Primary Abbe Flap for Median Cleft Lip Deformity: New Trends on an Old Concept.

OBJECTIVE: The authors present our experience using primary Abbe flaps for median cleft lip. METHODS: The records of 9 patients who underwent primary Abbe flaps were reviewed for indications, operative details, and complications. Flap design is discussed, including the "W" method to facilitate columellar reconstruction. Maxillomandibular fixation was not used. RESULTS: From 2004 to 2015, 9 patients with median cleft lip received primary Abbe flaps. Mean age was 10.2 ± 6.2 months. Lobar holoprosencephaly was confirmed by imaging in 5 of the 9 patients. Operative time averaged 70 ± 22 minutes. Flap division was performed at a mean of 3.6 ± 0.9 weeks. Patients were followed for a mean of 45 ± 49 months. No flaps were lost to vascular compromise and no dehiscences or infections were noted after initial repair. Five patients required prolonged intubation ranging from 4 to 7 days and 3 required transition to gastric feedings postoperatively. One patient with holoprosencephaly expired for reasons independent of surgery. Four patients have undergone 5 lip revisions to date. CONCLUSIONS: Patients with holoprosencephaly-spectrum disorder and median cleft lip have more favorable prognoses today. The Abbe flap may be safely and advantageously used for the primary repair of median cleft lip with extreme prolabial tissue deficiency.

Disappearing dermoid: fact or fiction?

Dermoid cysts are common periorbital lesions. They usually present early in life as mobile, palpable, well-circumscribed masses near the lateral brow. Transcranial extension of a superficial, nonmidline, periorbital dermoid cyst is rare. We report 2 cases of dermoid cysts of the lateral brow that were originally recognized during the newborn period. Over time, they appeared to be clinically resolved, but they presented years later with full-thickness bone erosion and transcranial extension. Excision was performed via a combined extracranial and intracranial approach. The treatment, pathophysiology, and natural history of orbitofacial dermoid cysts are reviewed.

Immediate mandibular distraction in mandibular hypoplasia and upper airway obstruction.

Distraction osteogenesis of the mandible has become an alternative to tracheostomy in infants and children who present with upper airway obstruction due to micrognathia. To avoid prolonged intubation during distraction, we have implemented a protocol of immediate distraction at the time of distractor placement, which results in acute airway improvement. Over 2 years, 22 patients with micrognathia and severe airway obstruction have undergone mandibular distractor placement. Indications for surgery were apnea and desaturations with feeding. Resorbable distraction devices were placed bilaterally and activated to 5 to 8 mm. Recombinant human bone morphogenetic protein 2 was placed in the gap. Distraction was implemented at postoperative day 2 at 2 mm/d. Forty-four distraction devices were placed in 22 patients (68% male, 32% female) with a mean age of 24.1 months (range, 3 days to 5.5 years). The average distance of distraction performed in the operating room was 5 mm. The average total distraction was 24 mm performed over 12 days. Overall, 89% of patients were extubated after distractor placement in the operating room. Two patients with difficult intubations were extubated 7 days later in the operating room with otolaryngology. Of the 4 tracheostomy patients, 1 patient was decannulated, whereas 3 patients are pending postoperative sleep studies. One patient had a minor wound complication. Tracheostomy and prolonged intubation in patients with mandibular hypoplasia have significant morbidity and mortality. We have implemented a successful protocol of immediate distraction in the operating room with placement of bone morphogenetic protein. Immediate distraction appears to be an effective method of avoiding postoperative intubation and tracheostomy.

Maxillary advancement in patients with severe maxillary hypoplasia: role of adjuvant stabilization techniques and materials.

