Mandibular Distraction in a Patient With Type II Collagenopathy.

Kniest dysplasia is an extremely rare form of type II collagenopathy associated with cleft palate, micrognathia, shortened trunk, arms and legs, and club foot. The authors present a case of an infant with this disorder who also had micrognathia and respiratory distress for which mandibular distraction was performed. Although abnormal collagen and impaired endochondral ossification is noted with Kniest dysplasia, adequate bone formation was observed across the distraction gap. Nonetheless, despite stable mandibular advancement, failure to consider concomitant restrictive lung disease resulted in tracheostomy dependence. The authors demonstrate that while successful bone regeneration can be achieved through distraction of intramembranous facial bones, discretion must still be employed in patients with collagenopathies.

Tongue suture placement after cleft palate repair.

OBJECTIVE: Postoperative airway obstruction is a complication of cleft palate repair. A technique to control the airway is to place a suture through the tongue at the conclusion of the palate repair, but it is not uniformly adopted by surgeons. Although it has been frequently performed, the use and effectiveness of the tongue suture have not been studied. Our purpose was to determine the usefulness of tongue suture placement. DESIGN: We surveyed health care providers as to their frequency of use and the value of the tongue suture in postoperative airway management of the cleft palate patient. The survey was sent via e-mail to 2080 members of the American Cleft Palate-Craniofacial Association, with a total of 396 responders. RESULTS: Surgeons were nearly equally split on placing a tongue suture, with 41.1% responders reporting that they use a tongue suture all of the time and 41.1% of responders reporting that they never used a tongue suture. Some criterion used for placement was the complexity of the case, syndromic patients, and overseas cleft missions. CONCLUSIONS: Many cleft palate repairs are done annually without using tongue sutures, but it does not seem to affect the outcomes among surgeons, thus confounding the question of effectiveness. At this time, tongue suture placement after cleft palate repair is variable and subjective. Further studies need to be performed to assess outcomes after placing a tongue suture.

Craniofacial distractor applicator.

Craniofacial distraction can be planned using cephalograms, computed tomography, medical models, and other forms of anatomic data. However, it is often difficult to translate this plan to the patient. Specifically, it is difficult to obtain true parallel placement of bilateral midface and mandibular distractors. Intraoperative translation of preoperatively determined vectors is also troublesome. One method of application uses computed tomography data with radiofrequency triangulation technology in a specially equipped room. This helps with the issue of placement on the patient but does not establish parallelism. We have developed a simple-to-use craniofacial application stabilization device that allows equal placement of bilateral distractors and measurement of distraction vectors. The applicator measures 20 cm in length in its open configuration. The terminal portion of the device has a coupler that holds the distractor during placement. The device is hinged in 3 points so that it can be easily folded into a compact and autoclavable device (7 x 3 cm). The hinges allow equal placement of bilateral distractors. Each hinge can be calibrated to determine the vector of distraction and confirm equal application. Lastly, the stabilizer can be fixed to nasion with a Steinmann pin for reference, allowing intraoperative translation of distraction vectors. We demonstrated on skull models that the craniofacial distractor applicator can accurately allow parallel intraoperative placement of craniofacial distractors. We demonstrated simultaneous placement of the distractors allowing a more precise determination of end points.