Craniofacial fibrous dysplasia of the fronto-orbital region: a case series and literature review.

PURPOSE: Craniofacial fibrous dysplais (CFD), involving the fronto-orbital region often is challenging to treat because of the proximity of neurovascular and ocular structures. This article presents the surgical experience with 6 patients. PATIENTS AND METHODS: Six patients ranging in age from 7 to 23 years, who had undergone surgery for CFD of the orbital region, were retrospectively reviewed. All patients had progressive complaints of deformity, pain, paresthesia or visual disturbances. Surgery generally involved extensive tumor excision and immediate orbital reconstruction with autogenous bone grafts. Two patients also were reconstructed with resected and treated autogenous bone, which then was immediately reimplanted, using rigid fixation. Three patients also had intracranial microsurgical optic canal decompression. All patients received perioperative corticosteroids. RESULTS: Five patients experienced partial relief of their sensory and visual disturbances. These patients also felt that there had been improvement in facial appearance. One patient with very extensive circumferential optic canal involvement, and who underwent 2-wall decompression, suffered visual loss. CONCLUSION: Early surgery to address progressive sensory disturbances is recommended so as to avoid the hazards of late-stage decompression. The risks of surgery must be weighed against the benefits of achieving a more functional and aesthetic result. Long-term neuro-ophthalmologic monitoring is essential.

Scar modification. Techniques for revision and camouflage.

The surgery and management of scars is a protracted and staged process that includes preparation of the skin through hygienic measures, scar softening (if indicated) with steroids, massage and pressure dressings, skilled execution of the surgical plan, and thorough postoperative wound care. This process generally covers a 1-year period for the various stages mentioned. Many general host and local skin factors will directly affect the final revision result. The two most important indirect factors that the surgeon must endeavor to control are optimal patient preparation and cutaneous health, and patient compliance with, and an ability to carry out, those wound care measures that the surgeon prescribes. Keloid and burn contracture scars represent two entities that are complicated and challenging to treat owing to their abnormal morphophysiologic features. Management of these scars is prolonged, and the patient must understand that the ultimate result will usually be a compromise. New grafting techniques, such as cultured autodermal grafts, offer improved initial management of burn wounds that may subsequently optimize scar revision in these patients. Keloids, and to a lesser extent hypertrophic scars, require steroid injections, pressure treatment, careful surgery, and protracted wound support and pressure treatment (exceeding 6 months) after surgery.

Correction of late enophthalmos with polyethylene implant.

High density porous polyethylene (HDPP) has been used extensively for craniofacial reconstructive procedures. The authors recently reviewed the records of 21 patients who had undergone correction of late (more than 6 months) posttraumatic enophthalmos utilizing HDPP implants to restore orbital integrity and volume. Follow-up after implant placement was 6 months to 54 months (mean 20 months). Twenty of 21 patients demonstrated a clinically significant reduction in enophthalmos to within 2 mm of the opposite globe, as demonstrated by Hertel and/or Naugle exophthalmometry and by clinical observation. There were no cases of worsened visual acuity, lid position or ocular mobility, and no HDPP implant became infected or was extruded. The authors' experience supports the safety and efficacy of HDPP implants for correction of mild to moderate posttraumatic enophthalmos.

Nasal symmetry after primary cleft lip repair: comparison between Delaire cheilorhinoplasty and modified rotation-advancement.

PURPOSE: Periosteal musculoaponeurotic (PMAS) reconstruction is a key objective in primary repair of the cleft lip. The reconstructed muscles provide a sound framework and stimulus for development of the nasolabial region. In this study two primary cleft lip repair techniques were retrospectively studied to determine if one [Delaire (Del)] which involves PMAS repair around the cleft results in more nasolabial symmetry than a technique that does not fully addressed the musculature [modified Rotation-Advancement, (mR-A)]. PATIENTS AND METHODS: Four-and 5-year-old children with complete unilateral cleft lip and palate were recalled for study (n = 33 children; Del group, 16; mR-A group, 17). Clinical and photographic records were obtained and anthropometric analyses determined for comparison between groups. RESULTS: In the mR-A group, noses had more asymmetrical tips, were shorter, projected less, and tended to be flatter or wider. Nasal indices tended to support these findings. Nasal height was similar for the mR-A and Del groups. When comparing cleft versus noncleft sides, alar width and length and nostril length were significantly different for both cleft groups. CONCLUSIONS: It appears from this study that reconstruction of the PMAS of the nasolabial region during primary cleft lip repair may positively influence growth and development of the nose in unilateral cleft lip and palate patients.

