An Ideal Multidisciplinary Cleft Lip and Cleft Palate Care Team.

We emphasize the benefits of a multidisciplinary team approach to the systemic management of patients with cleft lip and cleft palate (CL/CP) and their families. An ideal team offers support to families at each stage of their child's development and adapts to address new issues that are encountered throughout growth. We suggest the addition of a registered dietitian to the initial phase of the care team to ensure the child consumes adequate macronutrient intake, and to maximize growth and development. An ideal cleft palate care team focuses on a comprehensive, collaborative, and family-centered approach to the complex medical and surgical needs of infants with CL/CP as they grow to young adulthood. Although care teams may differ slightly depending on context and location, there are central tenets that make an ideal team. After reviewing CL/CP care team guidelines and recommendations from a variety of different countries, this article reflects what an ideal organization structure and care team composition could be comprised of.

Hydroxyurea treatment effect on children with sickle cell disease and obstructive sleep apnea.

BACKGROUND: While hydroxyurea is the mainstay of treatment for many of the comorbidities associated with sickle cell disease, its effect on obstructive sleep apnea has not been fully investigated. The purpose of this project is to help characterize the effects of hydroxyurea on obstructive sleep apnea in children with sickle cell disease and determine its therapeutic role in the condition. METHODS: Chart review was conducted on two pediatric patients with sickle cell disease who experienced resolution of obstructive sleep apnea following hydroxyurea administration. RESULTS: After undergoing approximately 11 months of hydroxyurea therapy, sleep apnea symptoms improved and obstructive sleep apnea resolution was confirmed by repeat polysomnography in both cases. This resolution was largely secondary to a reduction in the obstructive component of the apnea hypopnea index, highlighting a previously unreported association. CONCLUSIONS: As adenotonsillectomy is associated with significant risks in patients with sickle cell disease, it appears reasonable to consider a period of observation for improvement of obstructive sleep apnea following hydroxyurea administration rather than directly proceeding with surgery.

Retrospective Review of Management and Outcomes of Pediatric Descending Mediastinitis.

OBJECTIVES: To review the management and outcomes of pediatric patients treated for descending mediastinitis at a single institution and contribute to an updated mortality rate. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care pediatric hospital. SUBJECTS AND METHODS: This study is a 19-patient case series of all patients treated for descending mediastinitis at a tertiary pediatric hospital from 1997 to 2015, and it serves as an update to the case series published from this institution in 2008. Review of management included time to diagnosis, time to surgery, surgical procedures performed, and antibiotics administered. The primary outcomes measured were length of hospitalization and mortality. RESULTS: In addition to 8 previously reported patients, we identified 11 pediatric patients treated for descending mediastinitis in the period of review. All 19 patients were <18 months old, and all survived their hospitalization. Fourteen patients underwent surgical drainage at least twice. The median length of hospital stay was 15 days. Retropharyngeal abscess was the source of infection in 16 of 19 patients, and methicillin-resistant Staphylococcus aureus (MRSA) was the isolated organism in 14 of 15 positive cultures. CONCLUSION: This review represents the largest reported series of pediatric patients with descending mediastinitis. With 100% survival, our results suggest that pediatric descending mediastinitis can be safely managed by prompt surgical drainage. Broad-spectrum antibiotics covering MRSA and a low threshold for repeat surgical intervention have been an important part of our successful approach and may decrease length of stay.

Palatopharyngoplasty with bilateral buccal mucosal graft repair to alleviate oropharyngeal stenosis.

Oropharyngeal stenosis is rare, but known complication from tonsillectomy procedure. A 15-year-old female presented with refractory dyspnea, mild obstructive sleep apnea, and dysphagia. She underwent tonsillectomy 3 years prior. Severe cicatricial oropharyngeal scar involving soft palate, anterior tonsillar pillars, and base of tongue, resulted in 1-cm(2) airway. Case report describing lysis of severe palatopharyngeal scar bands from tonsillectomy misadventure with immediate buccal mucosal grafts to repair resultant oropharyngeal defects. Patient no longer complains of difficulty breathing or dysphagia.

