[Skip to Content]
[Skip to Content Landing]
Download PDF
Figure 1.
Assessment of velopharyngeal closure
in a patient using both multiview videofluoroscopy in lateral (A), frontal
(B), and base (C) projections and flexible fiberoptic nasopharyngoscopy (D).
In A and D, a indicates adenoid; p, posterior pharyngeal wall; and v, velum.
The arrows in B mark the lateral pharyngeal walls (LPW). In C, the posterior
pharyngeal wall (PPW) is marked by the white arrow.

Assessment of velopharyngeal closure in a patient using both multiview videofluoroscopy in lateral (A), frontal (B), and base (C) projections and flexible fiberoptic nasopharyngoscopy (D). In A and D, a indicates adenoid; p, posterior pharyngeal wall; and v, velum. The arrows in B mark the lateral pharyngeal walls (LPW). In C, the posterior pharyngeal wall (PPW) is marked by the white arrow.

Figure 2.
Hypertrophic tonsils (t) as seen
in the oropharynx during nasopharyngoscopy in 2 patients (A and B) with velocardiofacial
syndrome. The tonsils are interposed between the velum (v) and posterior pharyngeal
wall (p).

Hypertrophic tonsils (t) as seen in the oropharynx during nasopharyngoscopy in 2 patients (A and B) with velocardiofacial syndrome. The tonsils are interposed between the velum (v) and posterior pharyngeal wall (p).

Figure 3.
Hypertrophic tonsils (t) seen
in lateral view videofluoroscopy behind the velum (v) and positioned posteriorly
in the oropharynx.

Hypertrophic tonsils (t) seen in lateral view videofluoroscopy behind the velum (v) and positioned posteriorly in the oropharynx.

Figure 4.
Wide pharyngeal flap as seen endoscopically
1 year following surgery. The lateral ports are patent, but small.

Wide pharyngeal flap as seen endoscopically 1 year following surgery. The lateral ports are patent, but small.

Figure 5.
Medially deviated internal carotid
arteries in a coronal magnetic resonance angiography section (A) that are
located close to the mucosal surface as seen in an axial view (B). These images
are from case 15 in Table 3.

Medially deviated internal carotid arteries in a coronal magnetic resonance angiography section (A) that are located close to the mucosal surface as seen in an axial view (B). These images are from case 15 in Table 3.

