Arlen D. Denny

A unilateral deficit in velopharyngeal closure during speech production is an unusual presentation of velopharyngeal dysfunction (VPD) and few solutions have been described for this problem. This report details the long-term outcomes using this technique. We performed a retrospective chart review of all the patients who underwent a unilateral Orticochea pharyngoplasty for VPD. The authors identified 10 patients treated with 11 unilateral pharyngoplasty at a single tertiary care institutional setting. The mean age at repair was 8.6 years. The mean length of follow-up was 7.6 years. All the patients demonstrated improvement in velopharyngeal closure and speech resonance with 4 showing complete resolution of VPD. There were no revisions required or complications. One patient, after 10.7 years, required a unilateral procedure on the contralateral side which resulted in complete resolution of VPD postoperatively.

Frontal orbital advancement (FOA) for metopic synostosis results in cranial vault expansion, supporting underlying growth of the developing brain and improving head shape. Previous studies have shown that FOA in younger infants leads to a greater incidence of long-term growth restriction of intracranial volume; however, it is still unknown as to whether this is due to undercorrection at the time of surgery versus primary suture pathology. The purpose of our study is to provide a method for objective analyses of intracranial volumes in the early post-FOA period. A retrospective chart review was conducted on patients who underwent FOA over a 10-year period by a single surgeon. Data collected included patient characteristics and clinical outcomes. Radiological analysis of intracranial volumes pre- and post-FOA was determined using Amira volume-rendering software. Average increase in intracranial volume at 6 weeks post-FOA was 139.27 mL (80.01–225.25 mL) with average relative cranial vault expansion of 18.1% (3.3–48%). Patients who underwent FOA older than 12 months of age had an average increase in intracranial volume of 8.5% (3.3–13.1%). Patients younger than 12 months had a statistically higher average increase of 25% (12.8–48%). Frontal orbital advancement effectively increases intracranial volume in patients with metopic synostosis. When compared with older patients, patients younger than 1 year of age have a greater relative increase in intracranial volume in the immediate postoperative period. Quantifying cranial vault expansion after FOA can assist surgeons in surgical planning as well as in measuring and monitoring clinical outcomes within and across craniofacial centers regardless of technique.

Background: Cranial bone grafting for an alveolar cleft obtains membranous bone from a low-morbidity donor site. Although iliac crest bone is the favored donor site, there are no objective analyses of three-dimensional radiologic outcomes with cranial bone grafting and no studies evaluating complications and long-term outcomes in a large series of patients undergoing cranial bone grafting. Methods: A retrospective chart review was conducted on patients who underwent alveolar bone grafting from the cranium over a 25-year period performed by a single surgeon. Data collected included patient characteristics, complications, and clinical outcomes. Radiologic analysis of graft outcomes was determined using Amira volume-rendering software on the most recent 10 consecutive patients. Results: The authors’ study cohort included 308 patients, with an average age of 11.5 years. Complications involved harvesting the graft in 3.5 percent, the donor site in 1 percent, and the recipient site in 17.2 percent. Regrafting was required in 7.1 percent, with a clinical success rate of 92.9 percent. The average alveolar defect was 1.19 ml preoperatively and 0.19 ml postoperatively, with 85 percent fill of the cleft defect by radiologic analysis. Conclusions: Cranial bone grafting for the alveolar cleft is a low-morbidity operation and has success similar to that of iliac crest bone grafting. It should be considered more often as a viable option for the alveolar cleft patient. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Postpalatoplasty fistulas of the hard palate remain challenging. The reported recurrence rate after surgical correction ranges between 33 and 37 percent. The authors describe a surgical technique for the correction of postpalatoplasty fistulas by total elevation of the palatal gingivoperiosteum starting at the dental sulcus, excision of the fistula, and watertight separate closure of the nasal and palatal mucosa by means of the transoral approach. This technique has the advantages of providing adequate exposure for closure of the nasal mucosal gap and allowing closure of the palatal defect under minimal tension. Placement of interdental sutures leaves no raw bone surfaces exposed. The authors have used this technique on 60 consecutive patients with fistulas of the primary and secondary hard palate between 1998 and 2003. Their overall success rate of fistula closure was 90 percent. The success rate for previously unoperated postpalatoplasty fistulas was 96.9 percent. All of the failures occurred in patients who had bilateral cleft lip and palate. No complications of tooth loss or gingival recession were observed. A detailed description of the technique with illustrations is provided.

Tongue-lip adhesion can be effective in relieving tongue-based airway obstruction in the neonatal period. However, longitudinal evaluation of these patients demonstrates they require additional procedures for ultimate control of their airway, feeding, and orthognathic relationship. Charts of patients with Pierre Robin sequence who underwent unsuccessful nonoperative treatment and tongue-lip adhesion procedures by the senior author between 1989 to 1999 were reviewed. In particular, secondary interventions were quantified and qualified to determine if tongue-lip adhesion was a definitive treatment of the pathology caused by this sequence. Eleven patients with ages ranging from 2 to 6 weeks underwent initial tongue-lip adhesion. Seven patients were syndromic (3 Stickler’s, 1 Goldenhar’s, 1 Fragile X, 1 Miller’s, 1 chromosome 15 rearrangement), and four had isolated Pierre Robin sequence. Two patients had dehiscence of their initial repair within 2 weeks, for a primary surgical success rate of 82%, consistent with other published reports. A detailed follow-up of these patients revealed that 10 of 11 (91%) required additional intervention for airway or feeding issues. Of those requiring secondary intervention, an additional 1.9 secondary procedures were performed per patient to achieve control of the airway, feeding, and orthognathic problems. More than half of the patients needed gastrostomy tubes to overcome feeding difficulties. Five patients necessitated secondary surgery for recurrent airway obstruction within 4 months of the initial surgical treatment (four distractions and one repeat tongue-lip adhesion). Four others required distraction at an older age for orthognathic indications. Two patients were successfully treated with tongue-lip adhesion only. Tongue-lip adhesion has a high initial success rate for correction of neonatal airway obstruction. However, long-term follow-up indicates a high incidence of secondary intervention requirements. In retrospect, perhaps tongue-lip adhesion should be considered a temporizing procedure for most patients with Pierre Robin sequence.