Effects of adenotonsillectomy on polysomnographic parameters in children with sickle cell disease.

Obstructive sleep apnea (OSA) in the pediatric sickle cell disease (SCD) population can promote nightly hemoglobin oxygen desaturation, which increases the risk of central nervous system insult and may impair cognitive function. Adenotonsillectomy can ameliorate OSA symptoms, but its effect in children with SCD has not been fully investigated. We reviewed the effects of adenotonsillectomy in thirteen children with SCD by comparison of pre and post-adenotonsillectomy polysomnography (PSG) parameters. Significant reduction in hemoglobin oxygen desaturation, decreased apnea-hypopnea index, and increased rapid eye movement sleep occurred after adenotonsillectomy. Adenotonsillectomy promotes improvement in sleep quality in children with SCD and PSG-confirmed OSA.

Olaryngologic manifestations in children with chronic neutropenia.

OBJECTIVE: Chronic neutropenia of childhood (CNC) is a rare disorder in which the absolute neutrophil count is below 1500/µL over an extended period of time. The objective of this study is to describe the otolaryngolgic manifestations associated with CNC to facilitate diagnosis and treatment of this condition. METHODS AND MATERIALS: We performed a retrospective chart review of patients with the diagnosis of CNC between 1970 and 2005 at a tertiary pediatric hematology center. After Institutional Review Board approval, 43 patients were evaluated. The average age at hematologic diagnosis was 49 months (range: 1 month - 15 years with 35% <1 year, 44% 1-10 years, 21% >10 years). A total of 2049 encounters were analyzed from the hospital charts. RESULTS: Twenty four subjects (56%) presented with recurrent otitis media (ROM), sinusitis or pharyngo-tonsillitis, while 11% presented with oral mucosal lesions. After diagnosis, otolaryngologic problems persisted, including ROM (81%), viral upper respiratory tract infection (67%), oral ulcers or gingivitis (53%), tonsillitis (39%) and sinusitis (37%) and were more common than other systemic infections. Myringotomy tube placement, endoscopic sinus debridement, adeno-tonsillectomy or tracheostomy were required in 42% of patients. After G-CSF (granulocyte colony-stimulating factor) became available in the early 1990s, the infection rate markedly decreased. Five deaths occurred (12% mortality) including one due to sepsis from otolaryngologic infection. CONCLUSION: The majority of children with CNC had otolaryngologic problems at presentation and these continued after diagnosis. While managing common otolaryngologic infections in children, a high index of suspicion for chronic neutropenia is necessary. An otolaryngologist is frequently one of the first physicians to encounter children with this condition. Awareness of CNC and its management will enhance earlier diagnosis and more effective treatment for these children.

Effects of sensory or motor nerve deafferentation on oromotor function in mice.

OBJECTIVE: To investigate the effect of sensory or motor nerve damage to the tongue using a mouse model. STUDY DESIGN: Animal study. SETTING: Research laboratory. SUBJECTS AND METHODS: Adult male and female mice from inbred strains B6 (n = 19) and D2 (n = 25). Following lick training, bilateral lingual-chorda tympani nerve cuts (LX) (n = 6 B6, n = 7 D2), unilateral hypoglossal nerve cuts (HX) (n = 7 B6, n = 9 D2), or sham surgery (n = 6 B6, n = 9 D2) was performed. Mice were lick tested postsurgically with both water and sucrose (4 days total). Following testing, post mortem dissections and microscopic analysis of tongue papillae were performed. RESULTS: In both strains, HX and LX mice demonstrated a significant reduction in volume per lick (VPL) in the surgical groups relative to shams. Neither motor nor sensory nerve transection affected local lick rate. In most LX mice in both strains, taste papillae were reduced compared with HX or sham mice. CONCLUSION: Mice of either strain with either a sensory or a motor nerve injury have a significant loss of VPL during ingestion of either a neutral (water) or preferred (sucrose) stimulus. This reduction in VPL reflects a deficit in licking. Lick rate was not affected by deafferentation. A reduction in fungiform papillae following LX but not HX mice was noted.

