Intraoperative high-dose epinephrine infiltration in cleft palate repair.

OBJECTIVE: Local infiltration of epinephrine before surgical procedures is a well-accepted technique to promote vasoconstriction. Typically, the dose of epinephrine is limited by the co-administration of local anesthetic as well as the risk for arrhythmogenesis and hemodynamic changes. In addition, some controversy exists regarding the acceptable dose of epinephrine given to children. This retrospective review examines the use and safety of "high-dose" epinephrine in palatoplasty at our cleft-craniofacial center. DESIGN: A retrospective review of epinephrine use in primary palatoplasty at a tertiary children's hospital from 2003 to 2007 was performed. Operative and anesthetic records were reviewed for hypertension (systolic blood pressure, >120 or diastolic blood pressure, >70) and tachycardia (>190 beats per min) as defined by the American Heart Association guidelines, as well as dysrhythmias, intraoperative complications, and postoperative complications. RESULTS: A total of 102 patients who underwent consecutive primary palatoplasties performed by a single surgeon were identified. After the induction of anesthesia and before incision, the patients received an initial epinephrine infiltration (without local anesthetic) up to a maximum 10 µg/kg. The average total dose of epinephrine administered during palatoplasty was 12.8 µg/kg (range, 3.2-75.0 µg/kg). Doses up to a maximum of 10 µg/kg were administered as needed at 30-minute intervals. No instances of clinically unstable tachycardia or hypertension occurred. A total of 21.6% of the patients (22/102) experienced an instance of hypertension, and only 13.7% of these (14/102) were related to epinephrine administration. One (1%) postoperative fistula was identified. CONCLUSIONS: Locally infiltrated high-dose epinephrine during palatoplasty can be safely used as a means of vasoconstriction. Doses reaching a maximum of 10 µg/kg, administered as needed at 30-minute intervals, do not seem to be a significant risk for hemodynamic instability, intraoperative complications, or postoperative complications.

215 mandible fractures in 120 children: demographics, treatment, outcomes, and early growth data.

BACKGROUND: Optimal management of pediatric mandible fractures demands that the practitioner balance reduction and fixation with preservation of growth potential and function. The ideal synthesis of these goals has not yet been defined. The authors catalogue their experience with pediatric mandible fractures at a major pediatric teaching hospital with reference to demographics, injury type, treatment, and outcomes to inform future management of these injuries. METHODS: Demographics, management, and outcomes of pediatric mandible fractures presenting over 10 years at a pediatric trauma center were assessed. Cephalometric analysis was conducted. Relationships among demographics, fracture type, management, outcomes, and growth were explored. RESULTS: Two hundred fifteen mandible fractures in 120 patients younger than 18 years were analyzed (average follow-up, 19.5 months). The condylar head and neck were fractured most frequently. Operative management was significantly more likely for children older than 12 years (p<0.05). Operative management and multiple fractures were significantly associated with a higher rate of adverse outcomes (p<0.05), but no adverse outcomes were considered to significantly affect mandibular function by patient or surgeon. No significant growth differences existed on cephalometric analysis between our cohort and age- and sex-matched controls (p>0.05). CONCLUSIONS: This study reports the demographics, treatment, and early follow-up of a sizable cohort of pediatric mandible fractures. Management principles for these injuries are outlined. Although definitive recommendations must be withheld until longer follow-up is available, the data presented here show that the treatment protocols used at the authors' center have yielded largely uncompromised mandibular function and growth thus far.

Relaxin does not rescue coronal suture fusion in craniosynostotic rabbits.

OBJECTIVES: Craniosynostosis affects 1 in 2000 to 3000 live births and may result in craniofacial and neural growth disturbances. Histological data have shown that thick collagenous bundles are present in the sutural ligament, which may tether the osteogenic fronts, resulting in premature fusion. The hormone relaxin has been shown to disrupt collagen fiber organization, possibly preventing craniosynostosis by relaxing the sutural ligament and allowing osteogenic fronts to separate normally and stay patent. This study tested this hypothesis with a rabbit model of delayed-onset coronal suture synostosis. METHODS: A total of 18 New Zealand White rabbits with craniosynostosis were randomly assigned to one of three groups: sham control, protein control (BSA), relaxin treatment. After initial diagnosis, sham surgery, BSA, or relaxin was delivered to the fusing coronal suture in a slow-release (56-day) collagen vehicle. Longitudinal radiographs and body weights were collected at 10, 25, 42, and 84 days of age, and sutures were harvested for histology. RESULTS: Relaxin-treated animals had more disorganized intrasuture content than control groups. These specimens also appeared to have relatively wider sutures ectocranially. There were no significant differences in relaxin-treated animals for all craniofacial growth measures, or suture separation compared with controls. CONCLUSIONS: These data do not support our initial hypothesis that the use of relaxin may rescue sutures destined to undergo premature suture fusion. These findings suggest that collagen fiber arrangement may not be important for suture fusion. This protein therapy would not be clinically useful for craniosynostosis.

