Timothy A. Lander

Airway interventions in children with Pierre Robin Sequence.

Objective To describe the interventions required for successful airway management in children with Pierre Robin Sequence (PRS). Study Design Case series. Subjects and Methods The records of both a cleft and craniofacial clinic and a pediatric otolaryngology clinic were searched, and all children with PRS were identified. Data concerning feeding interventions, airway interventions, and comorbid conditions were extracted. Results Seventy-four cases of PRS were identified. Thirty eight of the 74 children required airway intervention other than prone positioning. Fourteen of these 38 were managed nonsurgically with nasopharyngeal airway and/or short-term endotracheal intubation, whereas the remaining 24 required surgical intervention. Eighteen of the 24 underwent distraction osteogenesis of the mandible, one underwent tracheostomy, and five underwent tracheostomy followed by eventual distraction. Conclusion In our series, over 50 percent of children with PRS required an airway intervention. These were both nonsurgical and surgical. As otolaryngologists, we must be prepared for the challenges children with PRS may present and the interventions that may be necessary to successfully manage these difficult airways.

Resolving Feeding Difficulties With Early Airway Intervention in Pierre Robin Sequence

Objectives/Hypothesis: To observe rates of gastrostomy tube (g‐tube) placement in Pierre Robin Sequence (PRS) and to determine whether relieving airway obstruction solves feeding difficulties.

Correlation of PCR-detected clonal gene rearrangements with bone marrow morphology in patients with B-lineage lymphomas.

Bone marrow biopsy is the conventional staging and posttherapy evaluation method for assessing marrow involvement by lymphoma. Polymerase chain reactions (PCR) for antigen receptor rearrangements have the potential to increase the detection of minimal degrees of marrow involvement. The present study is a concurrent morphologic and PCR evaluation of 225 staging or posttherapy marrow biopsies from 127 patients with B-lineage non-Hodgkins lymphoma. The biopsies were morphologically categorized into four groups: group 1 (positive for lymphoma), 60 biopsies (27%); group 2 (suspicious for lymphoma), 20 biopsies (9%); group 3 (lymphocytic lesions of indeterminate biology), 22 biopsies (10%); and group 4 (negative for lymphoma), 123 biopsies (54%). Molecular studies were performed on concurrently obtained aspirates and used consensus immunoglobulin-heavy-chain (IgH) and IgH/bcl-2 gene PCR primers. A molecular clone was detected in 53 of the 225 aspirates (24%): group 1, 34 aspirates (57%); group 2, five aspirates (25%); group 3, one aspirate (5%); and group 4, 13 aspirates (11%). A PCR-positive aspirate was present in 47% of follicular lymphomas, 58% of diffuse large cell lymphomas, and 72% of the other lymphomas in the group I specimens. Morphology or PCR was positive in 79 of the 225 cases (35%). The molecular detection of clonality in the aspirate DNA from cases with positive morphologic findings was lower than anticipated. The discordance between morphology and PCR results may be related to sample variation between the trephine biopsy and aspirate, a failure to aspirate sufficient lymphoma cells, or insufficient primer homology for amplification. DNA extracted from trephine sections may provide results more concordant with morphology, because PCR detected a clone in 10 of 11 DNA specimens extracted from trephine biopsies with positive morphologic findings and PCR negative aspirates.

Mandibular Distraction Osteogenesis in Infants Younger Than 3 Months

OBJECTIVES To examine the long-term outcomes and complications in infants with upper airway obstruction and feeding difficulty who underwent bilateral mandibular distraction osteogenesis (MDO) within the first 3 months of life and to identify any preoperative characteristics that may predict the long-term outcome following early MDO intervention for airway obstruction. METHODS An institutional, retrospective medical chart review was performed. Inclusion criteria were bilateral MDO performed at an age younger than 3 months, with a minimum follow-up of 3 years. A quantitative outcome measures scale was developed, and patients were scored based on long-term postoperative complications as well as airway and feeding goals. Factors such as need for an additional surgical procedure were also considered. RESULTS Nineteen children were identified as having undergone MDO before 3 months of age and having more than 3 years of follow-up data. The mean age at distraction was 4.8 weeks (range, 5 days-12 weeks); the mean length of follow-up was 5.6 years (range, 37-122 months). Of these 19 patients, 14 had isolated Pierre Robin sequence (PRS) and 5 had syndromic PRS. All patients with isolated PRS had a good or intermediate long-term result. Infants with comorbidities such as developmental delay, seizures, or arthrogryposis had the poorest outcomes. CONCLUSIONS Bilateral MDO is a relatively safe and effective means of treating airway obstruction and feeding difficulty in infants with PRS. The effects of this procedure, which carries a relatively low morbidity, persist through early childhood in most patients.