Table 1. 
Twenty Subjects With Velocardiofacial Syndrome According to
Age, Cleft Type, History of Previous Surgery for Velopharyngeal Insufficiency,
and the Presence of Congenital Heart Anomalies*
Twenty Subjects With Velocardiofacial Syndrome According to Age, Cleft Type, History of Previous Surgery for Velopharyngeal Insufficiency, and the Presence of Congenital Heart Anomalies*
Table 2. 
Perceptual Rating of Nasality Related to Rating of Velar Motion,
Lateral Pharyngeal Wall Motion, and Posterior Pharyngeal Wall Motion Prior
to Pharyngeal Flap Surgery*
Perceptual Rating of Nasality Related to Rating of Velar Motion, Lateral Pharyngeal Wall Motion, and Posterior Pharyngeal Wall Motion Prior to Pharyngeal Flap Surgery*
Table 3. 
Carotid Pulsations as Seen on Endoscopy in Relation to Magnetic
Resonance Angiography or Computed Tomography Evaluation and Findings at Surgery*
Carotid Pulsations as Seen on Endoscopy in Relation to Magnetic Resonance Angiography or Computed Tomography Evaluation and Findings at Surgery*
1.
Shprintzen  RJSiegel-Sadewitz  VLAmato  JGoldberg  RB Retrospective diagnoses of previously missed syndromic disorders among 1,000 patients with cleft lip, cleft palate, or both. Birth Defects Orig Artic Ser. 1985;2185- 92
2.
Lipson  AHYuille  DAngel  MThompson  PGVandervoord  JGBeckenham  EJ Velocardiofacial (Shprintzen) syndrome: an important syndrome for the dysmorphologist to recognize. J Med Genet. 1991;28596- 604Article
3.
MacKenzie-Stepner  KWitzel  MAStringer  DALindsay  WKMunro  IRHughes  H Abnormal carotid arteries in the velocardiofacial syndrome: a report of three cases. Plast Reconstr Surg. 1987;80347- 351Article
4.
D'Antonio  LDMarsh  JL Abnormal carotid arteries in the velocardiofacial syndrome. Plast Reconstr Surg. 1987;80471- 472Article
5.
Mitnick  RJBello  JAGolding-Kushner  KJArgamaso  RVShprintzen  RJ The use of magnetic resonance angiography prior to pharyngeal flap surgery in patients with velo-cardio-facial syndrome. Plast Reconstr Surg. 1996;97908- 919Article
6.
Witt  PDMiller  DCMarsh  JLMuntz  HRGrames  LM Limited value of preoperative cervical vascular imaging in patients with velocardiofacial syndrome. Plast Reconstr Surg. 1998;1011184- 1195Article
7.
Shprintzen  RJ Discussion: limited value of preoperative cervical vascular imaging in patients with velocardiofacial syndrome. Plast Reconstr Surg. 1998;1011196- 1199Article
8.
Golding-Kushner  KJArgamaso  RVCotton  RT  et al.  Standardization for the reporting of nasopharyngoscopy and multiview videofluoroscopy: a report from an International Working Group. Cleft Palate J. 1990;27337- 347Article
9.
Shprintzen  RJ Pharyngeal flap surgery and the pediatric upper airway. Int Anesthesiol Clin. 1988;2679- 88Article
10.
Shprintzen  RJSinger  LSidoti  EJArgamaso  RV Pharyngeal flap surgery: postoperative complications. Int Anesthesiol Clin. 1992;30115- 124Article
11.
Arvystas  MShprintzen  RJ Craniofacial morphology in the velocardiofacial syndrome. J Craniofac Gen Dev Biol. 1984;439- 45
12.
Golding-Kushner  KJCraniofacial Morphology and Velopharyngeal Physiology in Four Syndromes of Clefting [dissertation] New York Graduate School and University Center, City University of New York1991;
13.
Shprintzen  RJSher  AECroft  CB Hypernasal speech caused by hypertrophic tonsils. Int J Pediatr Otorhinolaryngol. 1987;1445- 56Article
14.
MacKenzie-Stepner  KWitzel  MAStringer  DALaskin  R Velopharyngeal insufficiency due to hypertrophic tonsils: a report of two cases. Int J Pediatr Otorhinolaryngol. 1987;1457- 63Article
15.
Argamaso  RV The pharyngeal flap in cleft lip and palate. Kernihan  DARosenstein  SWeds.Cleft Lip and Palate: A System of Management Baltimore, Md Williams & Wilkins1990;263- 269
16.
Senders  CWEmery  BESykes  JMBrodie  HA A prospective, double-blind, randomized study of the effects of perioperative steroids on palatoplasty patients. Arch Otolaryngol Head Neck Surg. 1996;122267- 270Article
17.
Senders  CSEisele  JH Lingual pressure induced by mouthgags. Int J Pediatr Otorhinolaryngol. 1995;3353- 60Article
18.
Shprintzen  RJ Conceptual framework for pharyngeal flap surgery. Bardach  JMorris  Heds.Management of Unilateral Cleft Lip and Cleft Palate Philadelphia, Pa WB Saunders1990;806- 809
19.
Golding-Kushner  KJ Treatment of articulation and resonance disorders associated with cleft palate and VPI. Shprintzen  RJBardach  JCleft Palate Speech Management: A Multidisciplinary Approach St Louis, Mo Mosby–Year Book Inc1995;327- 351
Citations 0
Original Article
April 2002

Pharyngeal Flap and the Internal Carotid in Velocardiofacial Syndrome

Author Affiliations

From the Division of Facial Plastic and Reconstructive Surgery, Departments of Otolaryngology and Pediatrics (Dr Tatum); the Division of Neuroradiology, Department of Radiology (Dr Chang); the Communication Disorder Unit (Ms Havkin); and the Center for the Diagnosis, Treatment, and Study of Velo-Cardio-Facial Syndrome, Department of Otolaryngology and Communication Science (Dr Shprintzen), State University of New York Upstate Medical University, Syracuse.

 

From the Division of Facial Plastic and Reconstructive Surgery, Departments of Otolaryngology and Pediatrics (Dr Tatum); the Division of Neuroradiology, Department of Radiology (Dr Chang); the Communication Disorder Unit (Ms Havkin); and the Center for the Diagnosis, Treatment, and Study of Velo-Cardio-Facial Syndrome, Department of Otolaryngology and Communication Science (Dr Shprintzen), State University of New York Upstate Medical University, Syracuse.

