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Biomedical subjects

Fernando D Burstein

Publications and source records attributed to Fernando D Burstein.

14 recordsLinked to original sources
PubMed2006

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.

Child↗
PubMed2006

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.

Child, Preschool↗
PubMed2006

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.

Adrenal Cortex Hormones↗
PubMed2005

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.

Abnormalities, Multiple↗
PubMed2004

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.

Cranial Sutures↗
PubMed2004

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.

Absorbable Implants↗
PubMed2003

Cartilage-sparing complete otoplasty technique: a 10-year experience in 100 patients.

Otoplasty is one of the most frequently performed esthetic surgical procedures in children and adolescents. Several techniques can give satisfactory results, but few address all the components of the prominent ear deformity. The author reports on the evolution and application of a cartilage-sparing otoplasty technique that addresses all the components of the prominent ear deformity. One hundred patients (200 ears) were treated over a 10-year period. An 8% revision rate and minimal complications were encountered.

Adolescent↗
PubMed2003

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.

Adolescent↗
PubMed2003

Predicting the risk of reoperation in metopic synostosis: a quantitative CT scan analysis.

Children with metopic synostosis have a well-described clinical picture of trigonocephaly, often with hypotelorbitism. The craniofacial disorder itself is well recognized; however, objective prognostic factors for predicting the risk of reoperation are not well known. In 39 children with metopic synostosis, measurements of the cranial length, cranial width, anterior intercoronal distance, anterior interorbital distance (intercanthal distance), lateral orbital distance, and interzygomatic buttress distance were taken from preoperative computed tomographic (CT) scans and were normalized relative to each child's age. To separate overall facial hypoplasia from regional hypoplasia, a ratio of intercanthal distance to interzygomatic buttress distance was determined. These prognostic factors were analyzed with respect to reoperation rate. A stepwise logistic regression analysis was used to determine the interrelationships between the prognostic factors. Twenty-eight percent of the children underwent reoperation (N=11, 1 total reoperation and 10 minor recontouring). All of the reoperations occurred in children with a decreased intercanthal distance (p=0.30). The ratio of intercanthal distance to midfacial width was related to reoperation rate, with those children who had a ratio < or =0.80 having a reoperation rate of 44% (8 of 18 total children with an intercanthal-to-zygomatic ratio < or =0.8, p=0.07). This relationship was significant in children younger than the age of 12 months (6 of 13 total children with an intercanthal-to-zygomatic ratio < or =0.8, 46% reoperation rate, p=0.006). This study suggests that preoperative CT measurements can be used as a means of risk stratification in outcome analyses of the surgical treatment of craniosynostosis. In children treated for metopic suture synostosis, a foreshortened intercanthal distance compared with the interzygomatic buttress distance was related to reoperation rate, especially in children younger than 12 months of age.

Cranial Sutures↗
PubMed2003

Midface distraction to alleviate upper airway obstruction in achondroplastic dwarfs.

OBJECTIVE: The use of midface distraction in patients with achondroplasia and upper airway obstruction secondary to midface hypoplasia has not been reported. In this report, we review the treatment of two patients with severe midface hypoplasia and obstructive sleep apnea secondary to achondroplasia using midface distraction osteogenesis. DESIGN, SETTING, AND PATIENTS: Two patients with achondroplastic dwarfism and midface hypoplasia with airway obstruction were treated in a tertiary referral center for craniofacial disorders. RESULTS AND CONCLUSIONS: Both patients had their tracheostomies decannulated after midface distraction of 25 mm. Midface distraction osteogenesis is useful to alleviate upper airway obstruction from midface hypoplasia seen in achondroplasia.

Achondroplasia↗
PubMed2002

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.

Adolescent↗
PubMed2002

Single-stage craniofacial distraction using resorbable devices.

We report on the use of a new type of internal bone distraction devices designed for craniofacial applications. These resorbable devices allow a single operative procedure for device placement, eliminating the need for a second open operative procedure for hardware removal. We report on three models of resorbable devices. The midface orbital frontal device was used for midface and monoblock advancement. The mandibular adolescent device was used in older children and adolescents. In neonates and young children, the mandibular infant device was used. Twenty-one patients (9 female, 12 male) aged 6 days to 15 years (mean = 53 months) underwent bony expansion of the craniofacial skeleton over a 2-year period. A total of 39 devices were implanted: 32 in the mandible, 3 in the maxilla alone, and 4 in the maxilla and frontal bones. Expansion distances ranged from 15 to 30 mm. Expansion took place at 1 to 2 mm/d. Latency periods ranged from 48 to 72 hours. There were no device structural failures and no major complications.

Absorbable Implants↗