William R. Boydston

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.

A longitudinal, statistical study of reoperation rates in craniosynostosis

&NA; 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 Childrens 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. (Plast. Reconstr. Surg. 100: 305, 1997.)

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.

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.

Gliomas of the Tectum and Periaqueductal Region of the Mesencephalon

Gliomas that arise in the tectal and periaqueductal region of the mesencephalon usually present with hydrocephalus secondary to occlusion of the aqueduct of Sylvius. A review of 486 brain tumors in children treated during a 5-year period revealed 6 children with gliomas of the tectal plate. The 6 children were shunted for hydrocephalus, presumed secondary to aqueductal stenosis, prior to establishing the diagnosis of tectal plate glioma. No abnormalities were noted on the initial, uncontrasted computed tomography (CT) scans. The tumors are isodense without contrast enhancement which makes the CT diagnosis difficult. Magnetic resonance imaging (MRI) is diagnostic and demonstrates the characteristic enlargement of the tectum with increased density on T2 images. T1 density and gadolinium enhancement are variable. Pathological confirmation was obtained by open biopsy in 2 patients, a stereotaxic biopsy was performed on 2 children; 2 children were not biopsied. The tumor histology obtained was that of pilocytic astrocytoma. Two patients were treated with radiation therapy at the time of diagnosis. One child was followed closely and subsequently irradiated after tumor progression. All patients in this series are alive and functioning adequately 2-10 years after the onset of symptoms.

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

&NA; The purpose of this investigation was to evaluate the prevalence of Chiari malformation, cervical spine anomalies, and neurologic deficits in patients with velocardiofacial 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. (Plast. Reconstr. Surg. 106: 16, 2000.)

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.

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.

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.

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.