A wide variety of disease processes produce alteration of midfacial skeletal growth, resulting in moderate-to-severe midface deficiency presenting as retrusion associated with Angle's class III malocclusion. Le Fort III osteotomies with advancement can provide an excellent tool for correction of this deformity. Recently, the corrective procedure of choice for advancement of midfacial segments has been distraction osteogenesis after osteotomy. Straight linear advancement is the most common choice for corrective movement of the midfacial segment, whether accomplished through acute surgical advancement or through the progressive distraction technique. Unfortunately, linear advancement can produce abnormal configurations, both at the nasal root and lateral orbits, regardless of the technique used. Enophthalmos, caused by orbital enlargement, may limit the advancement necessary to achieve class I occlusion. The authors have extended the utility of the Le Fort III procedure and have improved the final outcome by creating a controlled rotation advancement of the midfacial segment using distraction. The application of an existing internal distraction device is modified to control the movement of the midfacial segment in a rotation advancement path. Included in the series were 10 patients with severe midface retrusion secondary to multiple congenital syndromes, along with cleft lip and palate. The ages of the patients ranged from 6 to 14 years. An internal distraction system was used in all cases. Application of the distractor was substantially modified to simplify both fixation and removal and to produce controlled rotation advancement. The team orthodontist determined the final occlusal relationship. Percutaneous distractor drive rods were removed 4 to 6 weeks after active distraction to increase patient comfort. The distractors and all associated hardware were removed after 12 to 16 weeks of consolidation; follow-up periods ranged from 1 to 3 years. By using the modified distractor application to produce rotation advancement, the contour abnormalities at the nasal root and lateral orbit and the enophthalmos produced by linear advancement were eliminated. Significant improvement in facial contour and class I occlusion was obtained in all cases. Complications consisted of near exposure of the device in one patient. Stability has been excellent, with no relapse reported by the orthodontist. Rotational advancement of facial segments by distraction allows successful early intervention in patients with significant midface retrusion. The abnormal nasal root and lateral orbital configurations produced by direct linear advancement are avoided, and a stable and normalized facial configuration is produced. (Plast. Reconstr. Surg. 111: 1789, 2003.)

Over the past 5 years, the authors developed an application of mandibular distraction osteogenesis to eliminate existing tracheostomy. That experience led the authors to attempt mandibular distraction osteogenesis in neonates as an alternative before tracheostomy. Success with this approach using supporting objective airway measurements has been reported previously. This report includes six neonates diagnosed with Pierre Robin sequence. Of the six, five neonates ranging in age from 6 to 26 days (mean, 14.5 days) were treated by the authors with mandibular distraction over a 22-month period. The sixth neonate was treated with tracheostomy, because of other airway abnormalities. Findings included retrognathia, glossoptosis, incomplete cleft palate, and airway obstruction in each patient. Birth weights ranged from 2.8 to 3.2 kg. All patients were unable to control their airway during feeding, as evidenced by repeated episodes of choking and obstruction. Resting oxygen saturations were in the 70 to 80 percent range in all patients, with further deterioration during attempted feeding. Bronchoscopy was performed in all patients under anesthesia before distraction. Recurrent near-complete and intermittent complete airway obstruction were present in all patients at the level of the tongue base. There was a consensus by a pediatric intensivist, a pediatric anesthesiologist, and a pediatric otolaryngologist in all cases. Each patient met all criteria requiring ventilation for life support. Tracheostomy would be required if mandibular distraction osteogenesis was not performed, or if it failed. Patients with other airway abnormalities were not considered for treatment. Maxillomandibular disharmony measured at the midline ranged from 8 to 15 mm (mean, 11.2 mm). Active distraction was performed at the rate of 1 to 2 mm a day, with a consolidation period of 4 weeks. Total time of treatment was less than 6 weeks in all cases. All patients were extubated by the completion of active distraction. Distraction distance ranged from 8 to 15 mm (mean, 12.4 mm). All patients were discharged to home on apnea monitors, the use of which was discontinued after 90 days with no further apneic events. Weight gains met or exceeded the average 500 g a month after distraction. Bronchoscopy at the time of distractor removal showed correction of airway obstruction at the tongue base. Radiographs showed bilateral ossification of the distraction sites. Tracheostomy was avoided in all cases selected for treatment by distraction. Patient follow-up range was 9 to 22 months. In selected Pierre Robin sequence patients with tongue base airway obstruction, mandibular distraction osteogenesis can successfully avoid the need for and the associated mortality and morbidity of indwelling tracheostomy. (Plast. Reconstr. Surg. 109: 896, 2002.)