Occipital condyle to cervical spine fixation in the pediatric population.

Fixation at the craniovertebral junction (CVJ) is necessary in a variety of pediatric clinical scenarios. Traditionally an occipital bone to cervical fusion is preformed, which requires a large amount of hardware to be placed on the occiput of a child. If a patient has previously undergone a posterior fossa decompression or requires a decompression at the time of the fusion procedure, it can be difficult to anchor a plate to the occipital bone. The authors propose a technique that can be used when faced with this difficult challenge by using the occipital condyle as a point of fixation for the construct. Adult cadaveric and a limited number of case studies have been published using occipital condyle (C-0) fixation. This work was adapted for the pediatric population. Between 2009 and 2012, 4 children underwent occipital condyle to axial or subaxial spine fixation. One patient had previously undergone posterior fossa surgery for tumor resection, and 1 required decompression at the time of operation. Two patients underwent preoperative deformity reduction using traction. One child had a Chiari malformation Type I. Each procedure was performed using polyaxial screw-rod constructs with intraoperative neuronavigation supplemented by a custom navigational drill guide. Smooth-shanked 3.5-mm polyaxial screws, ranging in length from 26 to 32 mm, were placed into the occipital condyles. All patients successfully underwent occipital condyle to cervical spine fixation. In 3 patients the construct extended from C-0 to C-2, and in 1 from C-0 to T-2. Patients with preoperative halo stabilization were placed in a cervical collar postoperatively. There were no new postoperative neurological deficits or vascular injuries. Each patient underwent postoperative CT, demonstrating excellent screw placement and evidence of solid fusion. Occipital condyle fixation is an effective option in pediatric patients requiring occipitocervical fusion for treatment of deformity and/or instability at the CVJ. The use of intraoperative neuronavigation allows for safe placement of screws into C-0, especially when faced with a challenging patient in whom fixation to the occipital bone is not possible or is less than ideal.

Presurgical connectome and postsurgical seizure control in temporal lobe epilepsy.

OBJECTIVES: The objective of this study was to evaluate whether patients with surgically refractory medial temporal lobe epilepsy (MTLE) exhibit a distinct pattern of structural network organization involving the temporal lobes and extratemporal regions. METHODS: We retrospectively studied 18 healthy controls and 20 patients with medication refractory unilateral MTLE who underwent anterior temporal lobectomy for treatment of seizures. Patients were classified as seizure-free or not seizure-free at least 1 year after surgery. The presurgical brain connectome was calculated through probabilistic connectivity from MRI-diffusion tensor imaging from 83 anatomically defined regions of interest encompassing the whole brain. The connectivity patterns were analyzed regarding group differences in regional connectivity and network graph properties. RESULTS: Compared with controls, patients exhibited a decrease in connectivity involving ipsilateral thalamocortical regions, with a pathologic increase in ipsilateral medial temporal lobe, insular, and frontal connectivity. Among patients, those not seizure-free exhibited a higher connectivity between structures in 1) the ipsilateral medial and lateral temporal lobe, 2) the ipsilateral medial temporal and parietal lobe, and 3) the contralateral temporal pole and parietal lobe. Patients not seizure-free also exhibited lower small-worldness in the subnetwork within the ipsilateral temporal lobe, with higher subnetwork integration at the expense of segregation. CONCLUSIONS: MTLE is associated with network rearrangement within, but not restricted to, the temporal lobe ipsilateral to the onset of seizures. Networks involving key components of the medial temporal lobe and structures traditionally not removed during surgery may be associated with seizure control after surgical treatment of MTLE.

Subtypes of medial temporal lobe epilepsy: influence on temporal lobectomy outcomes?

