Outcome analysis for correction of single suture craniosynostosis using resorbable fixation.

A retrospective review was performed on 63 patients at Childrens Healthcare of Atlanta at Scottish Rite who underwent correction of single-suture craniosynostosis using a resorbable fixation system. Included in the series were 24 patients with metopic synostosis, 15 with sagittal synostosis, and 24 with unicoronal synostosis. The average age at operation was 22.7 months (range: 2.8 months-18 years), and mean follow-up time was 30.7 months (range: 7.1-10 years). Reoperation equal to or exceeding the magnitude of the original procedure occurred in 4.76% of the patients. This was comparable to the reoperation rate observed at our institution using traditional fixation systems. Minor complications related to the use of resorbable plates were also identified, and the final outcome for single-suture synostosis was favorable. Results suggest that resorbable plates and screws are as effective as titanium-based systems in the treatment of single-suture synostosis.

Posthemorrhagic hydrocephalus of infancy.

For a variety of anatomic and physiologic reasons, premature infants, especially those of very low birth weight, are most likely to sustain an intraventricular hemorrhage (IVH) and subsequent posthemorrhagic hydrocephalus (PHH). When to initiate treatment and what that treatment should be remains controversial. This article discusses the pathophysiology and clinical presentation of IVH and PHH and provides a guideline for when and how to treat hydrocephalus in this population. Fibrinolytic therapy and neurodevelopmental outcomes are reviewed.

Chiari malformation, cervical spine anomalies, and neurologic deficits in velocardiofacial syndrome.

The purpose of this investigation was to evaluate the prevalence of Chiari malformation, cervical spine anomalies, and neurologic deficits in patients with velocardio-facial syndrome. This study was a prospective evaluation of 41 consecutive patients with velocardiofacial syndrome, documented by fluorescence in situ hybridization, between March of 1994 and September of 1998. The 23 girls and 18 boys ranged in age from 0.5 to 15.2 years, with a mean age of 6.7 years. Nineteen patients were assessed with magnetic resonance imaging, 39 underwent lateral cephalometric radiography, and all patients were examined for neurologic deficits. Eight of 19 patients (42 percent) had anomalies of the craniovertebral junction, including Chiari type I malformations (n = 4), occipitalization of the atlas (n = 3), and narrowing of the foramen magnum (n = 1). One patient with Chiari malformation required suboccipital craniectomy with laminectomy and decompression. Fourteen of 41 patients (34 percent) had demonstrated neurologic deficits; 10 patients (24 percent) had velar paresis (6 unilateral and 4 bilateral). Chiari malformations, cervical spine anomalies, and neurologic deficits are common in velocardiofacial syndrome. Because these findings may influence the outcome of surgical intervention, routine assessment of patients with velocardiofacial syndrome should include careful orofacial examination, lateral cephalometric radiography, and magnetic resonance imaging of the craniovertebral junction.

The use of hydroxyapatite cement in secondary craniofacial reconstruction.

Sixty-one patients underwent secondary craniofacial reconstruction for contour defects using hydroxyapatite cement over a 3-year period (20-month mean follow-up). There were 56 children, aged 2.2 to 18 years (mean, 10.7 years), 21 boys and 35 girls. This is the first series of pediatric patients in whom the use of hydroxyapatite cement has been reported. There were five adults aged 21 to 46 years (mean, 32 years), 3 men and 2 women. Thirty-one patients underwent reconstruction for secondary orbitocranial defects after surgery for synostosis, 7 after surgery for hypertelorism, 10 for posttraumatic skull defects, and 13 for a variety of other facial skeletal defects. There were seven complications (11 percent), ranging from a retained drain to postoperative seromas, all of which required reoperation without loss of the contour correction. All of the complications occurred in the first 18 months of our study. There has been excellent retention of implant volume with no recurrence of contour defects to date. We have not found any visible evidence of interference with craniofacial growth over the study period. We conclude that hydroxyapatite cement is a versatile and safe biomaterial when used for the correction of secondary craniofacial contour defects in children and adults. The coupling of antibiotics with this biomaterial may have applications in the treatment of osteomyelitis.

Monobloc and facial bipartition distraction with internal devices.