Pediatric facial fractures: demographics, injury patterns, and associated injuries in 772 consecutive patients.

BACKGROUND: Pediatric craniofacial fractures are anatomically distinct from their adult counterparts and must be managed with respect for future growth and development. These injuries must be approached as entities fundamentally different from adult craniofacial fractures. Here, the authors aim to provide context for practitioners managing pediatric facial fractures by augmenting presently available demographic, diagnostic, and treatment data. METHODS: This is a retrospective review of demographics, diagnosis, and treatment of patients under 18 years of age presenting to the emergency department of a pediatric level I trauma center between 2000 and 2005 with facial fractures. Patients were included regardless of treating specialty, treatment modality, or inpatient status. RESULTS: A total of 772 consecutive patients met inclusion criteria. A significant majority (p < 0.001) of patients (68.9 percent) were male; older children were significantly more likely to sustain a facial fracture (p < 0.001). Fracture pattern, level of care, and cause of injury varied by age; 55.6 percent of patients had severe associated injuries. Male subjects, older patients, and patients of lower socioeconomic status were significantly more likely to sustain facial fractures secondary to violence (p ≤ 0.001). CONCLUSIONS: Pediatric facial fractures may be associated with severe concomitant injuries. Injury patterns are significantly correlated with socioeconomic metrics.

Pediatric craniofacial fractures due to violence: comparing violent and nonviolent mechanisms of injury.

BACKGROUND: This study examines the epidemiologic data of pediatric craniofacial fractures secondary to violence, comparing these data to craniofacial fractures sustained from all other causes. METHODS: A retrospective review was completed on all patients who presented to the emergency department of a major urban children's hospital from 2000 to 2005 with a craniofacial fracture. Data were compared between patients with fractures due to violent and nonviolent mechanisms. Socioeconomic analysis was performed using Geographic Information System mapping and 2000 US Census data by postal code. RESULTS: One thousand five hundred twenty-eight patients were diagnosed with skull and/or facial fractures. Isolated skull fractures were excluded, leaving 793 patients in the study. Ninety-eight children were injured due to violence, and 695 were injured from a nonviolent cause. Patients with violence-related fractures were more likely to be older, male, and nonwhite and live in a socioeconomically depressed area. A greater number of patients with violence-related injuries sustained nasal and mandible angle fractures, whereas more patients with non-violence-related injuries sustained skull and orbital fractures. Those with violence-related craniofacial fractures had a lower percentage of associated multiorgan system injuries and a lower rate of hospital admissions and intensive care unit admissions. The rate of open reduction and internal fixation for craniofacial fractures was similar in both groups. CONCLUSIONS: Patients with violence-related fractures had fewer associated serious injuries and lower morbidity and lived in a more socioeconomically depressed area. The information gained from this descriptive study improves our ability to characterize this population of pediatric patients and to identify the associated constellation of injuries in such fractures.

Outcomes in pediatric facial fractures: early follow-up in 177 children and classification scheme.