Surgical resection of the hippocampus is the most successful treatment for medication-refractory medial temporal lobe epilepsy (MTLE) due to hippocampal sclerosis. Unfortunately, at least one of four operated patients continue to have disabling seizures after surgery, and there is no existing method to predict individual surgical outcome. Prior to surgery, patients who become seizure free appear identical to those who continue to have seizures after surgery. Interestingly, newly converging presurgical data from magnetic resonance imaging (MRI) and intracranial electroencephalography (EEG) suggest that the entorhinal and perirhinal cortices may play an important role in seizure generation. These areas are not consistently resected with surgery and it is possible that they continue to generate seizures after surgery in some patients. Therefore, subtypes of MTLE patients can be considered according to the degree of extrahippocampal damage and epileptogenicity of the medial temporal cortex. The identification of these subtypes has the potential to drastically improve surgical results via optimized presurgical planning. In this review, we discuss the current data that suggests neural network damage in MTLE, focusing on the medial temporal cortex. We explore how this evidence may be applied to presurgical planning and suggest approaches for future investigation.

Difficulties of delayed treatment of craniosynostosis in a patient with Crouzon, increased intracranial pressure, and papilledema.

Crouzon syndrome is an autosomal dominant disorder characterized by cranial synostosis, hypertelorism, orbital proptosis, parrot-beaked nose, short upper lip, hypoplastic maxilla, and a relative mandibular prognathism, without extremity involvement. Surgical intervention should occur at the onset of progressive craniosynostosis to treat or to prevent intracranial hypertension and visual impairment. Throughout developing countries, early treatment is often not a viable option. Often, the only option for treatment is through humanitarian missions. Appropriate preselection of surgical patients is essential, as is having a multidisciplinary team and a well-equipped hospital and staff to perform the operations and to care for the postsurgical patient. This type of humanitarian care benefits the patient selected to receive the intense logistical and financial effort when there is no possibility of timely care in their own countries. This clinical report describes a patient with Crouzon syndrome brought to the United States from a developing country through humanitarian efforts. She presented at 19 months of age with bicoronal and sagittal synostosis and advanced visual impairment and papilledema. Surgical intervention included cranial expansion and reconstruction with a multidisciplinary team. This case illustrates not only the difficulty of delayed treatment but also some of the issues arising from this type of humanitarian medical care.

Time-dependent perioperative anesthetic management and outcomes of the first 100 consecutive cases of spring-assisted surgery for sagittal craniosynostosis.

BACKGROUND: The anesthetic risks and outcomes of the first 100 consecutive spring-assisted surgeries (SAS) for cranial expansion from a single institution are reported. The effect of number of procedures was also tested on hematocrit postoperative day 1 (POD1), anesthesia time, and surgery time of the first procedure. METHODS: The records of 100 consecutive patients undergoing SAS were reviewed. Anesthesia management and related complications are presented. Time series linear regression analysis was performed on hematocrit POD1, anesthesia time, and surgery time of the first procedure. RESULTS: The average age of the first insertion procedure was 4.4 and 9.0 months for the second removal procedure. Two patients were inadvertently extubated during positioning. Thirty-eight children had a decrease in blood pressure >20% from baseline. No child was admitted to the intensive care unit. No patient received any blood or blood product transfusion. Anesthesia time, surgery time, and hematocrit POD1 were correlated with procedure number or experience. CONCLUSIONS: Changes in anesthetic management resulted from changing the procedure. The reduction in volume resuscitation reduces the need for invasive monitoring. Facility and comfort with the surgical procedure increase with time and number of procedures performed. This experience further reduces blood loss and risk of transfusion.

Increased incidence of metopic suture abnormalities in children with positional plagiocephaly.