BACKGROUND: Maxillary advancement in patients with orofacial clefts or craniofacial syndromes can be technically challenging. We present our experience with the use of adjunctive stabilization materials and techniques in patients undergoing single-stage maxillary advancements of more than 6.0 mm. METHODS: A retrospective review was performed of 25 patients who underwent maxillary advancements of more than 6 mm with the use of specially manufactured rigid fixation plates, allograft, tongue-and-groove locking osteotomies, and bone morphogenic protein. No patients had intermaxillary fixation or postoperative external stabilization. There were 19 patients with orofacial clefts and 6 with a craniofacial syndrome, 13 male and 12 female patients, aged from 14 to 60 years with a mean age of 19 years. Six cases represented secondary operations. The maxilla was advanced at a range of 6.0 to 17.0 mm, mean of 9 mm. All patients were followed up by the same orthodontist preoperatively and postoperatively. Patient charts were reviewed for complications, relapse, malunion, and nonunion. RESULTS: There was 1 complication requiring embolization for bleeding. Three patients (12%) developed velopharyngeal incompetence postoperatively. There was no evidence of relapse, malunion, or nonunion. No patients required a second advancement. CONCLUSIONS: The combination of specialized rigid fixation plates, allograft, tongue-and-groove locking osteotomies, and bone morphogenic protein was found to be safe and effective in preventing significant surgical relapse even after significant maxillary advancements.

Does use of bupivacaine-soaked pledgets aid in the care of postoperative cleft palate patients?

UNLABELLED: Bupivacaine-soaked pledgets have been shown to decrease postoperative pain in patients requiring iliac crest harvesting, even with the use of field blocks. This finding was applied to cleft palate repair. METHODS: A retrospective review of cleft palate cases over a 1-year period, 6 months with hemostatic pledgets, and 6 months with bupivacaine-soaked hemostatic pledgets. Demographic data, postoperative analgesic use, time to feeding and discharge, and complications were gathered. The study was limited to isolated primary palate repair in non-syndromic patients. RESULTS: There were a total of 85 total primary palate patients. Exclusion of patients with concomitant procedures and syndromic patients left 18 patients in the control group and 16 patients in the bupivacaine group. Morphine (0.26 v 0.22 mg/kg), acetaminophen (9.5 v 6.4 mg/kg) and acetaminophen with codeine (0.86 v 0.75 mg/kg) were not significantly different. Time to first significant oral feeding was 12.4 v 11.7 hrs, time to discharge 27 v 31 hours. There were no postoperative hemorrhages or infection. CONCLUSION: There was no significant decrease in postoperative analgesic use, time to first significant oral intake feeding or discharge. There was no post operative bleeding or infection. Bupivicaine soaked pledgets do not significantly decrease postoperative analgesic use or shorten time to first or sustained feeding. However the use of hemostatic pledgets may decrease postoperative infection and bleeding rates.

Use of hydroxyapatite cement in pediatric craniofacial reconstructive surgery: strategies for avoiding complications.

The emergence of powdered hydroxyapatite (HA) has dramatically expanded the repertoire of reconstructive tools for craniofacial surgeons. Although several groups have reported mixed success using HA powder, to date there have been few large series retrospective reviews of HA use in an exclusively pediatric population. The purpose of our study was to assess the incidence of complications using a single surgeon's experience in an entirely pediatric cohort of patients. We present 78 pediatric patients (mean age, 9 [SD, 5.1] years; 26 girls, 52 boys) who have undergone craniofacial reconstruction with HA powder in the form of Mimix or BoneSource (mean, 57.3 [SD, 27.9] g). From this cohort, we discuss in detail 7 patients who experienced complications after their reconstruction with HA. These complications were related to trauma, infection, and exposure. Of these 7 patients with problems, 6 required eventual HA removal. In addition, all 7 patients who had complications after HA reported having a traumatic event with respect to their cranial reconstruction before presenting with a complication. Our series concludes that HA powder is a safe and effective material for use in pediatric craniofacial reconstruction. When certain principles are not violated, meticulous hemostasis and surgical planning are of paramount importance to reduce the incidence of seroma, exposure, and infection of the overlying HA placement. However, should these complications arise, prompt removal of HA is usually necessary.

Prevention and correction of airway compromise in rhinoplasty.