Arch Facial Plast Surg. 2002;4(2):73-80. doi:
Abstract

Internal carotid artery anomalies have been documented as a common clinical feature in velocardiofacial syndrome. There has been some controversy over the need for preoperative imaging procedures, such as magnetic resonance angiography, when planning pharyngeal surgery for correcting velopharyngeal insufficiency. The purpose of this article is to describe 20 patients with velocardiofacial syndrome who received comprehensive evaluation and underwent pharyngeal flap surgery within a 2-year period and to report the technique used for dissecting the flap and the surgical outcomes. Anomalies of the major neck vessels were present in all cases, but 5 of these 20 cases had particularly severe anomalies of the internal carotid arteries that placed the vessels directly deep within the donor site for the pharyngeal flap. Surgery was carried out successfully in all 20 cases using a modified approach after radiographic imaging was performed to locate the arteries. In the 5 cases with severe malpositioning of the internal carotid arteries, it was clear that the vessels could have been injured had their location not been identified and the surgical approach modified to avoid them.

Velocardiofacial syndrome (VCFS) is the most common multiple anomaly syndrome associated with cleft palate, constituting 8% of patients with cleft palate,1 including overt, submucous, and occult submucous cleft palate. Although the frequency of VCFS among individuals with cleft lip is not known, cleft lip does occur as a finding in the syndrome at least occasionally.2 It has been reported that approximately 5% of all patients at large interdisciplinary cleft palate–craniofacial centers have VCFS.1-2 Because velopharyngeal insufficiency (VPI) is such a common disorder in the spectrum of anomalies in VCFS, it is likely that many of the patients will present for surgical management of hypernasal speech.

Anomalies of the internal carotid arteries in VCFS were initially reported in 1987.3-4 MacKenzie-Stepner et al3 used standard angiography to demonstrate ectopic and medial placement of the internal carotid arteries in 3 cases selected specifically because of previous observations from nasopharyngoscopy that showed prominent arterial pulsations in the posterior pharyngeal wall during workup for pharyngeal flap surgery. The abnormal placement of the arteries was considered a contraindication to pharyngeal flap surgery in these cases.3 In a more comprehensive study using magnetic resonance angiography (MRA), Mitnick et al5 assessed 19 consecutive patients with VCFS referred for pharyngeal flap surgery. The MRA results were correlated to findings from nasopharyngoscopic examinations for observations of visible pulsations in the pharyngeal walls. It was found that observations of pulsations did not predict medial deviation of the internal carotid arteries, and medially deviated arteries did not always result in visible pulsations. Mitnick et al5 concluded that some type of vascular imaging procedure was necessary before undertaking pharyngeal flap surgery because the placement of the arteries in several of their cases was directly within the donor site of a pharyngeal flap.

Witt et al,6 using a questionnaire and anecdotal reports to determine if there had been any fatalities in patients with VCFS during pharyngeal flap surgery, reported the absence of data to support the notion that internal carotid anomalies warranted preoperative MRA in patients with VCFS. Based on a sample of 30 surgeons (selection criteria were not reported), they indicated that the absence of reported deaths or bleeding complications was sufficient evidence to recommend against the added costs of MRA studies. In a discussion of the Witt et al6 article, Shprintzen7 pointed out design flaws and lack of scientific evidence. Shprintzen7 reinforced the scientific evidence from the original prospective research of Mitnick et al,5 pointing out hard scientific data that supported the necessity of preoperative vascular imaging in patients with VCFS.

The purpose of this article is to describe 20 consecutive patients with VCFS who had pharyngeal flap surgery within a 2-year period. The surgical technique, modifications, outcomes, and the intraoperative status of the internal carotid arteries are also reported.

SUBJECTS AND METHODS
SUBJECTS

The study sample comprised 20 consecutively referred patients with VCFS. All cases were confirmed by FISH (fluorescent in situ hybridization) to have a 22q11 deletion, and all patients were examined by the fourth author (R.J.S.) to confirm the clinical diagnosis. There were 11 male subjects and 9 female subjects, ranging in age from 4 to 17 years (Table 1). This sample represents all cases of VCFS referred for surgical management of VPI from the Center for the Diagnosis, Treatment, and Study of Velo-Cardio-Facial Syndrome of the State University of New York Upstate Medical University, Syracuse, within a 2-year period (1998-1999). These 20 cases represented 25% of all cases of VCFS referred to the VCFS center within this 2-year period. The other cases were not referred for surgery for a variety of reasons, including age (too young), no evidence of VPI (about 10% of the sample), refusal of additional surgery because of previous failures elsewhere, or successful treatment elsewhere prior to referral.