Metopic craniosynostosis has traditionally been reported to be the third most common form of single-suture synostosis. The purposes of this article were to analyze the relationship between metopic craniosynostosis and positional plagiocephaly and to define more clearly the differences between the changes seen with true suture fusion compared with metopic abnormalities secondary to positional changes. This is an institutional review board-approved retrospective review of three-dimensional computed tomographic scans for abnormalities of the metopic suture in all children treated at our institution for positional plagiocephaly between 1997 and 2007. We also independently reviewed the images of all patients treated for metopic synostosis for evidence of positional plagiocephaly during the same period. Among the positional plagiocephaly group, 39% were noted to have some form of metopic suture abnormality. Of the children treated for metopic craniosynostosis, a much smaller percentage also was noted to have evidence of positional plagiocephaly. Between 1997 and 2007, we treated 93 patients (50%) for sagittal synostosis, 41 patients (22%) for unicoronal, 41 patients (22%) for metopic, 4 patients (2%) for lamdoid, and 7 patients (4%) with multiple-suture involvement. This study demonstrates an increasing trend toward metopic suture abnormalities during the past 10 years, which corresponds to the same time interval of an increased incidence of positional abnormalities. It is postulated that some of these abnormalities are related to deformational forces resulting from posterior pressure. Altered mechanical forces from supine infant positioning may be associated with early metopic suture ridging and dysmorphology distinct from true craniosynostosis.

Sudden appearance of new upper extremity motor function while performing neurophysiologic intraoperative monitoring during tethered cord release: a case report.

UNLABELLED: : Tethered cord syndrome occurs when the distal spinal cord or filum adheres to adjacent structures resulting in progressive sensorimotor deficits in the lower extremities, fecal and/or urinary incontinence, and musculoskeletal deformities. Tethering of the distal cord may be idiopathic, may be associated with an intraspinal abnormality such as a lipoma, but most commonly the distal spinal cord remnant is adherent to the area of the original dysraphism repair in patients with myelodysplasia. Surgery to untether the cord is indicated in patients with worsening pain symptoms, progressive limb deformity or spasticity, or before any acute correction of an associated spinal deformity. Neurophysiologic intraoperative monitoring is used to minimize the risk of inadvertent nerve root or spinal cord injury during the untethering procedure and to assess any changes in cord function at the time of an associated spinal deformity correction. We present a patient with a lumbar level myelodysplasia, Chiari II malformation, severe scoliosis, and tethered cord that underwent concurrent scoliosis correction and tethered cord syndrome surgery, who demonstrated immediate intraoperative improvement in neurophysiologic responses in a previously flaccid upper extremity after untethering. These monitoring changes correlated with clinical improvements noted by physicians and family postoperatively. LEVEL OF EVIDENCE: Level IV.

Outcome analysis of our first 75 spring-assisted surgeries for scaphocephaly.

BACKGROUND: Spring-assisted surgery (SAS) has demonstrated promising results for the treatment of sagittal craniosynostosis. The purpose of this study was to assess the outcomes of the first 75 cases compared with a prospectively collected group of patients treated with cranial expansion (cranial vault remodeling [CVR]). METHODS: Seventy-five children with scaphocephaly have completed this institutional review board-approved study. Patients underwent cranial surgery with removal of a 1-cm strip of sagittal suture and insertion of a mean of 2 spring distractors. Clinical outcome assessment included analysis of changes in cephalic index, shape, and volume on three-dimensional laser scans for both the SAS and the CVR treatments. Perioperative variables for both techniques were also compared. RESULTS: All patients successfully underwent SAS without significant complications with a mean follow-up of 46 months. Perioperative variables including odds ratio, time, blood loss, transfusion requirements, intensive care unit and hospital stay lengths, and hospital costs differed significantly in favor of SAS. The mean cephalic index improved from 69 preoperatively to 75.4 after SAS, comparable with the change from 66 to 72.5 for CVR. This correction was maintained at 3- and 5-year follow-ups. Anterior frontal bossing was corrected on three-dimensional scan volume measurements. CONCLUSIONS: Spring-assisted surgery is a safe, effective, minimally invasive treatment of scaphocephaly. It combines the low morbidity and the operative time of a strip craniectomy with dynamic reshaping techniques while the implanted spring gradually distracts the skull, improving head shape. Our 7 years of experience has shown that SAS effectively corrected cranial shape including frontal bossing with maintained results over time.