A comprehensive study of adverse outcomes after pediatric facial fractures has not been published. This study aimed to determine the incidence and classify adverse outcomes after facial fractures in children while reporting our early results. A retrospective chart review was performed on facial fracture patients identified in the Craniofacial Trauma Database of the Children's Hospital of Pittsburgh and seen in follow-up from 2003 to 2007. An Adverse Outcome Classification Scheme was developed: type 1, outcomes resulting from the fracture; type 2, outcomes resulting from fracture treatment; and type 3, outcomes resulting from the interaction between the fracture, its treatment, and subsequent growth and development. Fisher exact or χ analyses were completed. A total of 177 pediatric facial fracture patients were identified with 13.3 months of average follow-up. Mean age was 9.8 years (range, 0.4-18.7 y). Of these patients, 41.8% underwent surgery and 57 patients (32.2%) had adverse outcomes (type 1, 14.1%; type 2, 11.3%; and type 3, 15.8%); 26.3% of these had multiple adverse outcomes. Isolated fractures resulted in fewer adverse outcomes and fewer multiple adverse outcomes compared with combined fractures (26.6% versus 45.3%, P = 0.015; 4% versus 18.9%, P = 0.002). Patients treated operatively exhibited more types 1, 2, and 3 and multiple adverse outcomes compared to those treated conservatively (P < 0.01). In our pediatric cohort, 32.2% of patients had an adverse outcome. With longer follow-up and growth and development studies, we will likely see an increase in the incidence of type 3 adverse outcomes. We recommend, whenever possible, conservative treatment of pediatric facial fractures.

Masticatory hypermuscularity is not related to reduced cranial volume in myostatin-knockout mice.

It has been suggested recently that masticatory muscle size reduction in humans resulted in greater encephalization through decreased compressive forces on the cranial vault. Following this logic, if masticatory muscle size were increased, then a reduction in brain growth should also occur. This study was designed to test this hypothesis using a myostatin (GDF-8) knockout mouse model. Myostatin is a negative regulator of skeletal muscle growth, and individuals lacking this gene show significant hypermuscularity. Sixty-two [32 wild-type (WT) and 30 GDF-8 -/- knockout], 1, 28, 56, and 180-day-old CD-1 mice were used. Body and masseter muscle weights were collected following dissection and standardized lateral and dorsoventral cephalographs were obtained. Cephalometric landmarks were identified on the radiographs and cranial volume was calculated. Mean differences were assessed using a two-way ANOVA. KO mice had significantly greater body and masseter weights beginning at 28 days compared with WT controls. No significant differences in cranial volumes were noted between KO and WT. Muscle weight was not significantly correlated with cranial volume in 1, 28, or 180-day-old mice. Muscle weights exhibited a positive correlation with cranial volume at 56 days. Results demonstrate that masticatory hypermuscularity is not associated with reduced cranial volume. In contrast, there is abundant data demonstrating the opposite, brain growth determines cranial vault growth and masticatory apparatus only affects ectocranial morphology. The results presented here do not support the hypothesis that a reduction in masticatory musculature relaxed compressive forces on the cranial vault allowing for greater encephalization.

Recombinant human bone morphogenetic protein-2-induced craniosynostosis and growth restriction in the immature skeleton.

BACKGROUND: Recombinant human bone morphogenetic protein-2 (rhBMP-2) delivered on an absorbable collagen sponge is a U.S. Food and Drug Administration-approved therapy effective at generating bone formation. In pediatric patients for whom other therapeutic options have been exhausted, rhBMP-2 is used off-label to address problematic bony defects. In the skeletally immature patient, the safety of rhBMP-2 therapy remains uncertain. Experiments are needed that investigate the effect of rhBMP-2 on growth and development in clinically relevant models. METHODS: Ten juvenile rabbits underwent creation of a parietal skull defect that was treated with either 0.2 mg/cc rhBMP-2/absorbable collagen sponge or a neutral buffer solution/absorbable collagen sponge. Amalgam markers were placed at suture confluences to track suture separation and skull growth. Cranial growth was assessed radiographically at 10, 25, 42, and 84 days of age. Means and standard deviations for the various craniofacial growth variables were calculated and compared. Mean differences were considered significant for values of p < 0.05. At 84 days, sutures were analyzed by means of micro-computed tomographic scanning and histologic staining. RESULTS: Treatment with rhBMP-2 resulted in fusion of the coronal sutures bilaterally, with variable fusion of the sagittal suture by cephalometric, radiographic, and histologic analysis. There were statistically significant changes to coronal suture growth, sagittal suture growth, skull height, craniofacial length, and intracranial volume (p < 0.05). CONCLUSIONS: The use of rhBMP-2 in this juvenile animal model resulted in skeletal changes that may be undesirable in a clinical setting. The appearance of these fused sutures suggested a direct effect of rhBMP-2. Further work is required to limit the effect of rhBMP-2 to the target defect when used in the immature skeleton.

Blocking bone morphogenetic protein function using in vivo noggin therapy does not rescue premature suture fusion in rabbits with delayed-onset craniosynostosis.

