ABSTRACT. Objective. Surgery for craniosynostosis has evolved rapidly over the past two decades, with increased emphasis on early, extensive operations. Older published series may not accurately reflect more recent experience. Our study was designed to analyze outcome in a large series of consecutive patients treated recently at a single center.Methods. We reviewed 250 consecutive patients who underwent surgical treatment of craniosynostosis between January 1, 1987 and December 31, 1992. They were divided into nine groups by suture involvement: sagittal, unilateral coronal, bilateral coronal, unilateral lambdoid, bilateral lambdoid, metopic, multiple suture, the Kleeblattschädel deformity (cloverleaf skull), and acquired craniosynostosis. Outcome was analyzed in terms of residual deformities and irregularities, complications, mortality, as well as the need for additional surgery.Results. There were 157 males (62.8%) and 93 females (37.2%), with most of the male preponderance accounted for by the large sagittal synostosis group, which consisted of 82 males and 25 females. Median age at first operation was 147 days. A named syndrome was present in 23 patients (9.2%) and was more common than expected with bilateral and unilateral coronal synostosis, the Kleeblattschädel deformity, and multiple suture synostosis. There were two deaths (0.8%), both with Kleeblattschädel patients, and 17 other complications (6.8%). Morbidity and mortality were significantly associated with secondary vs primary operations and syndromic vs nonsyndromic patients. Outcome analysis revealed the best surgical results with metopic synostosis and significantly less good results with the Kleeblattschädel deformity, multiple suture synostosis, and bilateral coronal synostosis.Conclusions. Using modern surgical techniques, craniosynostosis can be corrected with good outcomes and relatively low morbidity and mortality, particularly for otherwise healthy, nonsyndromic infants. Pediatrics 1997;100(1). URL: http://www.pediatrics.org/cgi/content/ full/100/1/e2; craniosynostosis, craniofacial anomalies, craniofacial surgery, facial deformities.

Surgical management of velopharyngeal insufficiency by attachment of posterior pharyngeal flap or construction of sphincter pharyngoplasty is reviewed. Posterior pharyngeal flap surgery is well established, with a long history dating back to the 19th century. Flaps have been based superiorly, inferiorly, or laterally. There have been reports of airway obstruction and obstructive sleep apnea associated with posterior pharyngeal flap surgery. The concept of surgical creation of a dynamic sphincter pharyngoplasty to provide velopharyngeal closure was first introduced by Hynes in 1950. Hynes and others have proposed several subsequent anatomic modifications. Airway dysfunction has also been reported following sphincter pharyngoplasty, but may not be as frequent or severe as with posterior pharyngeal flap. While several studies have compared posterior pharyngeal flap and sphincter pharyngoplasty in terms of speech outcome or complications, there is not, as yet, a consensus regarding the specific choice of one versus the other for surgical management of velopharyngeal insufficiency.

Despite strongly held views in the literature concerning the relative effectiveness and safety of flap and sphincter pharyngoplasty, no significant differences were detected 1 year postoperatively.

Objective and Methods Between February 1987 and September 1997, 247 patients underwent primary repair of a cleft of the secondary palate by one surgeon, using the double-opposing Z-plasty (Furlow) technique. This retrospective study reviews perioperative and postoperative airway compromise among these patients. The purposes of this study were to identify factors associated with airway obstruction following palatoplasty and to analyze the management of those patients. Although infants experiencing airway problems following Wardill-Kilner and Von Langenbeck palatoplasty have been described, airway complications in a group of Furlow repair patients has not been previously reported. Results Fourteen patients (5.7%) had airway problems. The average age of these patients was 18 months, which was not significantly different from those without airway problems. Airway obstruction occurred as late as 48 hours after the completion of surgery. Twelve of the 14 patients had severe airway compromise requiring continued postoperative intubation, reintubation, or tracheostomy (one). There were no deaths. Thirteen of the 14 patients with postoperative airway problems (93%) had other congenital anomalies in addition to clefting, a named congenital disorder, or both. Seven of those 13 had Pierre Robin sequence. In contrast, only 40 of the 233 patients without airway problems (17%) had additional congenital anomalies or named disorders. Presence of other congenital anomalies was associated with a significantly increased risk of airway obstruction (p = .005). Conclusion Patients with cleft palate with the Pierre Robin sequence or other additional congenital anomalies had an increased risk of airway problems following palatoplasty. Awareness of this risk permits identifying those patients prior to surgery so that they can be monitored and managed appropriately, minimizing the likelihood of major complications or death.

No abstract