Revision of pharyngeal flaps causing obstructive airway symptoms: an analysis of treatment with three different techniques over 39 years.

BACKGROUND: Pharyngeal flaps are used to treat velopharyngeal insufficiency. Occasionally the flap exceeds its intended function and results in an obstructive airway. In this article, the results of management of these cases are analysed. METHODS: This is a 39-year retrospective review of patients who had revision of pharyngeal flaps causing obstructive airway symptoms. Three methods of relieving the airway obstruction were used, and the patients were thus separated into three groups, namely group 1 (release and Z-plasty of the lateral ports), group 2 (division of the pharyngeal flap) and group 3 (division of the pharyngeal flap combined with Furlow palatoplasty). The results obtained were based on four parameters: symptomatic improvement, velopharyngeal sufficiency, the presence of re-attachment of the pharyngeal flap and the necessity for re-operation. These results were then pooled into two groups: 'good result' and 'bad result'. The respiratory disturbance index (RDI) was also obtained and analysed separately. Statistical analysis was performed with the Fisher's exact test and the paired t-test in SPSS v.11. RESULTS: A total of 44 patients were included in the study. There were 20, 11 and 13 patients in groups 1, 2 and 3, respectively. The Pearson's chi-square test indicated that group 3 patients had a statistically significant proportion of 'good results' when compared to groups 1 (p=0.019) and 2 (p=0.004). There was a statistically significant reduction in RDI in group 3 (p=0.003). There was no statistically significant difference between the groups 1 and 2. CONCLUSIONS: Division of a pharyngeal flap for obstructive airway complications should be accompanied by a Furlow palatoplasty to reduce the myriad complications that arise from either a release+Z-plasty or a simple division of the pharyngeal flap.

Hemipalatal hypoplasia.

Asymmetric velopharyngeal incompetence (VPI) has a diverse etiology, but those without any underlying cleft, hemifacial microsomia (HFM), or facial asymmetry are rarely encountered. Such cases have been reported within the last few years, with unilateral velopharyngeal hypoplasia identified to be the underlying abnormality in these patients. However, there have been no reports to date on asymmetric VPI from idiopathic hemipalatal hypoplasia. A retrospective review of patients whose conditions were diagnosed with asymmetric VPI owing to hemipalatal hypoplasia and who do not have underlying cleft, HFM, or any significant facial asymmetry was performed. During a 10-year period, 5 patients with asymmetric VPI from idiopathic hemipalatal hypoplasia were treated at our center. Four of 5 of these patients presented with nasal regurgitation. Two were found to have tonsillar enlargement on the side of the hemipalatal hypoplasia, and another had an ipsilateral tongue mass that subsequently required wedge excision. Two were managed conservatively with speech therapy, whereas the other 3 developed speech problems that required surgery. The first patient underwent a skewed pharyngeal flap, but her speech problem improved only after a second surgery, which involved a centralized pharyngeal flap. The speech problems of the last 2 patients were corrected with a centralized pharyngeal flap. Hence, we conclude that patients with asymmetric VPI from idiopathic hemipalatal hypoplasia, compared with those of other etiology, particularly unilateral velopharyngeal hypoplasia, seem to present in a different manner. When surgical intervention is indicated for correction of the speech problem that eventually develops, centralized rather than skewed pharyngeal flap seems to be more reliable.

Comparison of outcomes of velopharyngeal surgery between the inferiorly and superiorly based pharyngeal flaps.