BACKGROUND: Craniosynostosis is defined as the premature fusion of one or more cranial sutures. Bone morphogenetic proteins (BMPs), regulators of ossification, have been implicated in premature suture fusion. Noggin, an extracellular BMP inhibitor, has been shown experimentally to inhibit resynostosis following surgery. The present study was designed to test the hypothesis that BMP inhibition using noggin therapy may rescue sutures destined to fuse by inhibiting initial ossification. METHODS: Twenty-six, 10-day old rabbits with familial, delayed-onset, coronal suture synostosis were randomly divided into three groups: (1) the sham surgical control group, (2) the bovine serum albumin-treated group [10 µg/suture (protein/vehicle controls)], and (3) the noggin therapy group (10 µg/suture; experimental group). Sutural growth was monitored by radiopaque markers implanted at 10 days of age. At 25 days, the bovine serum albumin or noggin was combined with a slow-resorbing collagen vehicle and injected subperiosteally above the coronal suture. Somatic and sutural growth data were collected at 10, 25, 42, and 84 days of age. Coronal sutures were harvested at 84 days to histologically assess fusion. RESULTS: Results showed no significant (p > 0.05) differences in suture separation at any age. Suture fusion assessed by histomorphology did not differ among the three groups. Although previous data showed noggin to inhibit postoperative resynostosis in this craniosynostotic rabbit model, here there was no effect on initial suture fusion. CONCLUSION: These results suggest that in this rabbit model of craniosynostosis, BMPs do not play a role in the pathogenesis of craniosynostosis and only play a role in postoperative bony wound healing.

Effects of flutamide therapy on craniofacial growth and development in a model of craniosynostosis.

Research has implicated the faulty regulation of transforming growth factor beta signaling as one mechanism for premature calvaria suture fusion. Androgens have been shown to increase the expression and activity of the transforming growth factor beta, resulting in increased osteoblast proliferation and differentiation and possibly premature suture fusion. The present study was designed to test the hypothesis that flutamide, an androgen receptor-blocking agent, would "rescue" a coronal suture destined to fuse and improve craniofacial growth in a familial rabbit model of craniosynostosis. Thirty rabbits with delayed-onset, coronal suture synostosis were examined via longitudinal cephalometry. The rabbits were divided into 4 groups: (1) sham surgical controls (n = 10), (2) bovine serum albumin (500 ng) protein controls (n = 6), (3) flutamide diluent controls (n = 6), and (4) flutamide (15 mg dissolved in ethanol) experimental group (n = 8). At 10 days of age, radiopaque amalgam markers were implanted in all rabbits on either side of the coronal suture to monitor sutural growth. At 25 days of age, the bovine serum albumin, ethanol, and flutamide were combined with a slow-resorbing collagen vehicle and injected subperiosteally above the coronal suture into the respective groups. Although results revealed a slight but significant increase in coronal suture marker separation in flutamide-treated rabbits compared with controls at 42 days of age, few significant differences were noted for craniofacial growth and intracranial volume among groups. Results suggest that androgen receptor-blocking using flutamide may only provide a transient rescue to suture fusion in this model. Further research is needed to investigate the effects of hormones on suture development and maintenance.

Autologous bone grafting with adjunctive use of acellular dermal matrix for alveolar cleft defects: early outcomes.