Body mass index as an indicator of obstructive sleep apnea in pediatric Down syndrome.

OBJECTIVE: Our objective was to determine if higher body mass index (BMI) increases the likelihood of, obstructive sleep apnea (OSA) in pediatric Down syndrome (DS) patients. METHODS: We performed a, retrospective chart review of 63 DS patients evaluated by overnight polysomnography from December 1995 to February 2005. Patients aged less than 2 years were excluded. Remaining patients were grouped, according to presence (n=19) or absence (n=33) of OSA based on apnea hypopnea index (AHI). OSA, and non-OSA DS groups were age matched while blinded to patient attributes other than age and OSA, status. Patients without appropriate age matches were excluded. We recorded various patient information, including age, sex, height, weight, number of apneas, number of hypopneas, respiratory distress index (RDI), apnea-hypopnea index (AHI), lowest oxygen saturation during sleep, mean oxygen saturation, number of arousals per hour, and mean time spent in REM sleep. We calculated BMI using the, standard kg/m(2) formula and converted this into a Z-score. RESULTS: Fifty-two DS patients were analyzed with average age of 9.3+/-4.5 years (10.2+/-4.2 in 33 OSA patients, 7.8+/-4.3 in 19 non-OSA patients). There were 28 males and 24 females. The OSA group mean BMI Z-score was 2.09+/-0.94, and the non-OSA group Z-score was 1.4+/-1.40. The Z-scores for BMI were statistically significant between OSA and non-OSA patients with p=0.03 by t-test. CONCLUSIONS: When age and sex adjusted, BMI has a statistically significant association with the presence of OSA in Down syndrome patients. The incidence of OSA also increases with increasing age in this population.

Effects of adeno-tonsillectomy on polysomnography patterns in Down syndrome children with obstructive sleep apnea: a comparative study with children without Down syndrome.

OBJECTIVE: To determine if adeno-tonsillectomy (T&A) in children with Down syndrome (DS) improves breathing, measured by apnea hypopnea index (AHI), rapid eye movement AHI (REM-AHI) and the lowest oxygen desaturation (SaO2), and sleep disruption, measured by arousal index (ArI) and time spent in stages 1-4 and rapid eye movement (REM) sleep and compare these results with a group of non-DS children with obstructive sleep apnea (OSA). STUDY DESIGN: Retrospective chart review at pediatric sleep center. PATIENTS: Eleven DS and nine non-DS children underwent pre- and post-T&A polysomnography between 1997 and 2005. OUTCOME MEASURES: Pre- and post-T&A polysomnography parameters were compared using paired t-test and independent samples test. RESULTS: Mean age in DS group was 101 months and non-DS group was 80 months (64% males in DS and 88% in non-DS group). The average BMI was 29.8 and 27.6 for DS and non-DS group. The total AHI showed significant improvement after T&A but this was not as marked as the non-DS group. REM-AHI and lowest SaO2 did not show significant change in the DS children. The non-DS group showed significant improvement in all respiratory parameters. Both groups showed mild improvement in sleep parameters. With the modest overall improvement, 27% of the DS children required no further treatment. However, 73% required CPAP, BiPAP or oxygen for persistent OSA. CONCLUSION: This study supports the fact that T&A in DS children improves some parameters of OSA, however not as markedly as in non-DS children.

Cleft palate lateral synechia syndrome: an opportunity for unique surgical closure.

