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.

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.

Intralesional laser therapy of extensive hemangiomas in 100 consecutive pediatric patients.

The authors have treated 100 consecutive pediatric patients with capillary/cavernous hemangiomas (age range, 1.3 months to 16 years; mean age, 26.6 months; 30 male and 70 female patients) with intralesional laser therapy during a 3-year period. All patients have been followed for a minimum of 6 months after treatment (range, 6-36 months; mean, 18 months). Indications for intralesional laser treatment included interference with vision, blockage of the nose or mouth, ulceration, bleeding, and rapid, uncontrollable growth. The Nd:YAG laser was used in 70 patients, and the Potassium, Titanyl, Phosphate (KTP) laser was used in 30 patients. Fifty-five hemangiomas were in the head and neck region, excluding the orbit; 25 were in the trunk or extremities; 10 were periorbital; and 10 involved multiple sites. Seventy patients (70%) received one treatment, 20 patients (20%) received two treatments, 7 patients (7%) received three treatments, and 3 patients (3%) received four or more treatments. No appreciable differences were noted between treatment with the Nd:YAG and KTP lasers. Forty-six patients had more than a 90% reduction in the overall size of the hemangiomas whereas 54 patients had a 50% to 90% reduction in the size of the hemangioma. After maximal reduction in size of the cavernous component was achieved, the external capillary component, found in 68 patients, was treated with a tunable dye laser. Seventy-six patients underwent surgical resection after maximal lesion involution. Residual induration due to lesion fibrosis was treated with local steroid injections in 13 patients. There were four operative complications attributable to intralesional laser therapy. Two patients had residual midfacial weakness, and two patients had punctuated skin burns after intralesional treatment. The authors have found intralesional laser therapy to be a valuable tool in the treatment of large capillary/cavernous hemangiomas, often rendering an inoperable lesion safely resectable, or markedly decreasing the size and functional impact of the lesion.

Bone substitutes.

The search for the ideal bone substitute began hundreds of years ago, and continues today. While numerous choices have been proposed and tested, with varying degrees of success, there remain many challenges related to the use of bone substitutes in craniofacial reconstruction. This paper presents a review of the history of bone substitute research, a discussion of currently popular materials, and elucidation of the challenges to be faced as we approach the new millennium.

Iliac crest bone graft harvesting techniques: a comparison.

This study was undertaken to compare the morbidity of traditional iliac bone graft harvesting techniques for grafting alveolar clefts to minimally invasive techniques. Fifty-five age-matched patients, ages 6.5 to 16 years (mean, 11.2 years), 22 girls and 33 boys, were divided into three groups. The traditional bone window open harvesting technique served as the control group. Two different minimally invasive techniques, one that used a bone grinder and another that used a trephine, for bone harvesting were compared with the control. Both invasive techniques were statistically superior, p < 0.05, in terms of total time pain medication was necessary (mean of 12.0 hours for bone grinder, 17.6 hours for trephine, 26.0 hours for control), operative time for bone harvest (mean of 11 minutes for bone grinder and trephine, 20 minute for control), and mean incision length (2 cm for bone grinder and trephine, 5 cm for control). Patients exposed to the minimally invasive techniques had fewer complications, a trend toward earlier ambulation, and shorter hospital stays when compared with the bone grinder technique. The patients exposed to the bone grinder demonstrated earlier ambulation and fewer requirements for analgesia when compared with the trephine technique, although these results did not reach statistical significance. The trephine technique was useful when maxillary osteotomies were combined with alveolar bone grafting, because it provided structural bone grafts and cancellous bone. On the basis of these findings, the bone grinder is the preferred technique for harvesting alveolar bone grafts when no structural support is required. These authors no longer use the traditional bone window open harvesting technique.

An anatomic evaluation of the furlow double opposing Z-plasty technique of cleft palate repair.