Distraction osteogenesis of the mandible for airway obstruction in children: Long-term results

Objective: To determine the long-term results of distraction osteogenesis of the mandible for upper airway obstruction in children with micrognathia. Study Design: Case series with chart review. Setting: Tertiary care childrens hospital. Subjects and Methods: The records of a pediatric otolaryngology practice and tertiary childrens hospital were searched for patients treated with bilateral mandibular distraction osteogenesis for upper airway obstruction. Patients were selected if greater than three years of follow-up data were available. Data were analyzed for airway and feeding outcomes, and long-term surgical complications were identified. Results: Thirty-two patients met study criteria. Of the 11 patients who had tracheotomy prior to distraction, seven were decannulated after the procedure. Seventeen patients needed perioperative gastrostomy. Seven are now able to feed orally. Fifteen patients treated with mandibular distraction were able to avoid gastrostomy tube placement altogether. The complication of open bite deformity was experienced by nine patients (28%). Five of 32 patients (16%) had tooth malformation, tooth loss, or dentigerous cyst formation while an additional three patients (9%) had long-term facial nerve injury. Nineteen patients (59%) were under three months old at the time of their distraction. Only one of these patients (5.2%) required an additional distraction procedure. Conclusion: Long-term follow-up data on patients treated with mandibular distraction for upper airway obstruction show sustained airway improvement. Additionally, micrognathic children treated with distraction have improved outcomes in oral feeding with a relatively low rate of long-term complications. It remains important to follow these patients to monitor the need for secondary reconstructive procedures.

Airway management for intubation in newborns with Pierre Robin sequence.

To review airway management in Pierre Robin sequence (PRS) newborns undergoing general anesthesia and to determine if endotracheal intubation is safe in this population.

Sialendoscopy in children

BACKGROUND The definitive cause of most cases of recurrent salivary gland inflammation in children remains unknown. Relatively little has been written about the use of sialendoscopy as a diagnostic and therapeutic tool in children. OBJECTIVE To evaluate the safety and efficacy of sialendoscopy as a diagnostic and therapeutic tool for recurrent salivary gland inflammation in children. STUDY DESIGN Retrospective case series. METHODS Medical records of all patients who underwent sialendoscopy for recurrent salivary gland inflammation from a single tertiary-care pediatric otolaryngology practice were reviewed. Comparison of pre-procedure vs. post-procedure frequency and severity of disease was reviewed. Operative reports, images, and video were analyzed for causes of inflammation. RESULTS Six patients (aged 3-16 years old) underwent sialendoscopy (3/6 bilateral parotid, 2/6 unilateral parotid, 1/6 unilateral submandibular). There were no complications. No post-operative recurrence was noted in 3/6 patients; decreased frequency of recurrence was noted in 2/6 patients; repeat sialendoscopy was required in 1/6. Operative findings from sialendoscopy from 10 parotid glands showed fibrinous debris (7/10), mucoid debris (1/10), purulent debris (1/10), or duct stenosis (1/10). No stones were noted. CONCLUSIONS Sialendoscopy is a safe, minimally invasive procedure that may decrease the frequency of recurrences for salivary gland inflammation in children. In contrast to previously published work, the most common cause of salivary gland obstruction in this series was debris, rather than stones. Increased use of sialendoscopy as a diagnostic and therapeutic tool will allow for improved understanding of the causes of and management for recurrent salivary gland inflammation in children.

Complications and Satisfaction with Pediatric Osseointegrated External Ear Prostheses

To determine the complication rate and the patient satisfaction of titanium osseointegrated ear implants for congenital microtia and anotia. To discuss external ear prostheses as a treatment option for children with microtia.

Tracheal Agenesis in Newborns

Objectives/Hypothesis: A series of three newborns with tracheal agenesis is described. The preferred methods of diagnosis, description of the clinical course, and a review of the pertinent embryology, associated anomalies, and clinical management are presented.

The effect of cleft palate repair technique on hearing outcomes in children

OBJECTIVE Otitis media with effusion causing conductive hearing loss is a problem for many children with cleft palate. This study examines the association between palate repair technique and hearing outcomes in children at 3 and 6 years post-repair. PATIENTS AND METHODS Retrospective chart review of patients with all types of cleft palate that were repaired between 2001 and 2006 at a tertiary childrens hospital. Exclusion criteria included sensorineural hearing loss, ossicular chain abnormalities, and ear canal abnormalities. The primary outcome was pure tone average (PTA) from 0.5 kHz to 2 kHz. RESULTS 69 patients (138 ears) were analyzed. 30.4% of left ears and 31.9% of right ears had an abnormal (>20 dB) PTA at 3 years; at 6 years this significantly improved to 13.0% (p=0.008) and 15.9% (p=0.011). Double-reverse z-plasty was associated with the lowest median PTA of 10.0 dB (p=0.046) at 6 years. There was no difference in median PTA between children with and without comorbid diagnoses (such as Pierre Robin Sequence, arthrogryposis) at either 3 years or 6 years (p=0.075, p=0.331). Multivariate model showed that extent of cleft influenced technique choice (p=0.027), but only technique choice was associated with significant differences in PTA and only at 6 years post-repair. CONCLUSION The majority of children developed normal hearing by 6 years with palatoplasty and routine tube insertion. Double reverse z-plasty was associated with the best outcome, but is not ideal for hard palate clefts. Randomized controlled trials are needed to elucidate the relationship between technique, middle ear ventilation and time to recovery, irrespective of type of cleft.