OBJECTIVES: To report two cases of cleft palate lateral synechia (CPLS) syndrome in a single family and describe surgical closure using the synechia. STUDY DESIGN: Case report and literature review. METHODS: A case report is presented with a review of the literature of cleft palate in conjunction with lateral synechia. Clinical presentation with photographic images of surgical repair is presented as well as a genetic workup with pedigree. RESULTS: A 6-week-old male presented for evaluation of a cleft palate. Mucosa-lined, fibromuscular tissue bands were noted connecting the floor of mouth and the free edges of a bilateral complete secondary cleft palate. No other craniofacial, digital, genital or limb defects were noted. The patient's 13-month-old sister had similar synechial bands and cleft palate at birth. Another older sibling had cleft palate without synechia. The patient's mother and maternal great grandmother had cleft palates at birth without synechia. The three children share a common mother but have three different fathers. Genetic analysis failed to reveal chromosomal defects or a mutation in the interferon regulatory factor 6 (IRF6) gene, a locus linked to Van der Woude syndrome. At 2 years of age, the index patient was growing and feeding well. His intra-oral bands remained intact and were incorporated in the surgical repair using a novel approach. CONCLUSIONS: Since more otolaryngologists are performing cleft surgeries, the awareness of the differential diagnoses associated with a cleft palate is important. CPLS is an extremely rare condition. The report of this family supports the suspected pattern of autosomal dominant inheritance with variable expressivity. The unusual surgical approach will be discussed.

Airway evaluation and management in 7 children with malignant infantile osteopetrosis before hematopoietic stem cell transplantation.

Malignant infantile osteopetrosis (MIOP) is a rare disorder caused by dysfunctional osteoclasts. The classic MIOP features, such as frontal bossing, micrognathia, and small thorax, may place these children at risk for developing obstructive sleep apnea (OSA) and chronic hypoxemia. To objectively document OSA, airway evaluations were performed; results impacted management. We reviewed the records of 7 MIOP patients treated at St Jude. Six underwent polysomnograms during prehematopoietic stem cell transplantation (HSCT) evaluation. To determine the existence of a relationship between OSA and radiologic imaging, initial chest radiographs and bone mineral density studies were reviewed. Pre-HSCT patients had a median apnea-hypopnea index of 17.51 (normal, 0 to 2), with <25% being central events, thus indicating OSA. The median minimal oxygen saturation was 79%, indicating intermittent hypoxemia. Neither chest radiographs nor bone mineral density correlated with severity of OSA. Four patients received tracheostomies before or during HSCT. Three surviving children underwent polysomnograms 1 year after HSCT, and median apnea-hypopnea index was 1.3, indicating near to complete resolution of OSA. Resolution of OSA may have been multifactorial. Using a quantitative approach, we demonstrate that MIOP children have OSA and hypoxemia; thus, these children should have airway evaluations and treatments to potentially reduce the risk of life-threatening pulmonary complications.

Deep neck infection.

Deep neck infections present significant morbidity and mortality, particularly when associated with predisposing factors that impair a functional immunologic response. Familiarity with deep neck spaces and fascial planes is critical, because these form the basis for the emergent nature of the disease process. Common and potentially life-threatening complications include airway obstruction, jugular vein thrombosis, descending mediastinitis, sepsis, acute respiratory distress syndrome, and disseminated intravascular coagulation. The most common primary sources of deep neck infection are odontogenic, tonsillar, salivary gland, foreign body, and malignancy. Microbiology typically reveals mixed bacterial flora, including anaerobic species, that can rapidly progress to a fulminating necrotizing fasciitis. The treatment cornerstone remains securing the airway, providing efficient drainage and appropriate antibiotics, and improving immunologic status. A prolonged hospital stay should be anticipated.

Pediatric mediastinitis as a complication of methicillin-resistant Staphylococcus aureus retropharyngeal abscess.