The aim of this investigation was to examine velar anatomy following the Furlow double opposing Z-plasty in order to analyse the theoretical effects of this technique. Thirty patients with cleft lip and/or cleft palate who underwent primary Furlow palatoplasties between 1989 and 1994 were reviewed. The mean age at the time of surgery was 6.4 months. Evaluation was performed at a mean time of 2.9 years postoperatively, and consisted of oral examination of the position of the velar dimple and measurements of velar dimensions from standard lateral cephalograms. A comparative statistical analysis of velar length (n = 17) and thickness (n = 14) was performed using 2 historical control groups (non-cleft norms and non-Furlow cleft palate repairs). The Furlow procedure produced posterior dimples in 19 of 26 patients adequately rated on oral examination, suggesting successful repositioning of the velar musculature in transverse orientation. The mean velar length was not significantly different from that of norms (being 0.72 mm less), suggesting that the Furlow Z-plasty results in the attainment of near normal velar length. In contrast, the mean velar length was 0.46 mm greater compared to non-Furlow repairs. Although this difference was not statistically significant, it suggests that the Furlow Z-plasty may be more effective in increasing velar length compared to non-Furlow palatoplasty techniques. Velar thickness was significantly greater compared to both norms (P = 0.002) and non-Furlow repairs (P = 0.001). These data suggest that the Furlow double opposing Z-plasty repositions the velar muscles in transverse orientation, and increases both velar length and thickness, lending weight to the theoretical effects of this procedure. The anatomic basis of these changes and their functional implications are discussed.

The role of distraction osteogenesis in the management of craniofacial disorders.

Since its introduction in the medical literature in 1992 by McCarthy, distraction osteogenesis of the craniofacial skeleton has become a standard surgical therapy. The present report attempts to trace the development of craniofacial distraction from the perspective of one of the early proponents of the technique. Although the earliest application of distraction was in children with severe unilateral or bilateral mandibular deficiency, its use for functional abnormalities such as apnoea were especially appealing. Distraction osteogenesis for the midface began with external appliances that were attached to the teeth. Newer, buried devices have eliminated the need for external devices in all LeFort III and monobloc cases. For LeFort I and mandibular cases, the ideal internal device has not been manufactured.

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.

Alternatives to tracheostomy in infants and children with obstructive sleep apnea.

PURPOSE: Since 1989, 70 children have been treated surgically with varying degrees of obstructive sleep apnea (OSA). Of these, 29 patients had completely failed conventional medical and surgical treatment and were considered tracheostomy (T) candidates, whereas five had previously undergone T for severe OSA as infants and did not respond to standard decannulation protocols. The preoperative diagnoses were cerebral palsy (n = 5), Down's syndrome (n = 5), hemifacial microsomia (n = 4), Pierre Robin sequence (n = 6), and a mixed group of craniofacial disorders (n = 14). The patients ranged in age from 2.5 weeks to > or =18 months (mean, 7.32 years). Preoperatively, four patients were on ventilators and one suffered a cardiac arrest, attesting to the severity of OSA. METHODS: To enlarge the caliber of the airway, each patient underwent an aggressive surgical treatment protocol. All sites of upper airway obstruction were treated simultaneously by a combination of craniofacial skeletal expansion and soft-tissue reduction. RESULTS: Tracheostomy was avoided in 90.4% of patients. Temporary or "permanent T" were required in three patients (9.6%). One patient with cerebral palsy had recurrent OSA and died. A second patient with severe laryngotracheomalacia and retrognathia who did not respond to apnea surgery underwent a tracheostomy and ultimately died of pulmonary causes. Four patients (7.8%) required supplemental home oxygen or continuous positive airway pressure. The average preoperative respiratory disturbance index, defined as the average number of apneic and hypopneic events per hour of sleep, dropped from 25.9 to 4.4 after surgery. The average lowest recorded oxygen saturation during overnight polysomnography rose from 61% to 92% after surgery. Of the five patients with permanent T, four had thus far been decannulated. Complications occurred in 10 patients, 50% of which were related to minor problems with mandibular distraction devices. CONCLUSION: Our results confirm the efficacy of an aggressive surgical approach to the treatment of OSA in children, avoiding the necessity for tracheostomy or permitting decannulation of permanent T in the majority of cases.

Airway disorders in hemifacial microsomia.

