ABSTRACT
A case of invasive, keratinizing squamous cell carcinoma of the larynx in an 8-year-old female treated with laryngectomy is presented. Perinatal exposure to human papilloma virus and constitutional heterozygosity for a FANCC mutation were identified, though FANCC heterozygosity is not known to be cancer predisposing. An additional tumor-associated mutation in NOTCH1 was also identified potentially contributing to oncogenesis. This case illustrates an exceedingly rare type of cancer in the pediatric population and discusses diagnostic workup, evaluation of risk factors for head and neck cancer, and treatment options.
Subject(s)
Carcinoma, Squamous Cell/genetics , Fanconi Anemia Complementation Group C Protein/genetics , Head and Neck Neoplasms/genetics , Laryngeal Neoplasms/genetics , Carcinoma, Squamous Cell/virology , Child , Fanconi Anemia/genetics , Female , Head and Neck Neoplasms/virology , Heterozygote , High-Throughput Nucleotide Sequencing , Human papillomavirus 16 , Humans , Infectious Disease Transmission, Vertical , Laryngeal Neoplasms/virology , Papillomavirus Infections/complications , Papillomavirus Infections/transmission , Polymerase Chain Reaction , Receptor, Notch1/genetics , Squamous Cell Carcinoma of Head and NeckABSTRACT
OBJECTIVE: Lingual tonsillar hypertrophy is recognized as a cause of persistent obstructive sleep apnea (OSA) after adenotonsillectomy in children. However, little has been reported regarding the complications, postoperative course and effectiveness of lingual tonsillectomy (LT). Our objective was to review the safety and effectiveness of LT in children. METHODS: Retrospective review of children undergoing LT from January 2009 to December 2015 at a tertiary children's hospital. Complications, postoperative course and polysomnographic (PSG) outcomes were recorded for all patients. RESULTS: We identified 92 children (mean age = 8.6 years, 50% female) who underwent LT; 43.5% had a syndromic diagnosis. The most common complications were emergency department presentation for bleeding (4.4%) and poor oral intake (3.3%). The readmission rate was 4.4% including 2 children (2.2%) who required operative control of hemorrhage. No children required unplanned reintubation or ICU admission. In children with PSG data (n = 18), the median apnea-hypopnea index (AHI) decreased from 8.5 to 3.8 events/hour (p = 0.022) and the median obstructive AHI (oAHI) decreased from 8.3 to 3.1 events/hour (p = 0.021). In addition, the oxygen saturation nadir increased from 83.8% to 89.0% (p = 0.0007). After surgery the percentage of patients with oAHI<5 events/hour increased from 27.8% to 61.1% (p = 0.08). CONCLUSIONS: Readmission and bleeding rates after lingual tonsillectomy in children were similar to that seen with tonsillectomy. Polysomnographic data showed that lingual tonsillectomy resulted in a significant reduction of both AHI and oAHI with a postoperative oAHI <5 achieved in 61% of patients.