Recognition of patients who are at high risk for developing postrhinoplasty airway obstruction is essential. These patients may be at risk because of certain anatomic characteristics or may have a preexisting degree of airway obstruction from previous nasal surgery. Every surgeon who performs esthetic or reconstructive rhinoplasty procedures should be familiar with anatomic variants predisposing to airway obstruction and surgical techniques for airway improvement. The author presents case examples illustrating these principles and his methodology for recognizing and correcting airway compromise in primary and secondary rhinoplasty procedures. The use of spreader grafts, columellar graft, batten grafts, and piriform aperture widening are discussed. The author advocates incorporating these techniques to enhance nasal esthetics and function.

Crouzon syndrome.

BACKGROUND: Crouzon syndrome is a rare genetic disorder characterized by distinctive malformations of the skull and facial region. Premature cranial suture closure is the most common skull abnormality. Optic disc edema and proptosis are among the most common ocular findings. CASE REPORT: We present a case of a 5-year-old girl with Crouzon syndrome displaying classic facial abnormalities along with proptosis and papilledema. The child's condition was improved dramatically after a monoblock advancement procedure. CONCLUSIONS: The differential diagnosis of the condition and treatment options are discussed. The referring optometrist can play an integral role in the multidisciplinary care the patients require.

Mid-Term Dental and Nerve-Related Complications of Infant Distraction for Robin Sequence.

BACKGROUND: Mandibular distraction is effective for relieving airway obstruction in Robin sequence; however, mid-term dental and nerve-related complications have not been adequately studied. METHODS: Records were reviewed for patients with a single distraction in infancy using internal devices. Follow-up was 5 years or longer. Craniofacial dysmorphic syndromes and those affecting facial nerve function were excluded. Part I involved a review of dental records, whereas Part II involved assessment of inferior alveolar and marginal mandibular nerve function in returning patients with the use of 1,1,1,2-tetrafluoroethane cold stimulation and photography, respectively. RESULTS: Eighty-five patients met inclusion criteria. Dental records were complete in 44 patients (median follow-up, 7.3 years; range, 5.4 to 13.2 years). First permanent molar injury was seen in 42 of 88 half-mouths (48 percent); 32 of 42 (76 percent) were restorable. Primary second molar damage and ankylosis were observed in 12 of 88 (14 percent) and one of 88 half-mouths (1 percent), respectively. Mandibular second premolar absence was noted unilaterally in eight of 36 patients (22 percent) and bilaterally in six of 36 patients (17 percent). A mean 1.2 ± 0.95 operative rehabilitations were required. Nerve testing was completed in 20 patients (median follow-up, 8.7 years; range, 5.5 to 13.2 years). Complete absence of cold sensation was noted in one of 40 half-mouths (2.5 percent), whereas lower lip depressor weakness was seen in six of 40 half-mouths (15 percent). CONCLUSIONS: Infant distraction is highly successful in averting tracheostomy; however, dental and nerve-related complications remain underreported. Regular follow-up with a pediatric dentist and early recognition of injury is essential. Although inferior alveolar nerve injury appears infrequent (2.5 percent), permanent lower lip depressor weakness is more common than previously reported (15 percent of sides). CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Resorbable bone distraction: current status and future directions.

Distraction osteogenesis of the craniofacial skeleton has been one of the most exciting developments of the last decade. Parallel development of new biocompatible and resorbable materials inspired the authors to combine these technologies to create a new class of single-stage resorbable bone distraction devices. The authors present their experience to date in 50 patients whom have undergone distraction osteogenesis using resorbable devices.

Congenital nasal pyriform aperture stenosis: diagnosis and treatment.