Distraction osteogenesis (DO) permits gradual lengthening of the craniofacial skeleton. With the advent of new internal devices, monobloc (M) and facial bipartition (FB) DO are feasible. The rationale behind M and FB distraction is (1) gradual advancement of the M segment is not associated with a substantial retrofrontal dead space; (2) because 5 to 7 days elapse prior to distraction, the nasofrontal opening, in theory, is allowed to remucosalize; (3) gradual expansion of the soft tissues takes advantage of skin creep, potentially limiting relapse; (4) the procedure appears to be less invasive with decreased blood loss and operative time, enabling its use in infants; (5) overdistraction may eliminate or reduce the frequency of subsequent procedures; and (6) the procedure may be combined with FB and skull vault remodeling to provide excellent results in more complex craniofacial dysostosis problems. Five children underwent M advancement (N = 3) and M with FB (N = 2) at 9 months to 5 years of age to correct functional abnormalities such as corneal exposure, increased intracranial pressure, and apnea, as well as severe craniofacial disfigurement. Each patient underwent from 22 to 30 mm of distraction with the Modular Internal Distraction (MID) system, developed by the first author (SRC). There was one infection late in the series along the DO cable track. There were no cases of epidural abscess. In conclusion, MDO, with and without FB, appears to be a safe and effective technique for transcranial frontofacial advancement. The morbidity of the procedure appears to be less than that of conventional M advancement.

Neonatal brain tumors: a review.

This article presents two cases of infants with brain tumors and reviews the literature pertinent to congenital and neonatal brain tumors. Information regarding epidemiology, presentation, prognosis, and clinical management are also addressed with specific regard to differences between neonatal and childhood brain tumors. An appeal is made to consider (1) coordination of the care of these children through pediatric multidisciplinary neuro-oncology programs; (2) enrollment, whenever possible, in clinical trials; and (3) submission of available tumor tissue to pediatric tumor banks to assure its availability to interested researchers.

Paroxysmal rage as a presenting symptom of the Chiari I malformation. Report of two cases.

Signs of meningeal irritation including occipital and cervical pain are common in symptomatic children with the Chiari I malformation. The author reports on two children with Chiari I malformations who presented with a previously undescribed symptom presumably caused by intermittent meningeal irritation: paroxysmal rages. In both cases the rages stopped after decompressive surgery. Evaluation including magnetic resonance imaging should be considered in children with new onset of paroxysmal behavioral disorders.

Conformations of Gly(n)H+ and Ala(n)H+ peptides in the gas phase.

High-resolution ion mobility measurements and molecular dynamics simulations have been used to probe the conformations of protonated polyglycine and polyalanine (Gly(n)H and Ala(n)H+, n = 3-20) in the gas phase. The measured collision integrals for both the polyglycine and the polyalanine peptides are consistent with a self-solvated globule conformation, where the peptide chain wraps around and solvates the charge located on the terminal amine. The conformations of the small peptides are governed entirely by self-solvation, whereas the larger ones have additional backbone hydrogen bonds. Helical conformations, which are stable for neutral Alan peptides, were not observed in the experiments. Molecular dynamics simulations for Ala(n)H+ peptides suggest that the charge destabilizes the helix, although several of the low energy conformations found in the simulations for the larger Ala(n)H+ peptides have small helical regions.

Shunt revision by coagulation with retention of the ventricular catheter.

Intraluminal coagulation of a ventricular catheter followed by its removal and replacement is a technique used for proximal shunt revision. The authors describe experience leaving the original catheter in place when flow of CSF is established by the coagulation. Advantages of this modification include maintenance of ventricular access when the ventricles are small and the possibility of reducing the incidence of subsequent shunt obstruction by mitigating intraventricular hemorrhage caused by catheter removal.

Spinal intradural cerebellar ectopia.

An ectopic cerebellum, as in Chiari malformations and ectopic cerebellar dysplastic tissue, is a common finding; however, the presence of an organized ectopic cerebellum is exceedingly rare. We describe the MR imaging, surgical, and histologic appearance of an intraspinal ectopic cerebellum in an infant.

Multiple suture synostosis and increased intracranial pressure following repair of single suture, nonsyndromal craniosynostosis.