The anatomic components of hemifacial microsomia have been classified previously, but their relevance to functional abnormalities has not been stressed. In a recent review of the literature, we found that the frequency and severity of airway disorders, especially those leading to upper airway obstruction and/or obstructive sleep apnea, had not been reported. Accordingly, a retrospective study of 38 patients (21 male, 17 female), aged 6 months to 22 years (mean, 8 years 3 months) with hemifacial microsomia was undertaken to ascertain the frequency and severity of airway disorders in hemifacial microsomia. Upper airway disorders fell broadly within three categories: group I (n = 22, 58 percent) was asymptomatic for airway disturbances; group II (n = 7, 18 percent) had a medical history suspect for intermittent obstructive sleep apnea or had a perioperative apneic event; and group III (n = 9, 24 percent) had a definite history of obstructive sleep apnea or upper airway obstruction requiring tracheotomy or apnea surgery. Group III versus groups II and I had a higher incidence of bilateral involvement (33 percent versus 14 percent and none), a greater percentage of M2, M2a, M2b, and M3 mandibular deformities (88.9 percent versus 28.6 percent and 18.2 percent), more severe orbital involvement (33 percent O2 and O3 versus none in group II and 9 percent in group I), and more severe soft-tissue involvement (89 percent S2 and S3 versus 29 percent and 23 percent). Patients with more severe mandibular and orbital deformities, but not ear or vertebral abnormalities, appear at a greater risk for obstructive sleep apnea. The relationship of OMENS-Plus (extracraniofacial anomalies) to apnea was variable but was found more commonly in group II (86 percent) and group III (56 percent) than in group I (32 percent) patients. Group III patients had a higher frequency of cardiac anomalies (44 percent versus 29 percent in group II and 23 percent in group I). The incidence of obstructive sleep apnea in our population of patients with hemifacial microsomia approaches 24 percent. Patients with hemifacial microsomia should undergo routine screening for obstructive sleep apnea: a positive history warrants polysomnographic and anatomic workup.

Skeletal expansion combined with soft-tissue reduction in the treatment of obstructive sleep apnea in children: physiologic results.

Twenty consecutive children, ranging in age from 6 days to 18 years, were treated with skeletal expansion, in addition to soft-tissue reduction, for medically refractory obstructive sleep apnea. The underlying diagnoses were craniofacial microsomia (n = 6), Down syndrome (n = 3), Pierre Robin syndrome (n = 3), cerebral palsy (n = 3), Nager's syndrome (n = 1), Treacher Collins syndrome (n = 1), cri du chat syndrome (n = 1), juvenile rheumatoid arthritis (n = 1), and temporomandibular joint ankylosis (n = 1). Fourteen children had severe medically refractory sleep apnea and were tracheostomy candidates; in the remaining six, tracheostomies were placed shortly after birth and could not be decannulated. Overnight, 12-channel polysomnography was obtained before and after surgery. The mean apnea index improved from 7.42 to 1.26, the mean respiratory disturbance index improved from 25.24 to 1.72, and the mean lowest apnea-related oxygen saturation improved from 68% to 88%. Of the 14 children with medically refractory obstructive sleep apnea, two required tracheostomies. Of the six patients with tracheostomies, five have been decannulated at the time of this writing. Skeletal expansion in conjunction with soft-tissue reduction in the pediatric population permits substantial increases in the volume of both the nasopharynx and oropharynx. Creative use of conventional osteotomies and the application of distraction osteogenesis have enabled surgeons to apply maxillofacial and craniofacial techniques in treating children with obstructive sleep apnea.

Sleep apnea surgery versus tracheostomy in children: an exploratory study of the comparative effects on quality of life.

Successful surgery for medically refractory obstructive sleep apnea in children has prevented tracheostomy in many cases. However, sleep apnea surgery requires postoperative ventilatory support and intensive care, and the magnitude of the surgery may be substantial. Tracheostomy, in contrast, is a simple procedure that is considered the standard of care for relief of upper airway obstruction. To determine their relative benefits, the posttreatment quality of life in children with airway obstruction who underwent either sleep apnea surgery or tracheostomy was evaluated and compared in this exploratory study. A 76-item questionnaire was developed to assess the quality of life in this population, including an investigation of physical symptoms, psychosocial function, and costs. Forty-four parent questionnaires were returned; 16 of these parents had children who had had clinically successful sleep apnea surgery and 6 had children who had had tracheostomies placed for obstructive sleep apnea. Results revealed that the parents of children in the tracheostomy group ranked 95 percent of all items on the questionnaire as worse than the parents of children in the surgery group. These rankings included statistically significant group differences (p < 0.05) on number of hospital, emergency room, and physician visits, and hours per day spent on their child's respiratory care. In addition, parents of the successful sleep apnea surgery group reported significant improvement (p < 0.05) in 100 percent of symptom variables (i.e., choking, snoring, and daytime sleepiness), 75 percent of parental care variables (i.e., assisting with their child's breathing, suctioning), 67 percent of medical visit items, and 75 percent of the stress and coping variables (i.e., perception of child's distress, worrying about their child's breathing, level of family stress), indicating substantial gains in quality of life. Despite initially higher costs, successful surgery for obstructive sleep apnea was associated with substantial benefits in quality of life, health, and psychosocial outcomes when compared with tracheostomy.