Congenital nasal pyriform aperture stenosis is an unusual form of nasal airway obstruction in the neonate. Pediatric plastic surgeons are often involved in the management of these children and should recognize this condition and know the treatment options. Fifteen cases of children with congenital nasal pyriform aperture stenosis were reviewed for presentation of the disorder, management, and effectiveness of treatment, making it the largest series to date. There were nine male patients and six female patients in the series. They all experienced varying degrees of nasal obstruction at birth and were managed on the basis of the severity of their symptoms. Twelve patients were treated surgically in the first year of life, with a mean age at operation of 97 days (range, 3 to 362 days). Two patients required surgical intervention during their teenage years (age, 14 and 18 years) because of persistent symptoms, and one patient (age, 2 years) with mild symptoms was managed medically. Associated craniofacial anomalies were present in six cases (40 percent). Surgical enlargement of the pyriform aperture was successfully performed through an upper buccal sulcus incision in 14 patients. Preoperative symptoms of upper airway obstruction were improved in all patients at an average follow-up of 2.4 years (range, 1 month to 5 years). Congenital nasal pyriform aperture stenosis varies in presentation and severity, occurring either as an isolated congenital anomaly or in association with developmental craniofacial anomalies. It can be effectively managed by surgical enlargement of the pyriform aperture without significant recurrence or long-term morbidity.

An outcome evaluation of sphincter pharyngoplasty for the management of velopharyngeal insufficiency.

Sphincter pharyngoplasty is frequently used for the management of children with velopharyngeal insufficiency. The purpose of this study was to evaluate outcome and revision rates of sphincter pharyngoplasty at the authors' institution. Two hundred fifty patients underwent sphincter pharyngoplasty for velopharyngeal insufficiency between January of 1987 and March of 2001. There were 117 female patients and 133 male patients, with a mean age at primary sphincter pharyngoplasty of 7.6 years (range, 1 to 45 years). Diagnoses included velopharyngeal insufficiency alone (n = 63), velopharyngeal insufficiency associated with cleft palate (n = 127), velocardiofacial syndrome (n = 32), submucous cleft (n = 15), and other (n = 13). Pharyngoplasty revision was defined as any secondary surgical revision of the sphincter as determined by clinical evaluation and objective speech assessment. The pharyngoplasty revision rate was found to be 12.8 percent (n = 32). A favorable outcome was demonstrated in 30 of these patients (93.8 percent) after pharyngoplasty revision. Two patients, one with a diagnosis of a submucous cleft and velocardiofacial syndrome and the other with a cleft palate, required a second revision because of persistent velopharyngeal insufficiency. The revision rate was highest in those patients with velocardiofacial syndrome (21.8 percent) and lowest in patients with velopharyngeal insufficiency alone (6.3 percent). Patients who required revision had significantly higher preoperative oral sentence nasometry (55.2 percent versus 46.1 percent; p < 0.01) and larger velopharyngeal areas (23.7 mm2 versus 18.9 mm2). There was no significant difference in age or sex for those patients who required a revision compared with those who did not require revision. Mean follow-up was 2.4 years (range, 4 months to 13.6 years). Sphincter pharyngoplasty is an effective procedure for the treatment of velopharyngeal insufficiency using revision rate as the standard of success. It had an 87 percent primary success rate that increased to 99 percent after a single revision. Patients with velocardiofacial syndrome, more severe preoperative hypernasal resonance, and larger velopharyngeal areas were more likely to require pharyngoplasty revision.

American society of maxillofacial surgeons outcome study: preoperative and postoperative neurodevelopmental findings in single-suture craniosynostosis.