All patients received a comprehensive evaluation that included MRA or computed tomography (CT) scanning, video nasopharyngoscopy, and multiview videofluoroscopy. Five patients had previously undergone other surgical procedures for VPI (Table 1). Four of the patients had previous failed sphincter pharyngoplasties, and 1 had a failed Furlow palate repair as a secondary procedure. In all 5 cases, hypernasality was not corrected by the surgery.

Of the total sample, 1 patient had an overt cleft of the secondary palate, 10 had obvious submucous clefts including bifid uvula, and 8 had occult submucous clefts (Table 1). One patient had no evidence of a cleft, and 2 patients had asymmetric VPI related to pharyngeal hypotonia on the left (Table 2). The frequency of congenital heart anomalies is also listed in Table 1. All patients had grossly normal expressive language at the time of surgery.

ASSESSMENT PROCEDURES

All patients were evaluated by the interdisciplinary team at the Center for the Diagnosis, Treatment, and Study of Velo-Cardio-Facial Syndrome. Evaluation procedures included the following:

  1. speech and language evaluation including a group rating of nasal resonance on a 5-point scale (hyponasal, normal, mild, moderate, and severe hypernasality)

  2. genetic/dysmorphologic evaluation

  3. cytogenetic/molecular genetic evaluation including FISH

  4. flexible fiber optic nasopharyngoscopic evaluation

  5. multiview videofluoroscopic evaluation in at least frontal and lateral views

  6. facial plastic surgery

  7. immunologic evaluation

  8. audiologic evaluation

  9. magnetic resonance angiography of the neck vessels and magnetic resonance imaging of the brain and spine or CT scanning

  10. otolaryngologic evaluation

  11. a variety of evaluations from other disciplines, as needed, including endocrinology, neurology, cardiology, nephrology, and hematology.

  1. speech and language evaluation including a group rating of nasal resonance on a 5-point scale (hyponasal, normal, mild, moderate, and severe hypernasality)

  2. genetic/dysmorphologic evaluation

  3. cytogenetic/molecular genetic evaluation including FISH

  4. flexible fiber optic nasopharyngoscopic evaluation

  5. multiview videofluoroscopic evaluation in at least frontal and lateral views

  6. facial plastic surgery

  7. immunologic evaluation

  8. audiologic evaluation

  9. magnetic resonance angiography of the neck vessels and magnetic resonance imaging of the brain and spine or CT scanning

  10. otolaryngologic evaluation

  11. a variety of evaluations from other disciplines, as needed, including endocrinology, neurology, cardiology, nephrology, and hematology.

Velopharyngeal insufficiency was assessed from clinical speech and language evaluation, multiview videofluoroscopy, and nasopharyngoscopy using the International Working Group rating scale (Figure 1).8 The ratings of the components of velopharyngeal closure are listed in Table 2 along with the degree of perceived hypernasality.

FLUOROSCOPIC AND ENDOSCOPIC ASSESSMENTS

Preoperatively, velar motion varied among the sample but was rated above 0.5 for only 1 case (Table 2).8 A rating of 0.5 indicates velar motion of half of the distance from the rest position to the posterior pharyngeal wall. In 14 cases, velar motion was under 0.5. Poor or absent lateral pharyngeal wall motion (a rating of 0.2 or lower) was found in all cases except 1. In 2 cases, there was asymmetric lateral pharyngeal wall motion, with the right lateral pharyngeal wall showing motion rated at 0.2 and the left lateral wall showing no motion (0.0) in both cases. A rating of 0.2 indicates motion less than half of the distance to the pharyngeal midline. In all other cases, the lateral pharyngeal walls were rated at 0.0 or 0.1 bilaterally. In such cases, very wide subobstructing pharyngeal flaps are recommended.