BACKGROUND: Pharyngeal flaps have been widely used for the correction of velopharyngeal incompetence. The aim of this study was to compare the outcomes of velopharyngeal surgery between those who received the superiorly and inferiorly based pharyngeal flaps. METHODS: A retrospective review of medical records of patients with cleft palates who received pharyngeal flap surgery for the correction of velopharyngeal incompetence at one craniofacial center was performed. The superiorly based flaps were elevated and inset using the fish-mouth method. The inferiorly based flaps were sutured to the soft palate where a distally based mucosa flap was turned over to cover the raw surface of the flap pedicle. The velopharyngeal functions were categorized as adequate, marginal, or inadequate. Complications associated with the operation were documented. Statistical comparisons between the two groups were made. RESULTS: There were 65 patients in each group. No statistically significant differences were found for sex distribution and age at operation. The outcomes of the velopharyngeal surgery were better in the group of patients who received the inferiorly based pharyngeal flaps (p = 0.030). The complications were not significantly different between the two groups, and were all relatively mild. CONCLUSION: The inferiorly based pharyngeal flap was more effective than the superiorly based pharyngeal flap for the correction of velopharyngeal incompetence. A probable explanation may be the fibrotic changes and scar contracture occurring in the pedicle of the superiorly based pharyngeal flap that may have impaired the velopharyngeal closure.

Optic nerve compression in craniofacial fibrous dysplasia: the role and indications for decompression.

BACKGROUND: Fibrous dysplasia is a benign bone disorder in which craniofacial bones are involved in approximately one-fifth of cases. Optic nerve compression is one of its most potentially devastating complications. The approach to visual disturbance caused by disease involvement of the optic canal has been varied and controversial. This is especially the case with regard to surgical decompression, particularly prophylactic decompression. METHODS: The authors performed a retrospective review of fibrous dysplasia patients who showed clinical or radiographic evidence of optic canal involvement over a 27-year period. RESULTS: Twenty-two optic canals in 18 patients were affected by disease. Of these, 14 optic canals had symptoms (i.e., affected visual acuity or visual field). Twelve therapeutic and six prophylactic optic nerve decompressions were performed in total. At long-term follow-up (of at least 1 year), there was deterioration of vision in one-third of the patients who had undergone prophylactic decompression. Therapeutic decompression seemed to prevent visual deterioration in slightly more than half of the patients, with the majority having improvement in vision. There was visual deterioration in the remaining patients within this group, with most ultimately ending up with blindness. CONCLUSIONS: Therapeutic optic nerve decompression is advocated in patients with continuous deterioration of vision. Prophylactic decompression, in contrast, is not advised to be performed as a primary surgical procedure but as a procedure secondary to excision of lesion in the anterior skull base during the same operation.

Van der Woude syndrome: clinical presentation in 64 patients.

OBJECTIVE: Van der Woude syndrome, characterized by lip pits and cleft lip/ palate, presents with variable expressions. This retrospective study was designed to obtain a better understanding of its clinical pattern in Taiwanese patients. MATERIALS AND METHODS: Of 13,147 cleft patients treated at the Chang Gung Craniofacial Center from 1976 to 2004, there were 64 with Van der Woude syndrome. Clinical expressions and family histories were collected and analyzed. RESULTS: The male to female ratio was 1:1. The majority had complete cleft types. Severe cleft was found among the patients, with 52% having bilateral cleft lip and palate, 31% having unilateral cleft lip and palate, and 17% having isolated cleft palate. A positive family history was found in 53.1% of the patients. The size, shape, location, and depth of the pits varied among patients. CONCLUSION: Clinical presentations did not always parallel those reported in the literatures. These differences warrant further investigation.

Craniofacial fibrous dysplasia: an update.

Fibrous dysplasia was first described by Lichtenstein in 1938 as a disorder characterized by progressive replacement of normal bone elements by fibrous tissue. It is a bone tumor that, although benign, has the potential to cause significant cosmetic and functional disturbance, particularly in the craniofacial skeleton. Its management poses significant challenges to the surgeon. Its compression of the optic nerve with resulting visual impairment is especially alarming. Over the years, we have gained a better understanding of its etiology, clinical behavior, and both surgical and non-surgical treatments. Its characteristics, under various imaging modalities, have been thoroughly described in recent years. These developments have taken place with the goal of optimizing treatment of those who suffer from this disease. However, the role of prophylactic optic nerve decompression in cases of optic canal involvement has recently been challenged: the results of a few recent studies have raised questions regarding its role. Further studies would be required to assess its value.