The purpose of this study was to prospectively determine the neurodevelopmental effects associated with single-suture, nonsyndromic craniosynostosis before and after surgery. Children diagnosed with single-suture craniosynostosis were evaluated by a psychologist using the Bayley Scales of Infant Development-Second Edition (BSID-II) within 2 months before and again 1 year after surgical correction. The BSID-II is a widely used measure of infant cognitive and motor development. The scale consists of three parts, the Mental Developmental Index (MDI), the Psychomotor Developmental Index (PDI), and the Behavior Rating Scale. The MDI and PDI yield age-standard scores (mean, 100; SD, 16). The children ranged in age from 2.5 to 10 months at the time of the craniofacial reconstruction (average age, 5.9 months). Metopic synostosis was diagnosed in 23 percent, sagittal synostosis in 45 percent, and unilateral coronal synostosis in 32 percent of patients. Twenty-two patients were evaluated preoperatively, of whom 15 patients were evaluated postoperatively. Mean baseline BSID-II scores revealed a mild delay in mental and motor scores (MDI, 82.3; PDI, 79.5). Mean postoperative BSID-II scores still revealed a mild delay in mental scores but significantly improved motor scores (MDI, 79.3; PDI, 89.3). Of the 15 children, four (27 percent) had BSID-II evaluations that were in the average range for all scales and nine infants (60 percent) had at least one MDI or PDI score in the significantly delayed range (<70). Among children with single-suture nonsyndromic craniosynostosis, mean Bayley scores indicated mild baseline deficits in both mental and motor scores. After surgical treatment, improvement was seen in the motor scale. It appears from this sample that neurodevelopmental abnormalities may be present in children with single-suture synostosis, and some may persist at 1 year of follow-up.

Resorbable distraction of the mandible: technical evolution and clinical experience.

The author has used various designs of single-stage resorbable distractors to lengthen the mandible in 100 patients aged 7 days to 16 years (mean, 4.24 y; 49 girls, 51 boys) with predictable results and minimal morbidity since 2002. The range of distraction was 15 to 30 mm (mean, 25.4 mm). Indications for surgery included mandibular hypoplasia associated with Pierre Robin sequence, craniofacial microsomia, Treacher Collins, and Nagers syndrome. The history of the development of resorbable polymers for use in craniofacial surgery and the evolution of distraction osteogenesis are reviewed. The melding of these 2 innovative technologies has led to the development of a new class of single-stage resorbable devices. These devices are quite different from the titanium distraction devices that have been used in clinical practice for more than 15 years. Through continued clinical application, the surgical methodology for resorbable distraction has been refined and simplified, although still-evolving continued experience with resorbable distraction has greatly decreased operative time and improved results.

Measurement of torque during mandibular distraction.

In a prospective study, 26 patients aged 9 days to 12 years old underwent mandibular distraction. There were 18 bilateral and 8 unilateral distractions performed. Five patients had previous distraction. Torque measurements were performed during the distraction process. A modest linear increase in torque was noted during the distraction process. Older patients required more torque to achieve the same distraction length as younger patients. The results of this study suggest that distraction forces are relatively modest, which may allow for greater freedom of distractor design.

Surgical treatment of the nasal-maxillary complex in adolescents with cleft lip and palate.

Rather than treating nasal, maxillary, and soft tissue deformities as separate problems, the author has approached the deformities as a single aesthetic and functional unit, the nasal-maxillary complex. This complex includes the maxilla, nose, and overlying soft tissues, including the upper lip. Successful reconstruction is based on a thorough knowledge of the underlying anatomy and physiology of these structures. Treatment of nasal-maxillary complex deformities in adolescents represents the final stages in a lifetime of reconstructive procedures. A team approach is preferred that includes a craniofacial surgeon, orthodontist, dentist, prosthodontist, and speech and language pathologist. The author's personal philosophy is based on sequential reconstruction of the underlying nasal-maxillary bony base. This is followed by reconstruction of the internal/external nasal complex and final soft tissue reconstruction. These principles are presented along with case examples.

Intralesional laser therapy treatment for hemangiomas: technical evolution.

The authors have treated over 500 consecutive pediatric patients with voluminous hemangiomas (thickness of over 10 mm), since 1996. They were all treated with intralesional laser therapy using the potassium, titanyl, phosphate (KTP) laser. Since the initiation of KTP laser therapy for deep hemangiomas in 1996, the authors have significantly modified their treatment regimen. Changes from our original treatment protocol include lower power settings, and decreased treatment intervals. Additionally, we are now simultaneously treating the deep component, using intralesional KTP laser, and the superficial component using a pulsed dye laser. Fibrosis associated with intralesional therapy has been greatly decreased by injecting small amounts of dilute steroid solution during treatment of the deep component. While maintaining the efficacy of the procedure, we have been able to greatly decrease complications associated with it. The authors detail their current techniques as well as the evolution and rationale for modifying the original treatment regimen.

