformation of somatic tissue most commonly found in the sacrococcygeal region and may resemble an uncomplicated spina bifida on prenatal ultrasonography. In this case report, we describe a female newborn with an extremely rare mature cystic teratoma in the thoracolumbar region. She presented prenatally with a preliminary diagnosis of meningomyelocele, diastematomyelia, and Chiari II malformation and a possible teratoma. However, a mass containing solid glandular tissues and bony calcifications approximately 3 × 4 cm in size was observed in the thoracolumbar region upon birth. During surgical resection, no nerve roots were found in the associated meningocele. The patient retained full lower body function postoperatively following surgical excision of the thecal sac and teratoma.

Changes in Cerebral Oxygenation in Preterm Infants With Progressive Posthemorrhagic Ventricular Dilatation.

BACKGROUND: Optimal timing of intervention in neonatal progressive posthemorrhagic hydrocephalus is often a difficult decision. Unchecked hydrocephalus can lead to irreversible brain injury through impaired perfusion, while placement of a shunt is not without long-term morbidity. The purpose of this study was to assess the use of near-infrared spectroscopy to measure changes in regional cerebral oxygen saturation as an indicator of cerebral perfusion in infants with progressive posthemorrhagic ventricular dilatation. METHODS: Near-infrared spectroscopy was used to measure regional cerebral oxygen saturation for more than a one-hour period in infants within 24 hours of cranial ultrasound. Simultaneous pulse oximetry was recorded and oxygen extraction was calculated. Ventricular size was measured by ultrasound using the frontal-occipital horn ratio and compared with average oxygen saturation and oxygen extraction. Statistical analysis was done using the Spearman rank test and analysis of variance. RESULTS: Ventricular measurements were made in 20 very low birth weight premature infants with periventricular-intraventricular hemorrhage and 12 infants with normal ultrasound scans. Ventricular dilatation was associated with lower cerebral oxygen saturation and higher oxygen extraction (P < 0.001). Progressive ventricular dilatation was inversely related to changes in cerebral oxygen saturation (P < 0.001). CONCLUSIONS: Progressive posthemorrhagic ventricular dilatation is associated with a significant decrease in cerebral oxygenation and increase in oxygen extraction suggesting a decrease in cerebral perfusion. Near-infrared spectroscopy could potentially provide additional clinical information to assist in determining optimal timing of surgical intervention in preterm infants with progressive ventricular enlargement.

Craniosynostosis of the lambdoid suture.

Craniosynostosis affecting the lambdoid suture is uncommon. The definition of lambdoid craniosynostosis solely applies to those cases demonstrating true suture obliteration, similar to other forms of craniosynostosis. In patients presenting with posterior plagiocephaly, true lambdoid craniosynostosis must be differentiated from the much more common positional molding. It can occur in a unilateral form, a bilateral form, or as part of a complex craniosynostosis. In children with craniofacial syndromes, synostosis of the lambdoid suture most often is seen within the context of a pansynostotic picture. Chiari malformations are commonly seen in multisutural and syndromic types of craniosynostosis that affect the lambdoid sutures. Posterior cranial vault remodeling is recommended to provide adequate intracranial volume to allow for brain growth and to normalize the skull shape. Although many techniques have been described for the correction of lambdoid synostosis, optimal outcomes may result from those techniques based on the concept of occipital advancement.

Minimizing transfusion requirements for children undergoing craniosynostosis repair: the CHoR protocol.

OBJECT: Children with craniosynostosis may require cranial vault remodeling to prevent or relieve elevated intracranial pressure and to correct the underlying craniofacial abnormalities. The procedure is typically associated with significant blood loss and high transfusion rates. The risks associated with transfusions are well documented and include transmission of infectious agents, bacterial contamination, acute hemolytic reactions, transfusion-related lung injury, and transfusion-related immune modulation. This study presents the Children's Hospital of Richmond (CHoR) protocol, which was developed to reduce the rate of blood transfusion in infants undergoing primary craniosynostosis repair. METHODS: A retrospective chart review of pediatric patients treated between January 2003 and Febuary 2012 was performed. The CHoR protocol was instituted in November 2008, with the following 3 components; 1) the use of preoperative erythropoietin and iron therapy, 2) the use of an intraoperative blood recycling device, and 3) acceptance of a lower level of hemoglobin as a trigger for transfusion (< 7 g/dl). Patients who underwent surgery prior to the protocol implementation served as controls. RESULTS: A total of 60 children were included in the study, 32 of whom were treated with the CHoR protocol. The control (C) and protocol (P) groups were comparable with respect to patient age (7 vs 8.4 months, p = 0.145). Recombinant erythropoietin effectively raised the mean preoperative hemoglobin level in the P group (12 vs 9.7 g/dl, p < 0.001). Although adoption of more aggressive surgical vault remodeling in 2008 resulted in a higher estimated blood loss (212 vs 114.5 ml, p = 0.004) and length of surgery (4 vs 2.8 hours, p < 0.001), transfusion was performed in significantly fewer cases in the P group (56% vs 96%, p < 0.001). The mean length of stay in the hospital was shorter for the P group (2.6 vs 3.4 days, p < 0.001). CONCLUSIONS: A protocol that includes preoperative administration of recombinant erythropoietin, intraoperative autologous blood recycling, and accepting a lower transfusion trigger significantly decreased transfusion utilization (p < 0.001). A decreased length of stay (p < 0.001) was seen, although the authors did not investigate whether composite transfusion complication reductions led to better outcomes.

