Cholesteatoma in Children with Sotos Syndrome.

BACKGROUND: Sotos syndrome is a rare genetic disorder characterized by neurodevelopmental delay and excessive childhood growth including macrocephaly. In this study, we present our experience of children with Sotos syndrome and cholesteatoma. METHODS: Retrospective case note review and cross-referencing with hospital picture archive and communication systems or cases identified from a prospective database of consecutive cholesteatoma surgeries. RESULTS: A total of 400 children underwent surgery for acquired cholesteatoma and 5 (1%) had Sotos syndrome (1 bilaterally). In comparison, 42(11%) had cleft palate which is around 10 times more common than Sotos syndrome, 5 (1%) had Down syndrome, and 3 (1%) had Turner syndrome. The median age at primary surgery was 8 years old (3.5-10.9 years), 124 children with Sotos syndrome were identified in picture archive and communication systems (4% with cholesteatoma) of which temporal bone imaging was available in 86 (70%) at the median age of 9 years (0-17.2), and 33/86 (38%) had normal ears bilaterally on all imaging. Changes consistent with fluid or inflammation were present in 9/30 (30%) computed tomography and 24/72 (33%) magnetic resonance imaging scans. Development of mastoid pneumatization was impaired in 20/30 (67%) computed tomography and 8/72 (11%) magnetic resonance imaging scans. At 5 years, children with Sotos syndrome (33%) had greater recidivism than those with cleft palate (15%) (Kaplan-Meier log-rank analysis, P=.001) CONCLUSION: Children with Sotos syndrome appear to be at increased risk of developing acquired cholesteatoma. Impaired temporal bone pneumatization is a common incidental finding in Sotos syndrome in keeping with this risk. Further study of this previously unreported association may improve the understanding of pathogenetic mechanisms in cholesteatoma.

Esterified Hyaluronic Acid Placed in the Middle Ear Does Not Improve Outcomes in Cholesteatoma Surgery.

BACKGROUND: The aim of this article is to assess the efficacy of esterified hyaluronic acid as a barrier to formation of adhesions and improvement of tympanomastoid ventilation. METHODS: A prospective cohort analysis was performed at a tertiary referral centre. 126 ears were analysed in children with cholesteatoma. Esterified hyaluronic acid was placed on the promontory of 63 ears at primary canal wall intact surgery for cholesteatoma. No esterified hyaluronic acid was used in 63 control ears. Cholesteatoma recurrence, histopathological analysis of scar tissue following second-stage procedure, and middle ear pressure were the main outcome measures. RESULTS: At 5 years, esterified hyaluronic acid (7%) and non-esterified hyaluronic acid (10%) did not differ in cholesteatoma recurrence (Kaplan- Meier log rank analysis, P=.52). Esterified hyaluronic acid (n=11) and non-esterified hyaluronic acid (n=2) ears formed scar at the site of packing material (n=11) (Fisher's exact test, P=.04). Foamy histiocytes/macrophages were found in esterified hyaluronic acid (n=15) and non-esterified hyaluronic acid ears (n=1) (Fisher's exact test, P-125 daPa) in 44% (14/32) esterified hyaluronic acid ears and 42% (15/36) non-esterified hyaluronic acid ears (P=1.0, Fisher's exact test). CONCLUSIONS: We have discontinued the use of esterified hyaluronic acid in cholesteatoma surgery due to lack of detectable benefit. Esterified hyaluronic acid in the middle ear neither reduces cholesteatoma recurrence nor appears to improve the ventilation of the middle ear. Furthermore, esterified hyaluronic acid alters the inflammatory process within the middle ear, the significance of which remains unclear.