The double-reversing Z-plasty in primary palatoplasty: operative experience and early results.

The double-reversing Z-plasty of Furlow for closure of the soft palate was used in 34 children with various types of cleft palate. Mean age at repair was 12.8 months. Intraoperative experience was favorable, with acceptable operating time and blood loss. Length of hospitalization averaged 1.9 days. Postoperatively, two children experienced temporary stridor, which resolved within 24 to 48 hours. One child had dehiscence of the hard palate (Von Lagenbeck repair) 4 weeks postoperatively, and three children developed small oronasal fistulae. Early speech evaluation demonstrated adequate soft palate mobility in 33 of 34 patients, with observable velopharyngeal function. Twelve children had mild velar compromise, with eight exhibiting slight nasal air escape.

Cranial base morphology in cleft lip and palate: a cephalometric study from 7 to 18 years of age.

Four cleft lip/palate groups were cephalometrically compared with a noncleft control group using three cranial base dimensions: anterior (ACB) and posterior (PCB) cranial base, and cranial base angle (CBa). The groups were compared by age from 7 to 18 years. The results showed that the cleft lip and/or alveolus group was not different from the control group in the three dimensions. Unilateral and bilateral cleft lip and palate and isolated cleft palate cases were generally shorter in ACB and PCB for most of the growth period, particularly after age 8 to 9 years. The cranial base angle remained similar for all the cleft groups when compared with the noncleft group. These findings support the view that intrinsic tissue defects or errors during embryologic development may result in basicranial abnormalities and orofacial clefting.

The incidence and relationship of cervical spine anomalies in patients with cleft lip and/or palate.

The relationship, incidence, and distribution of cervical spine anomalies were assessed in 468 patients with cleft lip and/or palate. The patients were placed into four groups: lip and/or alveolar; complete unilateral or bilateral; isolated palatal; and soft palate or submucous clefts. Cervical anomalies were observed in 22% of the cleft patients and in 7% of the noncleft group. Patients with soft palate and submucous clefts had the highest incidence of vertebral anomalies (45%), whereas patients with cleft lip and/or alveolus had an incidence similar to the noncleft group. Patients with complete unilateral and bilateral clefts also had a higher incidence (15.6% to 19.0%) of anomalies than the noncleft group. Cervical anomalies occurred primarily in the occipital-C1-C2 region. The possible implications of these findings are discussed.

Nonporous hydroxylapatite in the repair of alveolar clefts in a primate model: clinical and histologic findings.

Twelve adult Rhesus monkeys with surgically created alveolar clefts (ACs) underwent repair that used nonporous hydroxylapatite (NPHA) granules on one side and autogenous particulate bone in the contralateral defect. Clinical and radiographic evaluations at 1, 2, 3, 6, and 12 months postoperatively disclosed some displacement of granules initially, but this stabilized by 2 months. Three of 12 sites repaired with NPHA and four of 12 sites repaired with bone dehisced; however, all of these healed by 2 months. No untoward inflammatory or resorptive changes were observed beyond 3 months. The repaired alveolar processes were similar in bulk and contour in sites grafted with NPHA and with bone. Histologically, giant cells were noted at 3 months, but the number decreased at 6 months and remained at that level up to 12 months. The NPHA granules were embedded in mature fibrous connective tissue with osseous ingrowth evident in eight of the 12 defects. It was concluded that NPHA granules are an acceptable implant material for repair of residual alveolar clefts in which erupting teeth and orthodontic movements are not factors.

Desmoplastic fibroma of the jaws: surgical management and review of the literature.

Desmoplastic fibroma of the jaws is a rare and locally aggressive tumor that has a high rate of recurrence. The cellularity, extent of the tumor, and completeness of the local excision may be factors in its tendency to recur. We reviewed the literature in an attempt to determine if there was any correlation between the histologic features of a desmoplastic fibroma, or the surgical procedure utilized, and the tumor's recurrence. From the information gathered, it was found that a more cellular desmoplastic fibroma or inadequate surgical procedure may be factors that contribute to the recurrence of the tumor. A case report of desmoplastic fibroma of the mandible in a 9-year-old child is presented. The tumor, which displayed areas of moderate cellularity, was resected via a partial hemimandibulectomy. There has been no recurrence to date (46 months).