OBJECTIVE: Increased intracranial pressure, frequently associated with closure of multiple cranial sutures, has been reported to occur in 36% of cases following correction of syndromal craniosynostosis. Although much less common, multiple suture closure may occur following repair of single suture, nonsyndromal craniosynostosis and we present cases that concern two such children. RESULTS: Two children with nonsyndromal craniosynostosis, one metopic and one left-coronal, underwent fronto-orbital advancement at age 3 months. At age 19 months and at age 5 years, respectively, both patients re-presented with headaches, decrease in head circumference percentile, and acceptable cosmetic outcome. Both had computerized tomographic evidence of multiple closed cranial sutures and increased intracranial pressure (ICP) (determined by monitoring). Both patients improved following a cranial expansion procedure. CONCLUSION: Delayed closure of multiple sutures and resultant increased ICP may occur following correction of nonsyndromal, single suture craniosynostosis. This may be more likely when the initial suture is contiguous with the facial sutures. Children should be followed for many years following craniosynostosis repair with cranial, neurologic, and possibly funduscopic examinations as well as head circumference measurements to detect delayed closure of cranial sutures.

Treatment of posthemorrhagic hydrocephalus in the preterm infant with a ventricular access device.

Intraventricular hemorrhage (IVH) and subsequent posthemorrhagic hydrocephalus (PHH) commonly complicate the course of extremely preterm infants. Many methods for treating the hydrocephalus have been used, none of which are ideal. We present the largest series of infants with PHH treated with one modality, the ventricular access device (VAD). One hundred and forty-nine preterm infants with PHH were treated by placement of a VAD and serial taps to control intracranial pressure and ventricular size. Variables recorded include gender, race, gestational age, weight at birth, IVH grade, incidence of VAD infection, malfunction or local wound problems and indwelling time to either shunt placement or VAD removal. Of the 149 preterm infants, 91 were males and 58 females. The average birth weight was 994 g and the average gestational age at birth was 26.3 weeks. Three infants were IVH grade 1, 8 were grade 2, 62 were grade 3 and 76 were grade 4. VAD occlusion occurred in 15 infants (10%). Nine infants required contralateral VAD placement for a trapped ventricle. VAD infection occurred in 12 infants (8%), 5 of whom were treated successfully with a combination of systemic and intra-VAD antibiotics without removal of the VAD. The total rate of revision was thus 20% (15 for occlusion, 9 for trapped ventricle, 7 for infection). Wound problems were minimal and consisted of 4 cerebrospinal fluid leaks and 14 subgaleal fluid collections. For the 133 survivors, the rate of shunt placement was 88%. The VAD, while not ideal, is an excellent treatment at this time for PHH. It can be utilized for several months with acceptable rates of infection, blockage and wound complications. The VAD tap is simple to perform, not disruptive to minimal stimulation protocols, and can be done by physician extenders. In addition, medications can be administered via the access device, thus allowing treatment of some infections without VAD removal as well as instillation of thrombolytic agents such as urokinase.

The use of porous granular hydroxyapatite in secondary orbitocranial reconstruction.

The search for the ideal bone-graft substitute has been the focus of many research and clinical studies. Hydroxyapatite is one such material that combines osseointegration with maintenance of implant volume and excellent durability. We present our experience in 29 patients ranging in age from 3 to 22 years (mean age 10.5 years) who underwent secondary orbitocranial reconstruction of large contour defects utilizing porous granular hydroxyapatite. Follow-up ranges from 6 to 72 months (mean 30 months). Indications for secondary surgery included residual bony contour defects of the frontal bone, temporal areas, and superior orbital rims that were present 12 months or more after initial surgery. There was one infection secondary to a chronic seroma necessitating removal of the porous hydroxyapatite, and one patient required revision for underfilling and another for overfilling. Excellent permanent contour improvement was obtained with a smooth skin surface in the remainder of our patients. The contour corrections have been long lasting, without evidence of porous hydroxyapatite resorption or migration.

A longitudinal, statistical study of reoperation rates in craniosynostosis.