BACKGROUND AND PURPOSE: Alveolar bone grafting remains the standard for alveolar cleft repair. Compromised oral and/or nasal closure may impede healing and result in graft failure or persistent fistulae. Incorporating acellular dermal matrix into these repairs may protect the bone graft during mucosal healing by providing an additional layer of soft tissue coverage. METHODS: A retrospective review of alveolar bone grafts undertaken at our cleft-craniofacial center from 2005 to 2007 was performed. The use of acellular dermal matrix for nasal and/or oral lining augmentation was determined. A minimum 3 months' follow-up was required for inclusion. Outcomes included (1) mucosal disruption, (2) time to complete mucosal healing, (3) bone graft exposure, (4) postoperative bone graft incorporation using the Chelsea scale, and (5) canine eruption through the graft site. RESULTS: In total, 35 patients were included. Of those, 15 patients (four girls, 11 boys; seven Veau III, eight Veau IV) received acellular dermal matrix for mucosal augmentation (five nasal, one oral, nine nasal and oral lining). Average age at surgery was 10 years (range, 9 to 16 years). Average follow-up was 23 months (range, 3 to 35 months). Mucosal disruption occurred in 20% of the acellular group and in 30% of the control group (p=nonsignificant). Complete mucosal healing was achieved at an average of 4 weeks (range, 1 to 14 weeks) in the acellular dermal matrix group versus 4 weeks (range, 2 to 11 weeks) in the control group (p=nonsignificant). Exposure of bone graft occurred in 0% of the acellular dermal matrix group and in 30% of the control group (p=.016). The Chelsea scale demonstrated no significant radiographic difference in postoperative bone graft incorporation between the acellular dermal matrix and control groups. Canine eruption through the graft site was similar for both groups. CONCLUSIONS: These data support the conclusions that using acellular dermal matrix to augment nasal/oral mucosal lining in alveolar bone grafts (1) does not increase mucosal disruption or time to complete healing, (2) prevents postoperative bone graft exposure, and (3) appears to have no negative effect on postoperative bone graft incorporation or canine eruption through the graft site.

Age-related changes in craniofacial morphology in GDF-8 (myostatin)-deficient mice.

It is well recognized that masticatory muscle function helps determine morphology, although the extent of function on final form is still debated. GDF-8 (myostatin), a transcription factor is a negative regulator of skeletal muscle growth. A recent study has shown that mice homozygous for the myostatin mutation had increased muscle mass and craniofacial dysmorphology in adulthood. However, it is unclear whether such dysmorphology is present at birth. This study examines the onset and relationship between hypermuscularity and craniofacial morphology in neonatal and adult mice with GDF-8 deficiency. Fifteen (8 wild-type and 7 GDF-8 -/-), 1-day-old and 16 (9 wt and 7 GDF-8 -/-), 180-day-old male CD-1 mice were used. Standardized radiographs were taken of each head, scanned, traced, and cephalometric landmarks identified. Significant mean differences were assessed using a group x age, two-way ANOVA. Myostatin-deficient mice had significantly (P < 0.01) smaller body and masseter muscle weights and craniofacial skeletons at 1 day of age and significantly greater body and masseter muscle weights at 180 days of age compared to controls. Myostatin-deficient mice showed significantly (P < 0.001) longer and "rocker-shaped" mandibles and shorter and wider crania compared to controls at 180 days. Significant correlations were noted between masseter muscle weight and all cephalometric measurements in 180-day-old Myostatin-deficient mice. Results suggest that in this mouse model, there may be both early systemic skeletal growth deficiencies and later compensatory changes from hypermuscularity. These findings reiterate the role that masticatory muscle function plays on the ontogeny of the cranial vault, base, and most notably the mandible.

Nasal tip complications of primary cleft lip nasoplasty.

BACKGROUND AND PURPOSE: Complications of primary nasoplasty, at the time of definitive primary cheiloplasty, are underreported in the literature. This study endeavors to examine the occurrences of these complications at our cleft-craniofacial center, in an effort to identify causative factors and management strategies. A case series of patients with postoperative nasal complications after primary cleft lip nasal surgery is presented. METHODS: A retrospective chart review of primary cleft lip nasal repairs was conducted at our cleft-craniofacial center between January 2003 and December 2007. Consecutive cases of 3 staff surgeons were evaluated. Specific data points included number and type of complications, subsequent required interventions, and relevant history, with particular attention paid to the details of the primary nasoplasty. RESULTS: Eighty-six primary cleft lip nasoplasties were completed between the years 2003 and 2007. Six complications (6.9%) related to the primary cleft lip nasoplasty were identified. Four patients (4.6%) experienced nasal tip infections; all 4 required surgical drainage. Twenty-four patients (27.9%) undergoing primary cleft lip and nose repair had postoperative nostril conformers placed, and 2 (8.3%) of them experienced complications deemed conformer related. CONCLUSIONS: Postoperative nasal complications of primary cheiloplasty occur and are likely underreported. In this series, complications resulted from infection, often occurring late, and secondary to the use of nostril conformers. Surgeon awareness and caregiver education, to identify the early signs of postoperative nasal complications, are critical to the successful treatment of these occurrences. Although this study did not intend on examining antibiotic use, the significance of nasal tip infections might support the regular use of antibiotics in this population, and the use of postoperative nostril conformers must be followed closely.