Giant cell reparative granuloma of the pediatric cranium: case report and review of the literature.

PURPOSE: Giant cell reparative granulomas are rare bone tumors. Although benign, these tumors are locally destructive and can be highly vascular. They seldom occur in the cranial vault. We describe a multidisciplinary approach to a case of giant cell reparative granuloma of the cranium in a 3-year-old patient. CASE REPORT: A 3-year-old girl female referred to the pediatric neurosurgery department for evaluation of a retro-auricular mass. She had a history of recurrent otitis media with two subsequent courses of antibiotics without resolution. CT imaging revealed an expansive lesion located in the right mastoid region. Open surgical biopsy revealed a hemorrhagic tumor consistent with a giant cell reparative granuloma. Angiography identified a hypervascular tumor blush that was supplied by the occipital artery. Preoperative transcatheter embolization was performed followed by a multidisciplinary surgical resection and reconstruction. Blood loss was minimal, and the patient recovered well after surgery. CONCLUSION: Preoperative endovascular embolization and a multidisciplinary intraoperative approach with primary resection and cranial vault reconstruction is an effective approach to hypervascular giant cell reparative granulomas.

Management of patients with cochlear implants and ventriculoperitoneal shunts.

OBJECTIVES: The aim of this study is to provide an overview of each device, discuss conditions that may predispose to the requirement of both devices, and consider pre- and post-operative issues arising from the co-existence of advanced technologies. In addition, relevant surgical anatomy and technique in the placement of both cochlear implant (CI) after ventriculoperitoneal shunt (VPS) and VPS after CI are reviewed. METHODS: Review of literature. RESULTS: Issues relevant to CI and VPS include predisposing conditions, magnetic resonance imaging compatibility, infectious risk, surgical technique, complication management, and a host of other issues. DISCUSSION: Patients with hydrocephalus and severe-to-profound hearing loss are a unique patient population with unique pre-, intra-, and post-operative issues. Thorough understanding of the indications, radiology, anatomy, technology, and potential complications can allow for these patients to achieve the full benefit of these lifechanging surgeries.

Sonographic localization of a nonpalpable shunt: Ultrasound-assisted ventricular shunt tap.

BACKGROUND: Patients frequently present to the emergency department (ED) for evaluation of cerebrospinal fluid (CSF) shunt malfunction, often requiring urgent management. A typical evaluation in the emergency room setting includes a thorough history and physical examination, noncontrasted head computed tomography (CT), shunt series, and occasionally a ventricular shunt tap. CASE DESCRIPTION: We present the case of a 53-year-old male who initially presented to the ED in acute status epilepticus. His history was notable for seizures and multiple craniectomies and cranioplasties with subsequent placement of a ventriculoperitoneal shunt secondary to traumatic brain injury. Imaging in the ED suggested possible shunt failure. No previous imaging was available for comparison, and therefore a ventricular shunt tap was attempted. Initially, the tap could not be performed, as the shunt was not palpable secondary to the thickness of his scalp and location of the reservoir near his complex cranial reconstruction site. We report, for the first time, the utility of emergency ultrasound (EUS) to aid in such an encounter. CONCLUSION: EUS revealed the exact location of his shunt reservoir, and therefore enabled the shunt tap, which ultimately led to the discovery of the patient's proximal shunt failure in a setting that may have otherwise been missed. The patient underwent urgent shunt revision with a good outcome.

Desmoplastic fibroma of the pediatric cranium: case report and review of the literature.

