Frank L. Rimell

Pediatric Respiratory Papillomatosis: Prognostic Role of Viral Typing and Cofactors

Children with recurrent respiratory papillomatosis vary greatly in their clinical disease course. Many have mild disease with eventual remission while others present with an early aggressive airway obstructive course. This study consisted of 24 pediatric patients whose specimens underwent polymerase chain reaction analysis for cytomegalovirus (CMV), herpes simplex virus (HSV), and human papillomavirus (HPV) type. Nineteen of 24 specimens contained enough DNA for this study. None of the specimens were found to contain DNA from HPV‐16, ‐18, ‐31, ‐33; CMV; or HSV, which contrasts with our previous findings in adults. Ten patients were infected by HPV‐11 and seven of these underwent tracheotomy because of an aggressive tumorigenic clinical course. Nine patients were infected by HPV‐6 alone of whom only two required a tracheotomy (P = 0.05, Fishers Exact Test). The early airway obstructive course associated with HPV‐11, however, had no bearing on achieving eventual disease remission, with decannulation achieved in eight of nine children.

Cochlear implantation in infants less than 12 months of age.

OBJECTIVE Infants are diagnosed with severe to profound hearing loss at an earlier age due to the advent of universal newborn hearing screening. This offers the opportunity to provide intervention in the form of cochlear implantation at an earlier age than was previously possible. The purpose of this investigation is to evaluate the risk of cochlear implant surgery in children less than 12 months of age. DESIGN Retrospective review of children who underwent cochlear implantation before 12 months of age. SETTING Patients were identified from a database of pediatric cochlear implant patients at a tertiary care center. All patients were diagnosed with severe to profound hearing loss by otoacoustic emission and auditory brainstem response. Follow-up ranged from 2 months to 5 years. RESULTS Fourteen of 15 patients had full insertions of the electrode hardware. Less than full insertion and post-operative CSF otorrhea occurred in one patient with severe cochlear abnormalities. There were no other perioperative surgical complications. The average speech detection threshold was 27.6 dB (20-45 dB) at approximately 1-3 months post-stimulation and 25 dB (15-30 dB) at approximately 5-7 months. CONCLUSION In our experience, we feel cochlear implantation is safe for infants as young as 6 months of age. The current standard at our institution is to implant by 7 months of age for prelingual deafness as opposed to waiting additional time until 12 months of age before the brain is presented with speech.

Real-time, Cine magnetic resonance imaging for evaluation of the pediatric airway

Background Standard magnetic resonance imaging (MRI) and computed tomographic (CT) modalities are limited in their ability to image dynamic organs. New real‐time, dynamic, cine magnetic resonance imaging (CMRI) techniques have the potential to image moving structures.

Head and neck manifestations of Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome is a congenital disorder manifested by organomegaly, omphalocele, hypoglycemia, and macroglossia. We have found a significant number of these children to be at risk for upper airway obstruction during infancy or childhood. In this review of 13 children, 2 required tracheotomy during infancy for cor pulmonale caused by macroglossia. Seven of nine children older than 1 year required tonsillectomy and adenoidectomy to relieve upper airway obstruction. Although macroglossia can be a cause of airway obstruction in infants with Beckwith-Wiedemann syndrome, we have found that airway obstruction during childhood is related to tonsillar and adenoidal hypertrophy and not to macroglossia. Anterior tongue reduction is reserved for the correction of malocclusion, articulation errors, or cosmesis, whereas tonsillectomy and adenoidectomy may be curative of obstructive symptoms.

Infectious complications in pediatric cochlear implants

Objectives: Infectious complications may cause significant delay in cochlear implant device initiation and programming and be a source of additional morbidity. We reviewed our experience with infectious complications in the pediatric age group to determine specific sources that may not be seen in adults.

Diagnosis and management of pulmonary metastasis from recurrent respiratory papillomatosis.

OBJECTIVE: We sought to review the current and proposed management, as well as bring about discussion, of managing the patient with distal tracheal and pulmonary parenchymal involvement by recurrent respiratory papillomatosis (RRP). DESIGN, SETTING, AND PATIENTS: We conducted a review of 6 patients with pulmonary metastasis from RRP at 3 academic tertiary care hospitals. Interventions included surgical and medical management with antiviral, chemotherapeutic, and/or immunemodulating agents. RESULTS: Although treatment with ±–2-² interferon, isotretinoin, and methotrexate have not proved to eradicate pulmonary involvement by RRP, possible epithelial stabilization and slowing of disease progression are noted. CONCLUSIONS: The rates of distal tracheal and pulmonary metastasis as seen in our cohort were higher than previously reported. Approximately 12% of our patients with RRP have distal tracheal spread and as many as 7% of all patients with RRP at our institutions have pulmonary dissemination. Also, high suspicion for malignant conversion to squamous carcinoma in the patient with pulmonary spread should be maintained. In addition, aggressive treatment, although not proved to eradicate the pulmonary disease, should be undertaken due to the high morbidity and mortality associated with pulmonary dissemination of RRP in our cohort.