Pediatric orbital fractures: classification, management, and early follow-up.

BACKGROUND: Scarce literature exists addressing the presentation, classification, and management of pediatric orbital fractures. The aim of this study is to review the authors' experience with the presentation, management, and early follow-up of pediatric orbital fractures. METHODS: A retrospective review of pediatric orbital fractures presenting to the Children's Hospital of Pittsburgh between 2003 and 2007 was performed. Demographics, associated injuries, computed tomographic scan findings, management, and follow-up were collected. From these data, a pediatric orbital fracture classification system was devised. RESULTS: Seventy-four patients (81 orbits) were reviewed. Average age at presentation was 8.6 years. Fractures were distributed as follows: type 1, 40.7 percent; type 2, 33 percent; and type 3, 25.9 percent. Twenty-three orbits were treated surgically and 58 were treated nonoperatively. The operative rates were as follows: type 1, 9.1 percent; type 2, 14.8 percent; and type 3, 76.2 percent. Complications included minor enophthalmos in seven patients, and persistent cerebrospinal fluid leak in two growing skull fractures. For type 1 (pure orbital) fractures, three (12 percent) underwent surgical treatment for acute enophthalmos, vertical orbital dystopia, or muscle entrapment. Twenty-two orbits (88 percent) were managed nonoperatively. At an average follow-up of 13 months, minimal enophthalmos (1 to 2 mm) was found in one of the surgically treated fractures (33 percent) and in three of the conservatively managed fractures (13.6 percent). CONCLUSIONS: For type 1 (pure orbital) fractures, unless there is evidence of acute enophthalmos, vertical orbital dystopia, or muscle entrapment, a nonoperative approach is advocated. Type 2 (craniofacial) fractures should be followed with serial computed tomographic scans; and type 3 (common fracture patterns) fractures have a greater chance of requiring surgery.

A successful algorithm for limiting postoperative fistulae following palatal procedures in the patient with orofacial clefting.

BACKGROUND: Palatal procedures include (1) primary palatoplasty, (2) oronasal fistulas repair, and (3) secondary palatoplasty for velopharyngeal insufficiency. Any time a palatal procedure is performed, postoperative fistulas remain potential consequences. Presented here is a successful algorithm for performing palatal procedures and decreasing the rate of postoperative fistulas in a large, single-surgeon, consecutive series. METHODS: A retrospective review of all consecutive palatal procedures performed between 2002 and 2006 including (1) primary palatoplasty, (2) oronasal fistulas repair, and (3) secondary palatoplasty for velopharyngeal insufficiency was performed. Cleft Veau type, surgical technique, and outcomes are reviewed. The algorithm included (1) relaxing incisions, (2) complete intravelar veloplasty, (3) total release of the tensor tendon, (4) dissection of the neurovascular bundle with optional osteotomy of the foramen, and (5) incorporation of acellular dermal matrix to achieve complete nasal lining reconstruction. RESULTS: Two hundred sixty-eight palatal procedures were performed: (1) 132 primary Furlow palatoplasties yielding one symptomatic post-Furlow palatoplasty fistula (0.76 percent) (acellular dermal matrix was used in 39.4 percent of primary palatoplasties); (2) 55 oronasal fistula repairs yielding two symptomatic postoperative fistulas (3.6 percent) (acellular dermal matrix was used in 90.9 percent of fistula repairs); and (3) 81 secondary palatoplasties for velopharyngeal insufficiency resulting in no postoperative fistulas. Acellular dermal matrix was used in 14.8 percent of secondary palatoplasties for velopharyngeal insufficiency. No recommendations for speech surgery followed palatoplasty. CONCLUSIONS: Using the proposed algorithm in this single-surgeon consecutive series of 268 cases, the authors achieved the lowest reported incidence of postoperative fistulas in all forms of palatal procedures, including the lowest incidence (0.76 percent) of symptomatic palatal fistulas following primary Furlow palatoplasty.

Transpalatal endoscopic endonasal resection of a giant epignathus skull base teratoma in a newborn. Case report.