Reduced ratio of afferent to total vascular density in mesial temporal sclerosis.

Mesial temporal sclerosis (MTS) is the most common cause of drug-resistant temporal lobe epilepsy in adults. Despite nearly 2 centuries since the first reports of MTS, relatively little is known about its etiology and pathogenesis. Increasing attention has been directed toward the potential role of vascular abnormalities in MTS. We evaluated the hippocampal microvasculature in 9 MTS cases and 3 non-MTS controls using celloidin tissue sections and markers for total (collagen type IV) and afferent (enzymatic alkaline phosphatase) vessels. Tissue sections were assessed by light microscopy and quantified by threshold analysis of digital images and stereological analysis using the Space Balls probe. Although consistent alterations in the total microvascular density were not found, there was a significant reduction in the density of afferent vessels using both methodologies; these reductions were in areas CA2 and CA3 by image threshold analysis and in area CA3 using stereological measures of the ratio of afferent to total vessels. Increased numbers of string vessels (i.e. remnants of regressing vasculature) were also observed in Ammon's horn, suggesting vascular degeneration in the MTS hippocampus. These findings may help further our understanding of the pathophysiology of MTS.

Spring-assisted surgery-a surgeon's manual for the manufacture and utilization of springs in craniofacial surgery.

BACKGROUND AND PURPOSE: Spring-assisted surgery has been used for the treatment of craniofacial deformities since its 1997 inception in Sweden by Dr Lauritzen (Scand J Plast Reconstr Surg Hand Surg 1998;32:331-338). Initial applications have focused on the treatment of patients with single-suture craniosynostosis. Recently, indications and applications have expanded to include patients with syndromic craniosynostosis, multiple-suture synostosis, and midface hypoplasia. The advancement of spring-assisted surgery in this country has been hindered by the need for patient-specific spring fabrication because few surgeons understand how to make the springs for each application. We will review our spring design and treatment algorithms to facilitate wider use of this innovative treatment modality. METHODS: This is a retrospective institutional review board-approved analysis of the spring design for our first 90 cases of spring-assisted surgery used to treat sagittal synostosis at the North Carolina Center for Cleft and Craniofacial Deformities. Outcome analysis was done to generate a treatment algorithm based on diagnosis, patient age, spring design, number of springs, spring force and expansion, and clinical outcome. RESULTS: Ninety children with sagittal craniosynostosis (64 males, 26 females) were treated during an 8-year period (2001-2009) with spring-assisted surgery. Mean age at treatment was 4.4 months and mean age at spring removal was 8.8 months. Mean number of springs used was 2 (range, 1-3). Mean spring force used in sagittal synostosis was 5.5-9.5 (range) for the anterior spring and 5.5-9.5 (range) for the posterior spring with a mean posttreatment expansion of 6.65 cm. Analysis of the results shows that spring force and expansion required for optimal correction is dependent on the age at surgery, type of the deformity, and severity of the deformity. Specifically, the younger the child, the weaker the spring needed for surgical correction. General principles for spring application for scaphocephaly include (1) the longer the anterior posterior dimension of the skull deformity, the more likely a third spring is necessary; (2) the narrower the posterior occiput, the stronger the posterior spring required; and (3) if a postcoronal band is seen in the calvarium, a stronger anterior spring is needed. CONCLUSIONS: Long-term experience with spring-assisted surgery has facilitated the development of standardized, reproducible techniques allowing spring design modifications to optimize clinical outcome.

Classification system for sagittal craniosynostosis.