A prospective, statistical study of reoperation rates was done in the treatment of 167 consecutive children with nonsyndromic and syndromic craniosynostosis over a 6-year period at Scottish Rite Children's Medical Center in Atlanta, Georgia. Mean length of follow-up was 2.8 years, with a range of 3 months to 6 years. Reoperation equal to or exceeding the magnitude of the original procedure occurred in 7 percent of cases. Multiple regression analysis revealed several factors associated with reoperation: Females and children with syndromic synostoses were more likely to require reoperation. Total reoperation rates for syndromic and nonsyndromic synostoses were 27.3 and 5.9 percent, respectively. Age at initial surgery, length of operation, and estimated blood loss did not predict a higher reoperation rate.

Intrathecal urokinase as a treatment for intraventricular hemorrhage in the preterm infant.

Despite improvements in the care of preterm infants, intraventricular hemorrhage (IVH) and posthemorrhagic hydrocephalus (PHH) continue to be frequent occurrences in this patient population. Shunt procedures in these children are frequently complicated by obstruction and/or infection. As the hydrocephalus is usually caused by an obliterative arachnoiditis due to contact of the blood with the basilar meninges, it was postulated that infusion of urokinase into the ventricles of infants who have sustained an IVH would clear the blood, mitigate the arachnoiditis, and prevent the progression of PHH. Accordingly, 18 preterm infants who had sustained IVH and subsequently developed PHH were treated with intraventricular urokinase instilled via a surgically implanted subcutaneous reservoir. There were no complications associated with the urokinase. Infants were divided into two dosage groups: low dose (110,000-140,000 IU total) and high dose (280,000 IU total). One infant in the low-dose group died at 1 month of life of respiratory complications. In the low-dose group, 3 of 8 (37%) infants required shunt placement; in the high-dose group, all 9 required shunt placement. For the total group, the shunt rate was 71%. This compares to a historical control group shunt rate of 92%. While the difference between the treatment group as a whole and control group approaches, but does not reach, statistical significance (p = 0.068), there was a significant reduction in the shunt rate when the low-dose group was considered separately (p < 0.002). For those infants that required shunt placement, there were fewer shunt revisions performed in the treatment group than in the control group during the first 24 months following shunt placement: 0.67 versus 1.5 shunt revisions/shunted child. Initial experience with intraventricular urokinase following IVH and PHH in preterm infants suggests a beneficial effect in reducing the shunt revision rate in both high- and low-dose groups. Reduction in shunt placement rate is seen only in the low-dose group.

Frontal basilar trauma: classification and treatment.

We report our experience with 14 consecutive cases of frontal basilar trauma occurring in children and adolescents aged 18 months to 18 years (mean 9.5 years). Brain parenchymal injury resulting in functional deficit occurred in 5 patients (36 percent), 2 patients suffered bilateral blindness, and 1 suffered unilateral loss of vision. A classification system and treatment algorithm based on the clinical fracture pattern seen by computed tomography are introduced. Type I, central, is confined to the upper nasoethmoidal complex, central frontal bone, and medial third of the superior orbital rims. Type II, unilateral, involves the entire supraorbital rim and the upper lateral orbital wall, extending into the squamosa of the temporal bone and ipsilateral frontal bone. Type III, bilateral, involves fractures of the upper nasal ethmoidal complex, bilateral supraorbital and upper lateral orbital wall fractures, and bilateral frontal bone fractures. This classification was utilized to plan elective orbital and cranial osteotomies, similar to those used for frontal orbital advancement at the time of acute fracture repair. Frontal orbital osteotomies were used to access the anterior cranial fossa, orbital apices, and nasofrontal ducts and to obtain an intact bony template for side-table reassembly of the fracture fragments. There was no significant operative morbidity, one late cerebrospinal fluid leak, and no infections. Reoperation was necessary in four patients (29 percent) for aesthetic indications.

Geniculate ganglion meningioma.

Primary ectopic meningiomas are rare, but may be seen in the head and neck region. The temporal bone and its neural foramen are rarely the site of a primary meningioma. This report describes the CT and MRI appearance of an ectopic meningioma arising at the anatomic location of the geniculate ganglion, and discusses the differential diagnosis as well as the possible origin of the tumor.