Surgical correction of velopharyngeal insufficiency in children with velocardiofacial syndrome.

BACKGROUND: The surgical management of velopharyngeal insufficiency in children with velocardiofacial syndrome is inherently more difficult, with the need for revision being high. The purpose of this report was to evaluate and document the authors' experience with sphincter pharyngoplasty in the management of velopharyngeal insufficiency in children with velocardiofacial syndrome, and compare outcome. METHODS: In part I, 32 patients with velocardiofacial syndrome underwent sphincter pharyngoplasty for velopharyngeal insufficiency between January of 1987 and March of 2001. There were 18 girls and 14 boys, with a mean age at primary sphincter pharyngoplasty of 6.7 years. Pharyngoplasty revision was defined as any secondary surgical revision of the sphincter as determined by clinical evaluation and objective speech assessment. In part II, comparisons were made to 218 non-velocardiofacial syndrome patients with velopharyngeal insufficiency who underwent sphincter pharyngoplasty (cleft palate, n = 127; velopharyngeal insufficiency alone, n = 63; submucous cleft, n = 15; other, n = 13). There was no significant difference in the average age or gender in the two groups. All patients underwent screening of velopharyngeal function, which included perceptual speech evaluation, clinical screening of velopharyngeal closure, and oral examination. RESULTS: In part I, success of the primary sphincter pharyngoplasty was demonstrated in 78 percent of the velocardiofacial syndrome patients (n = 25), with a revision rate of 22 percent. Patients who required revision were slightly older, 8.6 versus 6.3 years (p = not significant). Preoperative nasometry scores were significantly higher in patients who required a pharyngoplasty revision (69 versus 54; p = 0.002). Patients who required revision of the pharyngoplasty were more likely to have larger velopharyngeal areas (30 mm versus 22 mm). In part II, the revision rate in patients with velocardiofacial syndrome was significantly higher than in those patients in the original cohort without velocardiofacial syndrome (22 percent versus 11 percent; p < 0.05). Preoperative objective speech data demonstrated significantly greater velopharyngeal incompetence in all categories (nasometry scores, pressure flow measurements, and radiographic measurements) for patients with velocardiofacial syndrome, and age at initial sphincter repair was slightly older (8.5 versus 7.7 years; p = not significant). CONCLUSIONS: The management of velopharyngeal insufficiency using sphincter pharyngoplasty in children with velocardiofacial syndrome is safe and effective. The higher need for surgical revision in velocardiofacial syndrome patients is most likely attributable to a greater degree of preoperative nasalance and a slightly later age of presentation. This should provide insight into various technique modifications in an attempt to minimize pharyngoplasty revision.

Mandibular distraction osteogenesis in Pierre Robin sequence: application of a new internal single-stage resorbable device.

Pierre Robin sequence may result in physiologically significant obstructive apnea in the neonatal and infant period. This may be life threatening and is most often treated by tracheostomy. To avoid tracheostomy or allow for early decannulation in severely affected infants and children, the authors have developed a new class of neonatal and infant mandibular bone distraction devices. These devices require a single operative procedure for placement and no operative removal is necessary. Fifteen infants (aged 7 days to 11 months; mean age, 3 months) and five children (aged 2 to 8 years; mean age, 5.5 years), 10 boys and 10 girls, with severe obstructive apnea and Pierre Robin sequence were treated with the mandibular infant devices over a 24-month period. Tracheostomy was avoided in 14 patients, whereas five of six patients who had previous tracheostomy were decannulated after mandibular distraction. The final tracheostomy status in one patient will be determined after surgery for gastroesophageal reflux. There were no major complications and no structural device failures.