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Biomedical subjects

J Kerwin Williams

Publications and source records attributed to J Kerwin Williams.

9 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

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↗
PubMed2004

Resorbable PLLA-PGA plate and screw fixation in pediatric craniofacial surgery: clinical experience in 1883 patients.

The need to provide rigid bony fixation in the surgical treatment of craniofacial deformities has inspired an on-going evolution of surgical innovations and implants. Because of the young age of many treated craniosynostosis patients and the unique pattern of cranial vault growth, the extensive implantation of metal devices is potentially problematic. The use of resorbable plate and screw devices offers all of the benefits of rigid fixation without many of their potential risks. Since the introduction of resorbable plate and screw devices in 1996, tens of thousands of craniofacial patients have received implants, but long-term results from a large series have yet to be reported. A combined prospective and retrospective analysis was done on 1883 craniosynostosis patients under 2 years of age treated by 12 surgeons from seven different geographic locations over a 5-year period who used the same type of resorbable bone fixation devices (poly-L-lacticpolyglycolic copolymer). Specifically, the incidence of postoperative infection, fixation device failure, occurrence of delayed foreign-body reactions, and the need for reoperation resulting from device-related problems were determined. Technical difficulties and trends in device use were also noted. From this series, significant infectious complications occurred in 0.2 percent, device instability primarily resulting from postoperative trauma occurred in 0.3 percent, and self-limiting local foreign-body reactions occurred in 0.7 percent of the treated patients. The overall reoperation rate attributable to identifiable device-related problems was 0.3 percent. Improved bony stability was gained by using the longest plate geometries/configurations possible and bone grafting any significant gaps across plated areas that were structurally important. The specific types of plates and screws used evolved over the study period from simple plates, meshes, and threaded screws to application-specific plates and threadless push screws whose use varied among the involved surgeons. This report documents the safety and long-term value of the use of resorbable (LactoSorb) plate and screw fixation in pediatric craniofacial surgery in the infant and young child. Device-related complications requiring reoperation occurred in less than 0.5 percent of the implanted patients, which is less frequent than is reported for metallic bone fixation. Resorbable bone fixation for the rapidly growing cranial vault has fewer potential complications than the traditional use of metal plates, screws, and wires.

Absorbable Implants↗
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

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↗
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

The constricted ear.

The constricted ear may be described best as a pursestring closure of the ear. The deformity may include lidding of the upper pole with downward folding, protrusion of the concha, decreased vertical height, and low ear position relative to the face. The goals of surgical correction should include obtaining symmetry and correcting the intra-auricular anatomy. The degree of intervention is based on the severity of the deformity and may range from simple repositioning, soft tissue rearrangement, or manipulation of the cartilage. Multiple surgical techniques are described.

Child↗
PubMed2002

Cryptotia: principles and management.

Adequate treatment of the cryptotic ear must address both recreation of the upper pole and correction of the vertical height. The degree of soft tissue manipulation ia dictated by the severity of the deformity. Often this must be combined with repositioning or reconstruction of the cartilaginous skeleton.

Child↗