OBJECTIVE: To examine changes in the incidence, bacteriology, and complications of retropharyngeal infection (RPI) over an 8-year period. DESIGN: Retrospective medical record review. SETTING: Tertiary children's hospital. PATIENTS: The study population comprised 108 patients younger than 18 years old. INTERVENTION: Medical record review of patients with a discharge diagnosis of RPI (International Classification of Diseases, Ninth Revision code 478.24). MAIN OUTCOME MEASURES: Cases from June 1997 to May 2001 were compared with those from June 2001 to May 2005 to examine changes in the incidence, bacteriology, and complications of RPI. RESULTS: The number of RPI cases doubled from 36 to 72 in the final 4 years. In the first 4 years, no isolates of methicillin-resistant Staphylococcus aureus (MRSA) were found, and 1 patient developed mediastinitis. In the last 4 years, 8 of 25 patients (32%) with positive cultures had MRSA isolated, and 7 cases of mediastinitis occurred. Of the 8 children with cultures positive for MRSA, 6 developed mediastinitis. The median age for all children with RPI was 32.5 months (n = 108). The median age for children with MRSA and mediastinitis was 6.5 months (n = 8) and 5.5 months (n = 8), respectively. CONCLUSIONS: An alarming increase in the number of RPI cases occurred over the final 4 years. Methicillin-resistant S aureus is now a significant pathogen in patients with RPI at our institution. Documented local increases in community-associated MRSA infections and universal sensitivity to clindamycin suggest that community-associated MRSA is responsible for the change in bacteriology. A high correlation exists between MRSA infection and mediastinitis. Patients with MRSA infections are younger and may be vulnerable to developing mediastinitis because of immature immune systems. A higher index of suspicion is needed for MRSA, especially in patients younger than 1 year.

Aural involvement in loxoscelism: case report and literature review.

An 11-year-old male presented with fever, rash, and a necrotic lesion on the lobule of the left ear. The lesion became tender and formed an eschar over 4 days. The patient developed leukocytosis, hemolytic anemia, and proteinuria, and was diagnosed with systemic loxoscelism from a brown recluse spider bite. He was managed with supportive therapy and improved in 4 days. Loxoscelism is a clinical diagnosis which should be suspected in an otherwise healthy patient with a necrotic wound, particulary in the endemic Southern and Midwestern United States. Physicians should be aware of this disease entity and its complications.

Methicillin-resistant Staphylococcus aureus as a pathogen in deep neck abscesses: a pediatric case series.

OBJECTIVES: Pediatric deep neck space abscesses are frequently treated by the otolaryngologist. We report four children with deep neck abscesses caused by methicillin-resistant Staphylococcus aureus (MRSA), including the first described case of descending mediastinitis caused by MRSA in a child. METHODS: Records from March 2001 to April 2002 were reviewed. RESULTS: Four patients presented with neck swelling, and three of these also had leukocytosis. All abscess cultures were positive for MRSA. Abscess drainage with antibiotic therapy successfully treated three cases without complication. The fourth case developed descending mediastinitis but survived after additional surgical treatment and prolonged antibiotic therapy. CONCLUSIONS: MRSA should be considered as a potential pathogen in deep neck space abscesses. A high index of suspicion is needed as well as aggressive treatment including incision and drainage along with culture-directed medical therapy. Surgical drainage may be the most important aspect of therapy.

Airway obstruction secondary to tracheobronchial involvement of asymptomatic undiagnosed Crohn's disease in a pediatric patient.

Tracheal involvement in Crohn's disease is extremely rare and can present with airway obstruction in any age group, including the pediatric patient. We describe the case of a 9-year-old female who presented with a 2-week history of intermittent stridor, resolving temporarily with the administration of systemic steroids. The patient denied any abdominal complaints and was otherwise healthy. Bronchoscopy revealed progressive tracheal inflammation necessitating an urgent tracheotomy. Subsequent laboratory investigation confirmed the diagnosis of Crohn's disease. This is the first reported case of previously undiagnosed Crohn's disease presenting with airway manifestations.

Hearing loss as a late complication of radiotherapy in children with brain tumors.