PURPOSE: Desmoplastic fibromas are primary bone tumors that seldom occur in the cranial bones. Furthermore, reports of desmoplastic fibromas of the skull in children are exceedingly rare. Although desmoplastic fibromas are histologically benign, they are locally aggressive and have a propensity to reoccur. Their radiographic appearance may mimic other more common central nervous system and bone neoplasms. There are only 19 reported cases of desmoplastic fibroma of the cranium in the literature, and only seven occurred in the pediatric age group. We present a case report of an 11-year-old female patient with a desmoplastic fibroma of the parieto-occipital region and review the literature. CASE REPORT: An 11-year-old female presented to the craniofacial clinic complaining of intermittent pain and a soft mass in the occipital region. There was a distant history of trauma to the region that did not require medical intervention. Computed tomography imaging revealed a lytic bone lesion overlying the sagittal sinus in the parieto-occipital region. Surgical resection with wide margins and immediate autologous reconstruction was performed. Pathological analysis revealed a desmoplastic fibroma. At 4 months of follow-up, no recurrence has been noted. CONCLUSION: Desmoplastic fibroma of the cranium is rare. Complete surgical resection with careful follow-up is the treatment of choice.

Intradiploic dermoid cyst of the lateral frontotemporal skull: case report and review of the literature.

BACKGROUND: Intradiploic dermoid cysts represent 0.04-0.7% of cranial tumors. Fewer than 20 cases of dermoid cysts occurring in the lateral frontotemporal region with a sinus tract and bony involvement are described, only 7 with intracranial extension. We present the first report of such a lesion arising within the lateral coronal suture. As the literature on this topic grows, the matter of preoperative imaging for soft tissue and bony lesions of the lateral frontotemporal region is evolving, and this report offers a preliminary set of criteria for when imaging is a necessity. CASE REPORT: A 2-year-old male presented with a bony lesion in the right frontotemporal region. Since birth the lesion had grown commensurately with the patient. Examination revealed an immobile hard mass overlying the right coronal suture with no discernable abnormality. Computed tomography demonstrated a cystic lesion without evidence of intracranial extension. Intraoperatively, the exophytic lesion was fully enclosed by bony matrix, interrupting the coronal suture as it approached the pterion. Following resection, pathology revealed an intradiploic dermoid cyst. CONCLUSION: Intradiploic dermoid cysts occurring within patent cranial sutures away from the midline are rarely described lesions. Complete surgical resection with careful follow-up is the treatment of choice.

Dental surgery and anesthetic precautions of a patient with Down syndrome and juvenile rheumatoid arthritis: a case report.

Down syndrome and juvenile rheumatoid arthritis have been associated with ligament laxity, specifically the atlantoaxial ligament, which maintains the proper positioning of the cervical first and second vertebrae. The joint consists of different pathological processes, and it is paramount that individuals with either condition be screened appropriately before surgery is contemplated. The purpose of this paper was to present a case of an individual with both conditions and describe precautionary measures that were undertaken to safely complete dental treatment under general anesthesia and avoid morbidity.

Intraventricular twin fetuses in fetu.

The authors report a rare case of multiple intracranial fetuses in fetu, fulfilling Willis' traditional criteria, which include an axial and appendicular skeleton with surrounding organized tissue. This case was ascertained from studies of a full-term female neonate who presented with ventriculomegaly. A CT scan showed intracranial calcifications that were suggestive of an axial skeleton. Her birth weight was 3.176 kg (50th-75th percentile), length was 52 cm (90th percentile), head circumference was 35 cm (50th-75th percentile), and Apgar scores were 7 and 8 at 1 and 5 minutes, respectively. Prenatal ultrasonography studies performed at 12 weeks and 5 days, and 19 weeks and 6 days revealed normal findings. A 37-week prenatal ultrasonography study showed ventriculomegaly and obstructive hydrocephalus, with a possible intracranial teratoma. Cranial imaging at birth with ultrasonography, CT and MR imaging, and MR angiography demonstrated 2 complex intraventricular masses with cystic, solid, and bony elements. A craniotomy with resection of the masses was performed at 3 months of age. The infant survived and is now 12 months old with some developmental progress. Two axial skeletons, with accompanying rib cage and extremities, including well-formed feet and toes, were noted. Both anencephalic structures had skin with hair, fat, skeletal and smooth muscle, and bony structures with bone marrow and focal areas of calcification. Multiple viscera were present and included thymus, bowel, stomach, salivary gland, kidney, adrenal gland, lung, and presumed adnexal structures. A diagnosis of fetuses in fetu was rendered. Chromosomal studies of the child and tissue from the 2 fetuses in fetu showed normal female karyotypes. A single nucleotide polymorphism array analysis from the proband infant and tissue from the 2 identified fetuses in fetu appeared to be genetically identical. These results are consistent with a monozygotic twin embryonic origin of the fetus in fetu tissue, which is a mechanism that has been suggested in previous reports in which karyotypes, blood types, and limited genetic loci have been studied. This is the first report of a rare example of intracranial intraventricular twin fetuses in fetu for which a genome-wide single nucleotide polymorphism assay has confirmed their genetic identity.

The alkylphospholipid perifosine induces apoptosis and p21-mediated cell cycle arrest in medulloblastoma.