MAGNETIC RESONANCE ANGIOGRAPHY

Nineteen patients had an MRA prior to pharyngeal flap surgery. One patient required contrast-enhanced CT scanning because of the presence of a pacemaker. In most cases, MRA was performed within a week of surgery, but in a few cases MRA was done several months prior to admission. The MRA protocol includes scanning of the entire head and neck and the upper chest to the aortic arch using 7-mm-thick abutting slices. We also scan the spine because of the frequency of tethered cord and other spinal anomalies in VCFS. The brain is also assessed from the magnetic resonance imaging scans. The MRA is formatted in coronal, transverse, and sagittal views, and 3-dimensional reconstructions of the vessels are done as well. The common carotid, internal carotid, external carotid, and vertebral arteries are all isolated in relation to their position within the pharyngeal soft tissues. One patient had a pacemaker, which necessitated the substitution of contrast-enhanced 3-dimensional CT angiography instead of MRA.

ASSESSMENT OF TONSILS

Previous reports have shown that tonsillectomy prior to pharyngeal flap surgery is an important component in the avoidance of obstructive sleep apnea following surgery.9-10 Tonsils were assessed using both videofluoroscopy and nasopharyngoscopy (Figure 2 and Figure 3). It has been our experience that tonsillar hypertrophy is not always well recognized on oral examination. In cases of VCFS, the pharynx (including both the oropharynx and nasopharynx) is typically deep secondary to platybasia11 and a short, deficient palate.12 When tonsils are assessed perorally using the familiar scale (0 to 4+), the rating is based on the medial projection of the tonsils. When the pharynx is deep, as in VCFS, it may be that the path of least resistance for tonsillar growth is posterior, posteroinferior, or posterosuperior. Previous reports have documented that tonsils can grow behind the palate and faucial pillars.13-14 When this posterior growth occurs, the tonsils can be seen on endoscopic examination (Figure 2) and on fluoroscopic assessment when barium contrast is used (Figure 3). In our sample, when tonsils are seen posteriorly in the pharyngeal airway, they are always removed 6 or more weeks before pharyngeal flap surgery to avoid respiratory complications. If not removed, the enlarged tonsils would occlude both the pharynx as well as the lateral ports beneath the pharyngeal flap, which has been associated with the development of obstructive sleep apnea.10 In addition, the presence of tonsils intruding into the pharynx would reduce the ability to create a flap of adequate width intraoperatively.

SURGICAL TECHNIQUE

The pharyngeal flap is intended to be as short a musculomucosal flap as possible; leaving a small donor site, which results in less throat discomfort and less circumferential narrowing of the pharynx after surgery.10, 15 After induction of general endotracheal anesthesia, standard prepping and draping is performed. Clindamycin, 10 mg/kg (maximum, 900 mg), and dexamethasone, 0.5 mg/kg (maximum, 12 mg), are administered intravenously.16 The Dingman mouth gag is introduced and suspended with towel rolls on the chest rather than the Mayo stand to reduce tongue ischemia. The gag is let down for 5 minutes every 30 minutes for ischemia reduction as well.17 The soft palate and posterior pharyngeal wall are infiltrated with 1% lidocaine with 1:100 000 epinephrine in preparation for the superiorly based flap. The palate is typically not split but retracted superiorly to provide adequate exposure for the surgery.

The desired flap width, having been predetermined by endoscopy and/or fluoroscopy, is then marked with a scalpel on the posterior pharyngeal wall. The width selected for this group of patients was typically 75% to 100% of the total posterior wall width. The length of the flap is determined by measuring the distance from the midsection of the soft palate to the posterior pharyngeal wall, while retracting the free edge of the soft palate slightly toward the posterior pharyngeal wall. The typical length of the flap is 1.5 to 2 cm, extending no lower than the midlevel of the oropharynx. Once this length is determined, the transverse portion of the incision is marked with a scalpel.

The relationship of carotid pulsations is then noted relative to the mucosal markings and the imaging studies. The incisions are carefully extended down through the constrictor musculature with a scalpel. Hemostasis is obtained with topical 1:1000 epinephrine and electric cautery. The dissection is extended through the visceral fascia, leaving the alar fascia intact. The superiorly based myomucosal flap is then elevated in an inferior to superior direction in the retropharyngeal space, superficial to the danger space or prevertebral space. The internal carotid artery, if underlying the dissection, is retracted laterally by direct pressure. The artery remains covered by the alar fascia.