Craniofacial growth in unilateral cleft lip and palate: skeletal growth from eight to eighteen years.

Sixteen individuals with complete unilateral cleft lip and palate (UCLP) were evaluated for determination of several craniofacial dimensional means and growth rates. Each had undergone primary lip and palatal closure and alveolar bone grafting. Serial cephalographs from ages 8 to 18 years, taken every 2 years, were utilized for determination of six cephalometric dimensions: anterior cranial base, upper and lower facial heights, posterior nasomaxillary height, maxillary horizontal length, and mandibular length. These were then compared to published cephalometric standards of a nonclefted group. All dimensions, except mandibular length, were smaller in the UCLP group. The horizontal maxillary length was the most diminished in mean length and growth rate; it appears to be most affected in UCLP. The remaining dimensions and growth rates are affected by UCLP, but to a lesser degree. These findings indicated that individuals with unilateral cleft lip and palate are primarily and adversely affected by clefting (and the surgery as described) in the horizontal maxilla, both in dimension and growth rate.

Cephalometric and anthropomorphic observations of Binder's syndrome: a study of 19 patients.

Binder's syndrome (maxillonasal dysplasia) is a disorder characterized by nasomaxillary hypoplasia. To ascertain the extent of underdevelopment of the midfacial skeleton and soft tissues, 19 of 29 patients with Binder's syndrome were retrospectively evaluated, both with cephalometry and anthropometry. Ten females and nine males were placed collectively into three age groups: 6 years, 10 years, and 16 to 17 years. Cephalometric measurements disclosed a short anterior cranial base (S-N), a normal length of the vertical maxilla (SE-PNS), a decreased horizontal maxilla (PNS-A, Co-A), a recessed orbitale (SNO), and a high-normal mandibular length (Co-Gn). Anthropometry revealed a large nasofrontal angle, acute nasal inclination and nasolabial angle, decreased nasal prominence (Sn-Prn), a decreased columellar length (C-Sn), and a normal vertical nose (N-Sn) and upper lip (Sn-Sto) length.

Arteriovenous malformation in the mandible of a young child.

Arteriovenous malformations rarely occur in the jaws, particularly in the very young child. The vascular and haemodynamic nature of the lesion is important in determining treatment and providing for a favourable prognosis, particularly in the high-pressure malformations. If the lesion is unstable and active, that is, there is enlargement, pain, bleeding or ulceration, successful treatment will depend upon early pre-operative embolization and surgical extirpation of the lesion. A 4-year old boy is presented who had a moderately active arteriovenous malformation of the left mandibular body. Angiography disclosed an arteriovenous malformation supplied by the inferior alveolar, lingual and facial arteries on the left side with no contralateral contribution. Pre-operative embolization via selective catheterization of the major arterial feeder was performed. Five days later, the involved portion of the mandible was resected and a corticocancellous bone-graft was placed. The 6-month follow-up disclosed no evidence of new arterial feeders or contralateral involvement.

Primary hemihypertrophy of the face: review and report of two cases.

Two patients who underwent surgical correction for facial hemihypertrophy are presented with a one year follow-up. The aid of computerized tomography in planning the procedures and assessing the stability of the result is discussed.

Maxillonasal dysplasia (Binder's syndrome): a critical review and case study.

Binder's syndrome (maxillonasal dysplasia) is a disorder of unknown etiology characterized by nasomaxillary hypoplasia and a 40-50% association of an underdeveloped frontal sinus and cervicospinal abnormalities. The midfacial retrusion is similar to that in chondrodysplasia punctata, resulting in confusion regarding diagnosis. This paper outlines the distinguishing features of Binder's syndrome, the treatment considerations, and presents 24 patients seen and treated. The facial and skeletal characteristics and developmental findings are emphasized to affirm the diagnosis of Binder's syndrome. A familial finding of Binder's features in five patients raises the possibility of a genetic mechanism, a previously undisclosed finding.