A Case of Robin Sequence Mandibular Distraction Performed on a 6-year-old Patient Resulting in an Ability to Make Vocalizations
2016 Volume 41 Issue 1 Pages 44-50
Details
2016 Volume 41 Issue 1 Pages 44-50
Background: Robin sequence is an etiologically nonspecific complex that could occur with various syndromes of known or unknown origin or in isolation. The disorder is derived from an underdevelopment of the mandible, which causes problems such as secondary glossoptosis and airway obstruction. The result brought by dyspnea is a dangerous problem. We experienced a case of mandibular distraction in a 6-year-old with serious airway obstruction.
Case Report: The patient was the second of female twins delivered at 34 weeks. Her respiration was managed by tracheal intubation from birth. Tracheostomy was performed to correct her airway obstruction at 2 months of age. No further surgical treatments were performed on her micrognathia for the next 6 years, during which time no significant growth or improvement was observed in the affected area. When the 6-year-old patient was brought in for consultation, her Robin sequence was confirmed. She was suffering from Goldenhar syndrome. Head and neck CT showed the following: (1) hypoplasia and deficit of the mandible, (2) severe glossoptosis, and (3) airway constriction resulting from marked hyperplasia of granulation tissue surrounding the trachea incision site. 3D models were created for preoperative consideration before performing bilateral mandibular body osteotomy distraction.
Results: Distraction was started at 1mm/day from postoperative day 4. On the final distraction (postoperative day 18), the patient's ability to make a sound was checked, when the tracheotomy hole was closed. On postoperative day 23, the patient's cannula was switched to a speech cannula for the first time and language training started.
Conclusion: Although the next goal is extubation, much time will be required to train the vocal cords and respiratory muscles. It will not be an easy process because the patient's organs and muscles have not been used for six years; it will take a number of years to reach extubation, and we believe it must be done carefully. The patient may need additional distractions in the future.