Blunt dissection with a Kitner dissector is performed as much as possible to elevate the flap off of the alar fascia. The flap is elevated at least to the level of the atlas if not higher. A transverse incision is then made on the nasal surface of the soft palate beginning at the base of the uvula approximately 5 mm superior to the free edge on either side of the uvula and extending out laterally to the most lateral aspect of the soft palate. The incision remains straight, not following the curvature of the free edge of the soft palate own along the palatopharyngeal fold. Angled scissors are used to create a pocket through this incision into the substance of the soft palate extending close to the junction of the hard and soft palate.

2-0 Chromic sutures on a tapered needle are passed through the oral mucosa of the soft palate near the junction of the hard and soft palate into the pocket, brought out through the pocket, and passed through the inferior edge of the flap. They are then passed back through the soft palate pocket and back out through the oral mucosal layer, approximately 5 mm away from the initial entry of the suture. Five sutures are equally placed along the soft palate and inferior edge of the flap from one corner to the next. They are not tied until all sutures are passed. These sutures are all tied, pulling the flap up into the pocket of the soft palate.

Attention is then turned to the posterior pharyngeal wall defect. The muscle and mucosa on the inferior aspect of the defect are elevated. This posterior pharyngeal wall elevation extends down into the hypopharyngeal region, freeing up the musculomucosal layer and allowing it to be advanced upward into the base of the flap where it is sutured to the prevertebral fascia and muscle with 2-0 chromic sutures (stents are generally not placed). At the termination of the procedure, neither the flap nor the posterior pharyngeal wall defect is visible through the mouth without superior retraction of the soft palate. The nasal cavity, nasopharynx, and oropharynx are all copiously irrigated, and all clots are removed. Oxymetazoline drops (0.05%) are instilled into the nasal cavity, and the patient is extubated in the operating room. The patient is observed for several minutes for airway difficulties before leaving the operating room.

POSTOPERATIVE CARE

The protocol at our institution following pharyngeal flap is to keep patients in the pediatric intermediate care unit for at least 1 postoperative day under apnea, heart rate, and pulse oximetry monitoring. Patients are sent to a regular pediatric room once it is confirmed that they have not had any respiratory complications or obstructive apnea. Their postoperative diet is advanced from clear liquids on the first postoperative day to full liquids and a soft diet on the second and third postoperative days, respectively. They continue to receive intravenous clindamycin until peroral intake is adequate. Dexamethasone is also given for 36 to 48 hours (0.25 mg/kg every 8 hours). Oxymetazoline (0.05%) drops are changed to isotonic sodium chloride solution after 24 to 48 hours. Patients are discharged with a prescription for amoxicillin-clavulanic acid and acetaminophen-codeine typically 2 to 3 days after surgery. An outpatient follow-up visit is generally scheduled for 7 to 10 days after surgery.

POSTOPERATIVE EVALUATION

Patients are asked to return to the center approximately 6 months after surgery. Of this group of patients, 2 lived within 20 miles (32 km) of the hospital, 2 within 120 miles (192 km), 4 within 250 miles (400 km), 4 within 500 miles (800 km), 6 within 2000 miles (3200 km), 1 had to travel from the West Coast, and 1 was from the United Kingdom. Therefore, follow-up, though preferred at 6 months, 1 year, and 2 years, was not always according to this schedule because of the distances involved. All patients had clinical speech evaluation at these same intervals, and we also evaluated all out-of-town patients by videotape on semimonthly intervals. Nearly all patients were in speech therapy under our prescription, and the speech pathologists treating in local communities were instructed to send videotapes with specific speech samples and spontaneous speech. Ratings of nasal resonance were made in the same manner as the preoperative protocol.

RESULTS
SPEECH RESULTS

Hypernasal resonance and abnormal nasal air escape during speech was successfully eliminated in 18 of 20 cases. Hyponasality is a typical short-term finding, usually persisting for 6 to 12 months. With growth, hyponasality has decreased, and resonance balance has normalized in all cases that are more than 1 year postoperative. Postoperative endoscopic assessment of velopharyngeal closure has shown very wide pharyngeal flaps in all 18 cases that had a successful outcome (Figure 4). In the 2 cases with some residual nasal air escape and hypernasality during speech, 1 lateral port was noted to be wider than the other, resulting in a unilateral VPI. Of interest, in both of these cases with unilateral VPI, a prominent pulsation of the internal carotid artery was seen in the wider port. Although there was improvement in the speech in these 2 cases, there was residual VPI and hypernasality, and they are considered to be failures.18 Of the last 10 cases undergoing pharyngeal flap, there has been a 100% resolution of abnormal hypernasality and VPI (Table 2).

