A missing vas.

Frontonasal dysplasia (FND) is a congenital malformation characterized by hypertelorism, broad nasion with a midline cleft in the bony dorsum, midline defect of the frontal bone, absence of the nasal tip, and deformities in the nasal alar region. The clinician should be aware of the mild forms of FND. We presented absence of crista galli in a mild case of FND. Computed tomography scanning should assess the facial bones, nose, and paranasal structures. If a surgical correction is planned, this complete work-up prevents unexpected complications and complements the evaluation of paranasal deformities.

Abnormalities, Multiple↗

PubMed2002

Robin sequence: review of treatment modalities for airway obstruction in 110 cases.

OBJECTIVE: The objective of this paper is to assess the treatment protocols used in our institution for the management of airway obstruction among patients with Robin sequence and to suggest a rationale for management based on the findings. METHOD: A retrospective study of 110 children with Robin sequence seen from 1988 to 1997 at the Chang Gung Memorial Hospital was done. Results of the management in airway obstruction and feeding difficulty were studied. RESULTS: Prone posturing was effective in the treatment of mild airway obstruction in 82 patients who had noisy breathing sounds. Twenty-eight infants required endotracheal intubation due to severe respiratory distress. Seven received a tongue to lip adhesion (TLA) operation. Three of these patients showed a relief of airway obstruction, while four of them needed a tracheotomy to maintain a patent airway because of wound dehiscence. Two other patients underwent tracheotomy without TLA. All the six patients who received tracheotomy were eventually decannulated. A nasopharyngeal tube was inserted in two other patients, and one of them improved only temporarily. With regards to feeding difficulties, 46 patients needed nasogastric tube feeding, while the rest were aided through the use of a cleft palate bottle and nipple. A gastrostomy was not needed in any of the patients in this series. CONCLUSION: The morbidity and mortality among Robin sequence patients had been widely decreased through the teamwork of the pediatrician, anesthesiologist, otolaryngologist, dentist, and plastic surgeon. Based on our experience, conservative management was sufficient for those patients with mild airway obstruction, while endotracheal intubation was required for patients with cyanosis, respiratory failure and sleep apnea. Tracheotomy was a better option than TLA for airway management among patients with failed extubation.

Abnormalities, Multiple↗