Scaphocephaly is an important but incompletely defined entity resulting from premature fusion of the sagittal suture. Our goal was to use the clarity afforded by three-dimensional computed tomographies (CTs) to discern what characteristics of this disease are most representative and singular. Furthermore, we sought to define a classification system wherein the dominant physical trait, as it results from varied sites of premature fusion, determines the type of scaphocephaly in a particular patient. All patients with CT-diagnosed isolated single-suture sagittal craniosynostosis seen by a single craniofacial surgeon and a single pediatric neurosurgeon over a 5-year period are included (N = 76). Patients were stratified into anterior, central, posterior, or complex subtypes dependent upon the presence of a single dominant characteristic as seen on CT scan. Anterior type features a transverse retrocoronal band; central type has a heaped sagittal ridge; posterior type has an especially prominent occiput; and complex type includes those patients in whom a single dominant feature is not present. Forty-eight patients fit into anterior, central, or posterior types. Thirteen percent (N = 7) lack a single dominant feature and are complex. Incidence and prevalence for each type in this population are the following: anterior, 13/24%; central, 16/29%; posterior, 19/35%; and complex, 7/13%. Heterogeneous points of initial fusion may be responsible for the differing representations. Clinical applications using this classification system are ongoing and may allow us to individualize surgical intervention to optimize clinical outcome.

Surgical treatment for scaphocephaly and a calcified cephalohematoma.

Craniosynostosis is the premature fusion of 1 or more of the cranial sutures, with sagittal synostosis being the most common nonsyndromic single suture synostosis. The pathogenesis of craniosynostosis has been extensively studied and is likely multi-factorial. A complex interaction between the dura and overlying suture via multiple growth factors seems to play the most important role. There have been 3 published studies with patients presenting with scaphocephaly and a cephalohematoma, which raises the question of how the 2 conditions may be related. Cephalohematomas can be seen after trauma and a number of other causative factors but usually resorb over time without sequela. In a small percentage of cases, the hematoma persists and calcifies, leading to significant asymmetry and deformity of the skull. Once it reaches this point, surgical intervention may be required to correct the resulting skull deformity. We present a child with scaphocephaly and a cephalohematoma who underwent surgical correction with resection of the cephalohematoma and sagittal suturectomy with spring-assisted surgery.

Clinical experience with radiation therapy in the management of neurofibromatosis-associated central nervous system tumors.

PURPOSE: Patients with neurofibromatosis (NF) develop tumors of the central nervous system (CNS). Radiation therapy (RT) is used to treat these lesions. To better define the efficacy of RT in these patients, we reviewed our 20-year experience. METHODS AND MATERIALS: Eighteen patients with NF with CNS tumors were treated from 1986 to 2007. Median follow-up was 48 months. Progression was defined as growth or recurrence of an irradiated tumor on serial imaging. Progression-free survival (PFS) was measured from the date of RT completion to the date of last follow-up imaging study. Actuarial rates of overall survival (OS) and PFS were calculated according to the Kaplan-Meier method. RESULTS: Eighty-two tumors in 18 patients were irradiated, with an average of five tumors/patient. Median age at treatment was 25 years (range, 4.3-64 years). Tumor types included acoustic neuroma (16%), ependymoma (6%), low-grade glioma (11%), meningioma (60%), and schwanomma/neurofibroma (7%). The most common indication for treatment was growth on serial imaging. Most patients (67%) received stereotactic radiosurgery (median dose, 1,200 cGy; range, 1,000-2,400 cGy). The OS rate at 5 years was 94%. Five-year PFS rates were 75% (acoustic neuroma), 100% (ependymoma), 75% (low-grade glioma), 86% (meningioma), and 100% (schwanomma/neurofibroma). Thirteen acoustic neuromas had a local control rate of 94% with a 50% hearing preservation rate. CONCLUSIONS: RT provided local control, OS, and PFS rates similar to or better than published data for tumors in non-NF patients. Radiation therapy should be considered in NF patients with imaging progression of CNS tumors.

Transfusion-related acute lung injury in an infant during craniofacial surgery.