Medulloblastoma is the most common malignant cancer of the central nervous system in children. AKT kinases are part of a survival pathway that has been found to be significantly elevated in medulloblastoma. This pathway is a point of convergence for many growth factors and controls cellular processes that are critical for tumor cell survival and proliferation. The alkyl-phospholipid perifosine [octadecyl-(1,1-dimethyl-4-piperidylio) phosphate] is a small molecule inhibitor in clinical trials in peripheral cancers which acts as a competitive inhibitor of AKT kinases. Medulloblastoma cell cultures were used to study the effects of perifosine response in preclinical studies in vitro. Perifosine treatment led to the rapid induction of cell death in medulloblastoma cell lines, with pronounced suppression of phosphorylated AKT in a time-dependent and concentration-dependent manner. LD(50) concentrations were established using viability assays for perifosine, cisplatin, and etoposide. LD(50) treatment of medulloblastoma cells with perifosine led to the cleavage of caspase 9, caspase 7, caspase 3, and poly-ADP ribosylation protein, although caspase 8 was not detectable. Combination single-dose treatment regimens of perifosine with sublethal doses of etoposide or irradiation showed a greater than additive effect in medulloblastoma cells. Lower perifosine concentrations induced cell cycle arrest at the G(1) and G(2) cell cycle checkpoints, accompanied by increased expression of the cell cycle inhibitor p21(cip1/waf1). Treatment with p21 small interfering RNA prevented perifosine-induced cell cycle arrest. These findings indicate that perifosine, either alone or in combination with other chemotherapeutic drugs, might be an effective therapeutic agent for the treatment of medulloblastoma.

A model for neurosurgical humanitarian aid based on 12 years of medical trips to South and Central America.

The pediatric neurosurgical mission trips led by physicians at Virginia Commonwealth University (VCU) Health Systems began in 1996 with the formation of Medical Outreach to Children, founded by 1 of the authors (J.D.W.) after a visit to Guatemala. Since then, 19 surgical trips to 4 different countries in Central and South America have been coordinated from 1996 to 2008. This humanitarian work serves a number of purposes. First and foremost, it provides children with access to surgical care that they would otherwise not receive, thereby significantly improving their quality of life. Second, the visiting surgical team participates in the education of local physicians, parents, and caregivers to help improve the healthcare provided to the children. Last, the team works to promote sustainable global health solutions in the countries it travels to by generating a forum for clinical and public health research discourse. Thus far, a total of 414 children have undergone 463 operations, including 154 initial shunt surgeries, 110 myelomeningocele repairs, 39 lipoma resections, 33 tethered cord releases, 18 shunt revisions, 16 encephalocele repairs, 9 lipomyelomeningocele repairs, and 7 diastematomyelia repairs. The complication rate has been 5-8%, and the team has obtained reliable follow-up in approximately 77% of patients. A correlation was found between an increase in the number of trained neurosurgeons in the host countries and a decrease in the average age of patients treated by the visiting surgical team over time. It is also hypothesized that a decrease in the percentage of myelomeningocele repairs performed by the surgical team (as a fraction of total cases between 1996 and 2006) correlates to an increase in the number of local neurosurgeons able to treat common neural tube defects in patients of younger ages. Such analysis can be used by visiting surgical teams to assess the changing healthcare needs in a particular host country.

Graft subsidence after instrument-assisted anterior cervical fusion.

OBJECT: Bone grafts used in anterior cervical fusion (ACF) may subside postoperatively. The authors reviewed a recent series in which instrument-assisted ACF was performed to determine the degree of subsidence with respect to fusion length, use of segmental screws, and patient smoking status, age, and sex. METHODS: Charts and implant records were reviewed for all 70 patients who underwent instrument-assisted ACF during a 2-year period. The procedures, grafting materials, plate types/lengths, and patient smoking status were recorded. The immediate postoperative and follow-up lateral radiographs were analyzed. The plate lengths and lengths of the fused segments were measured in a standardized fashion. The mean intraoperative and follow-up fusion segment lengths were 54.3 and 51.9 mm, respectively. Greater subsidence occurred in multilevel fusions than in single-level fusions. There were noticeable changes in the position of plates or screws on 14 of 70 follow-up x-ray films. No new neurological deficits related to graft subsidence occurred, and the reoperation rate was 3%. There was no statistical relation between subsidence and the following variables: segmental fixation, smoking status, sex, age, or dowel size when corrected for length of the plate. Hardware migration correlated significantly with plate length in cases of two- and three-level fusions. CONCLUSIONS: The length of a fusion segment decreases in the immediate weeks following instrument-assisted ACF. Construct length is the most important determinant of subsidence. When designing multilevel cervical constructs, consideration of the effects of graft subsidence may help to avoid hardware-related complications.