Fate of rigid fixation in pediatric craniofacial surgery

The advantages of rigid fixation in adult craniofacial surgery are well documented, and implanted hardware is not routinely removed unless specifically indicated. There is a tendency, however, to remove hardware in children because of concerns with growth restriction, plate migration, and the lack of information on the fate of miniplates when used in pediatric craniofacial surgery. It has been our practice during the past decade not to remove hardware in children unless specifically indicated. Our study included a total of 121 procedures in 96 children, with an average age of 3.9 years and an average follow-up of 5 years. We placed 375 titanium plates and 1944 screws from 3 manufacturers. Complications encountered in children with titanium plates were as follows: 5 cases of delayed growth and 1 instance of restricted growth, 4 screw migrations (none intracranial), 9 palpable plates causing pain, 3 fluid accumulations over plates, 2 cases of meningitis, and 8 instances of plate and screw removal from the above complications. Twenty-two of 96 patients (23%) had a total of 27 complications from 121 procedures (22%). There were 6 cases in which pain precipitated removal of hardware, 1 case of an excessively mobile plate, and 1 case of documented growth restriction requiring removal; therefore our overall reoperation rate for plate removal was 8%, with no intracranial plate or screw migration.

Tracheotomy in Children with Juvenile-Onset Recurrent Respiratory Papillomatosis: The Children's Hospital of Pittsburgh Experience

Despite the risk of airway obstruction, tracheotomy has been viewed with trepidation in the management of recurrent respiratory papillomatosis (RRP). The literature suggests that the injury associated with the tracheotomy site may initiate the progression of disease to the distal airway. Alternatively, patients who require tracheotomy for RRP may be predisposed to distal spread because of more aggressive disease. In an effort to clarify this issue, we reviewed the Childrens Hospital of Pittsburgh experience with 35 patients with RRP between 1984 and 1994; 13 patients received tracheotomies. Tracheotomy patients presented at a younger age with more widespread disease, often involving the distal airway prior to tracheotomy. Although distal spread occurred in 50% of patients, it was generally limited to the tracheotomy site. Overall, outcome in the tracheotomy group was satisfactory. Complications related to the tracheotomy were rare. We conclude that tracheotomy is an appropriate option for significant airway compromise in patients with RRP.

Expression of Mucins in Mucoid Otitis Media

A hallmark of mucoid otitis media (MOM, i.e., chronic otitis media with mucoid effusion) is mucus accumulation in the middle ear cavity, a condition that impairs transduction of sounds in the ear and causes hearing loss. The mucin identities of mucus and the underlying mechanism for the production of mucins in MOM are poorly understood. In this study, we demonstrated that the MUC5B and MUC4 were major mucins in MOM that formed distinct treelike polymers (mucus strands). The MUC5B and MUC4 mRNAs in the middle ear mucosa with MOM were up regulated 5-fold and 6-fold, compared with the controls. This upregulation was accompanied by the extensive proliferation of the MUC5B- and MUC4-producing cells in the middle ear epithelium. Further study indicated that the mucin hyperproduction was significantly linked to CD4+ and CD8+ T cells and/or CD68+ monocyte macrophages. It suggests that MUC5B and MUC4 expression may be regulated by the products of these cells.

Deposition of aerosolized particles in the maxillary sinuses before and after endoscopic sinus surgery.

Background Recent studies suggest that topical therapy is beneficial in many conditions underlying chronic sinusitis. Current literature has documented low aerosolized particle deposition efficiency into the paranasal sinuses. Mathematical modeling suggests that three factors influence the deposition efficiency: particle size, pressure gradient, and size of the sinus ostium. Ostium size is the most dominant factor. Therefore, we sought to determine if maxillary antrostomy and ethmoidectomy would increase the deposition efficiency. Methods Five cadavers underwent pre- and postoperative scintigraphy after administration of aerosolized Tc-99M. Images were obtained with a γ-camera and regions of interest (ROIs) were drawn around the maxillary sinuses. Counts per minute in the pre- and postoperative ROIs were then compared using the paired t-test. Results Results indicated a significant increase in deposition of radioactivity in the maxillary sinuses in the postoperative state (p < 0.01). Conclusion Topical therapy for chronic sinusitis may be more feasible in the postoperative population.