Late postirradiation hearing loss has been well described in the adult population. Few reports exist on the pediatric population. We conducted a retrospective review of 157 consecutive children with brain tumors treated exclusively with irradiation at St Jude Children's Research Hospital. Twenty-six patients developed a hearing loss, 74 did not, and 57 were excluded because of incomplete records. We report a statistically significant 27.41% cumulative risk of a stringent 20-dB hearing loss in the voice frequency range by the fifth year after radiotherapy. The right side demonstrated a significant frequency effect, with a higher incidence of loss in the higher-frequency region. We found no difference in cumulative incidence of hearing shift between the low-, middle-, and high-frequency ranges for either ear. This risk should be anticipated and managed as part of the treatment plan for radiotherapy for the treatment of malignancies. Radiation-induced hearing loss is important to acknowledge so that techniques of hyperfractionation, total dose, ports, preservative infusion medical therapy, or prolonged medical intervention (such as anticoagulants) can be developed that might reduce this disabling problem of postirradiation sensorineural hearing loss in future patients.

Ototoxic protection of sodium thiosulfate: daily vs constant infusion.

OBJECTIVE: Compare the effect of a daily dose of sodium thiosulfate (STS) to continuous infusion of STS on Hartley albino guinea pig (HAGP) on cisplatin (CP)-induced sensorineural hearing loss. Study design Prospective randomized pilot study comparing a single daily dose of STS to a constant controlled infusion of STS given the middle ear space (MES) with an ototoxic regimen of CP. The hearing thresholds will be obtained by auditory brainstem response (ABR) 1 week before and after treatment. Treatment effects on responses at 2, 8, and 16 kHz signals will be investigated by an analysis of variance with one between subjects' condition and two within subjects' conditions. SETTING: Basic science laboratory. RESULTS: STS protection resulted in significantly less hearing loss post-treatment for the protected ears. Guinea pigs with continuous infusion of STS demonstrated less loss in the protected ear than those who received the single daily dose. As expected, hearing loss at 2 kHz was less than that at 8 and 16 kHz. CONCLUSION: A continuous infusion of STS directly to the MES is better than a single daily dose of STS to the MES in reducing the ototoxicity of CP. SIGNIFICANCE: Any targeted protection, whether by continuous infusion or daily dose, is better than no protection against the sensorineural hearing loss of CP.

Childhood primary parotid non-Hodgkin's lymphoma with direct intracranial extension: a case report.

Childhood primary parotid non-Hodgkin's lymphoma (NHL) is a rare but well-recognized entity in the literature. Perineural extension of masses between the head and neck and cranium, although rare, has also been well documented. We report the first documented case, to our knowledge, of a left-sided primary parotid NHL in a child with direct intracranial extension through the foramen rotundum. The mass arose in a 1 1/2-month period. Following evaluation by computed tomography and magnetic resonance imaging, diagnostic procedures (first, fine-needle aspiration and, subsequently, an open biopsy) were undertaken. We discuss the case report and briefly review childhood NHL and perineural metastasis.

Airway management of the severely retrognathic child: use of the laryngeal mask airway.

Successful airway management of an infant or child with moderate to severe retrognathia first requires recognition of a potential problem. If the child cannot be intubated in a standard fashion, the use of a laryngeal mask airway (LMA) should be considered. We describe two cases wherein a toddler and an infant with severe retrognathia failed multiple attempts at traditional intubation. Both had an anterior larynx and hypoplasia of the mandible. In both cases, a subsequent LMA was successfully placed. The severely retrognathic newborn or child presents to the physician a unique challenge in airway management. Techniques to manage this difficult pediatric airway are different from those used in the adult. Otolaryngologists should be aware of this intubation technique and include it in their armamentarium of airway-management strategies. The LMA is not recommended as the technique of choice for securing a difficult airway, but it is an effective alternative when indicated, and it might be life-saving.