UNLABELLED: Transfusion-related acute lung injury (TRALI) is a potentially life-threatening, systemic, immune-mediated reaction to transfused blood product. The symptoms may be masked under general anesthesia. In this case report, we describe an infant who developed TRALI under general anesthesia for craniofacial surgery. The difficulty with diagnosis, the pathophysiology, and the need for understanding and recognition to reduce morbidity and mortality are discussed. IMPLICATIONS: Transfusion-related acute lung injury (TRALI) is a life-threatening problem that can occur during blood product transfusion in patients of any age. Understanding the pathophysiology may help make an earlier diagnosis to reduce more serious adverse outcomes.

Surgical advancement influences perioperative care: a comparison of two surgical techniques for sagittal craniosynostosis repair.

Methods for surgical correction of sagittal craniosynostosis have progressed. The hypothesis is that advances in surgical interventions for craniosynostosis affect perioperative anesthetic care. We reviewed the records of eight children who underwent cranial vault reconstruction (CVR) and nine who underwent spring-mediated cranial expansion (SME) for sagittal craniosynostosis. We compared the data from the CVR procedure to data from the combined procedures for SME (insertion and removal of springs). Anesthesia times were similar between the CVR (4 h 24 min) and the combined SME (4 h 27 min) groups, whereas surgical times were different between the CVR (3 h 25 min) and combined SME groups (2 h 21 min) (P = 0.002). Length of stay was 4.1 days for the CVR group (confidence interval [CI], 3.8-4.4 days) versus 3.1 days (CI, 2.9-3.4 days) in the combined SME group (P = 0.0001). Blood loss was significantly less in the combined SME group at 48 mL (CI, 29-83 mL) compared with the CVR group at 291 mL (CI, 230-352 mL). All eight patients in the CVR group received blood with a mean of 1.4 U (range, 1-2 U). No SME patient received any blood products. The reduction in blood loss with this new surgical treatment is significant for the patient in reducing blood transfusion and for the anesthesiologist in reducing concerns of volume resuscitation.

Surgical management of foramina parietalia permagna.

Enlarged parietal foramina are rare congenital skull defects identified on physical examination and confirmed radiographically. They are round or oval defects situated on each parietal bone approximately 1 cm from the midline and 2 to 3 cm superior to the lambdoid suture. Although small parietal foramina are common variants in up to 60% to 70% of normal skulls, large parietal foramina ranging from 5 mm to multiple centimeters are less common, with a prevalence of 1:15,000 to 1:25,000. We present a case series of four patients with large persistent parietal foramina managed surgically for the correction of this deformity. Two infants were treated with autologous calvarial bone grafts, and two were treated with a mesh plating system and hydroxyapatite. No patient developed any perioperative complications. No perioperative or delayed infections occurred in our patient population. The mean postoperative follow-up was 36 months. One patient required a second procedure with methylmethacrylate because of late bone graft failure, whereas the others were successfully treated by the initial procedure. Foramina parietalia permagna, otherwise known as fenestrae parietals symmetricae, enlarged parietal foramina, giant parietal foramina, or Catlin marks, are a rare clinical entity. A spontaneous decrease in the size of these defects with growth of the infant has been reported, but this closure is usually incomplete. Surgical intervention of persistent large foramina protects the child against potential injury to the underlying brain. We advocate cranioplasty for active young children and those at risk for injury (i.e., seizure disorder) to decrease the risk for potential intracranial injury.

Double myelomeningocele: case report and review.

The presence of a myelomeningocele at multiple levels along the spinal column is a rare event. There are only a few cases of double myelomeningocele reported in the world's literature. Large myelomeningoceles present surgical closure challenges due to the size and location of these defects. We report the case of a newborn female who had two myelomeningoceles with one at the thoracic level and the other at the lumbar level. Surgical closure must provide durable and stable coverage for the spinal cord. Stable coverage was provided for this patient using bilateral paraspinous myofascial turnover flaps. This unusual case report as well as a review of the literature is presented.