Teratomas are neoplasms composed of tissues from all three germ layers with varying degrees of differentiation. They are most commonly found in the sacrococcygeal and gonadal regions and rarely occur in the head and neck region. A teratoma is termed "epignathus" when it arises from the skull base or hard palate and is located in the oral cavity. The authors describe a case of a giant epignathus teratoma originating in the skull base of a neonate, extending bilaterally via two pedicles throughout the hard palate and protruding through the oral cavity. The tumor was completely resected using a transpalatal endoscopic endonasal approach. The excised tumor proved to be an immature teratoma with well-differentiated yolk sac elements. At the 1-year follow-up the patient showed no evidence of tumor recurrence and the child remains neurologically intact. This report demonstrates the use of a transpalatal endonasal corridor in a preterm infant. This approach provided an ample corridor into the ventral skull base without the need for external excisions and/or disruption of osseous elements.

Noggin inhibits postoperative resynostosis in craniosynostotic rabbits.

UNLABELLED: Inhibition of bone formation after surgery to correct craniosynostosis would alleviate the need for secondary surgeries and decrease morbidity and mortality. This study used a single dose of Noggin protein to prevent resynostosis and improve postoperative outcomes in a rabbit model of craniosynostosis. INTRODUCTION: Craniosynostosis is defined as the premature fusion of one or more of the cranial sutures, which causes secondary deformations of the cranial vault, cranial base, and brain. Current surgical intervention involves extirpation of the fused suture to allow unrestricted brain growth. However, resynostosis of the extirpated regions often occurs. Several bone morphogenetic proteins (BMPs), well-described inducers of ossification, are involved in bone healing. This study tested the hypothesis that a postoperative treatment with Noggin, an extracellular BMP inhibitor, can inhibit resynostosis in a rabbit model of human familial nonsyndromic craniosynostosis. MATERIALS AND METHODS: Thirty-one New Zealand white rabbits with bilateral coronal suture synostosis were divided into three groups: (1) suturectomy controls (n = 13); (2) suturectomy with BSA in a slow-resorbing collagen vehicle, (n = 8); and (3) suturectomy with Noggin in a slow-resorbing collagen vehicle (n = 10). At 10 days of age, a 3 x 15-mm coronal suturectomy was performed. The sites in groups 2 and 3 were immediately filled with BSA-loaded gel or Noggin-loaded gel, respectively. Serial 3D-CT scan reconstructions of the defects and standard radiographs were obtained at 10, 25, 42, and 84 days of age, and the sutures were harvested for histological analysis. RESULTS: Radiographic analysis revealed that Noggin-treated animals had significantly greater coronal suture marker separation by 25 days and significantly greater craniofacial length at 84 days of age compared with controls. 3D-CT analysis revealed that Noggin treatment led to significantly greater defect areas through 84 days and to increased intracranial volumes at 84 days of age compared with other groups. Histological analysis supported CT data, showing that the untreated and BSA-treated groups had significant healing of the suturectomy site, whereas the Noggin-treated group had incomplete wound healing. CONCLUSIONS: These data support our hypothesis that inhibition of BMP activity using Noggin may prevent postoperative resynostosis in this rabbit model. These findings also suggest that Noggin therapy may have potential clinical use to prevent postoperative resynostosis in infants with craniosynostosis.

The Pittsburgh Fistula Classification System: a standardized scheme for the description of palatal fistulas.

OBJECTIVE: Vague terminology is a problem in cleft palate research. No classification scheme for palatal fistulas has been proposed to date. Although a well-healed velum is a significant outcome of palatoplasty, it is nearly impossible to compare fistula-related palatoplasty results in the literature or in medical records without a standardized vocabulary. We endeavor to devise a palatal fistula classification system that may have clinical and research applicability. DESIGN: PubMed was searched for definitions and classifications of palatal fistula as well as incidence and recurrence rates of this outcome. Next, a 25-year retrospective review of our Cleft Center's records was performed, and fistulas were identified (n=641 charts reviewed). The fistula descriptions yielded by this chart review were evaluated in the context of anatomical descriptions in the literature, and a clinician-friendly classification scheme was designed. RESULTS: A literature review failed to reveal a standardized fistula classification system. An anatomically based numerical fistula classification system was devised: type I, bifid uvula; type II, soft palate; type III, junction of the soft and hard palate; type IV, hard palate; type V, junction of the primary and secondary palates (for Veau IV clefts); type VI, lingual alveolar; and type VII, labial alveolar. CONCLUSIONS: We propose a standardized numerical classification system for palatal fistulas. Its clinical adoption may prospectively clarify ambiguities in the literature and facilitate future cleft palate research and clinical practice.