Applications of endoscopic surgery in pediatric patients.

Although the advent of endoscopic technology is expanding the fields of reconstructive and aesthetic surgery in adults, there have been few reports of the use of this technology in the pediatric population. Because of their minimally invasive nature, yet wide range of exposure, endoscopic techniques have much appeal for this age group. Here we present our experience with endoscopic pediatric plastic surgery. From February of 1995 to August of 1997, 104 patients underwent 139 procedures utilizing 5- and 10-mm endoscopes. There were 58 male and 46 female patients. The mean age at surgery was 5.6 years (range, 3 weeks to 19 years). The most common type of procedures performed were insertion of tissue expanders (n = 34), excision of benign head and neck masses (n = 27), torticollis release (n = 20), excision of vascular lesions (n = 13), and miscellaneous procedures, (n = 10). There were 26 complications in 139 procedures (19 percent). Seventeen (65 percent) were in the tissue expander group. The rest were scattered among the groups with other diagnoses. Although there did not appear to be a specific type of complication associated with endoscopy, 77 percent occurred in the first 2 months of our study. This suggests a relatively steep technical learning curve. These results demonstrate that endoscopic techniques are eminently applicable in the pediatric population, providing the benefits of small and remote incisional wounds, with complication rates that are comparable with those of conventional open surgical treatment.

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.

Mandibular distraction osteogenesis in the treatment of upper airway obstruction in children with craniofacial deformities.

Over the past 3 years, 16 patients (12 males, 4 females) have undergone mandibular distraction osteogenesis in conjunction with soft-tissue procedures to treat medically refractory obstructive sleep apnea. Thirty distraction devices were placed for bilateral distraction in 14 children and unilateral distraction in 2. The mean age of treatment was 4 years and 8 months (range 14 weeks to 12 years and 8 months). Eight of the patients had failed conventional medical and surgical treatment of obstructive sleep apnea and were considered tracheostomy candidates, whereas the remaining eight had tracheostomies placed shortly after birth for upper airway obstruction. These eight children could not be decannulated by standard protocols. The average distraction distance was 25 mm (range 18 to 35 mm). To date, seven of the eight patients with tracheostomies have been decannulated, and one is still in progress. Clinical improvement in the signs and symptoms of sleep apnea and reduction or elimination of preoperative oxygen requirements occurred in seven of the eight children with medically refractory sleep apnea. Twelve-channel polysomnograms were obtained preoperatively and postoperatively in each of the eight patients without tracheostomies. Respiratory disturbance index decreased from a mean of 7.1 to 1.7 after surgery. Lowest oxygen saturation rose from a mean of 0.70 to 0.89 after surgery. Application of mandibular distraction osteogenesis is an important component in the treatment of obstructive sleep apnea and permits mandibular advancement in the younger child. As more experience is gained with distraction osteogenesis in the treatment of children with obstructive sleep apnea, the role of distraction will become better defined.

Endoscopic surgical treatment for congenital muscular torticollis.

Muscular torticollis, if untreated, may result in significant permanent craniofacial deformities. Surgical release of the sternocleidomastoid muscle is recommended for patients who fail to respond to physical therapy. Current surgical techniques all leave noticeable scars, and provide limited exposure. These limitations make complete muscular release and visualization of the spinal accessory nerve difficult. We have applied a new technique of endoscopic release of the sternocleidomastoid muscle in 12 infants and children ages 4 to 18 months and one adolescent with muscular torticollis. Complete muscular release was achieved in all patients with one minor complication. The spinal accessory nerve was preserved in all cases. Our endoscopic approach allows precise division of the muscle fibers and preservation of neurovascular structures, leaving an inconspicuous scar in the scalp.

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.