CAROTID ARTERY PLACEMENT

The radiographic studies performed prior to surgery proved to be highly predictive of potential problems related to the abnormal placement of the internal carotid arteries. Five cases had severe medial displacement of the arteries potentially placing them directly within the donor site for the flap (Figure 5). Table 3 shows the placement of the arteries as noted during surgery in relation to endoscopic and MRA or CT assessments. In 10 cases, the level of maximum medial deviation was at or above the base of C1. Although prominent pulsations were seen in the posterior pharyngeal wall in 13 cases, surgical exposure of the artery beneath the alar fascia occurred in 5. In the cases with the most significant medial deviation of the arteries, the placement was also close to the mucosal undersurface of the posterior pharyngeal wall. This abnormal position of the internal carotid arteries placed the arteries directly within the operative field for the donor site of the pharyngeal flap.

POSTOPERATIVE COURSE AND COMPLICATIONS

The average length of postoperative stay was 2.7 days, ranging from 2 days to 6 days. Ten patients were discharged after 2 postoperative nights, 9 after 3 postoperative nights, and 1 after 6 nights. There were no bleeding complications from the flap donor site. One patient developed a nosebleed unrelated to the surgery that required a return to the operating room to rule out the possibility that the bleeding was from the flap or soft palate. The total length of hospital stay was 3 days for this patient. Several patients had loud snoring in the immediate postoperative period without associated apnea or oxygen desaturations based on the monitoring protocol. The snoring tended to reduce markedly by the 14th postoperative day. Obstructive sleep apnea has not been observed in any of the 20 cases during hospitalization nor following discharge. There have been no bleeding complications and no transfusions. There were no other postoperative complications.

COMMENT

Velocardiofacial syndrome is the most common syndrome of clefting, constituting a high percentage of the disorders treated in cleft palate centers. Speech disorders, VPI, and hypernasality in particular, are more prevalent among patients with VCFS than in other patients with clefts. It is likely that there is a disproportionately increased frequency of cases of VCFS among patients undergoing surgical correction of VPI compared with the overall frequency of patients with VCFS in the cleft population. Because patients with VCFS have been documented to have significant pharyngeal hypotonia (supported by our findings of poor lateral pharyngeal wall motion in the present study), it is also likely that a relatively high percentage of failures to resolve VPI are in patients with VCFS.

Abnormal placement of the internal carotid arteries has been well documented in VCFS, leading to the recommendation for imaging of the cervical vessels prior to reconstructive pharyngeal surgery.5 However, in a study critical of that recommendation, Witt et al6 protested the financial cost to the medical care system that would be induced by the addition of MRA to the preoperative evaluation. In a response critical of the manner in which Witt and colleagues reached their conclusion, Shprintzen7 suggested that the risk of encountering abnormally placed internal carotid arteries during surgery far outweighed any potential cost issues.

Based on our findings in the present study, it is clear that preoperative MRA or CT angiography is essential in patients with VCFS who are to undergo pharyngeal flap surgery. In this series, 5 (25%) of the 20 cases had arteries that were within the donor site dissection. The procedure for pharyngeal flap described in this article is specifically designed to raise a very short musculomucosal flap leaving the alar fascia down, thus exposing the smallest possible segment of the carotids to potential injury. Even so, without specific knowledge of the placement of the arteries in these patients, the possibility for injury of the internal carotids was high in these 5 cases. As reflected in Table 3, in those 5 cases with the most medial placement of the arteries, the vessels were located very close to the mucosa of the posterior pharyngeal wall. The danger of this ectopic placement of the arteries is compounded by an abnormally thin pharyngeal muscle wall in patients with VCFS. Therefore, surgical dissection in these cases must be done very carefully. Other pharyngeal flap procedures require that very long and very wide flaps be raised, sometimes extending to the hypopharynx. In such procedures, there is no doubt that without specific knowledge of the placement of the internal carotid arteries, they would have been at risk to be injured or severed during surgery if performed in the 5 cases with arteries that were within the donor site dissection in our series.

Our series may have an ascertainment bias with respect to these arteries because several patients were referred only after other surgeons had recommended against surgery or discontinued the operation when the prominent vessel pulsations were observed intraoperatively. Although all patients assessed to be at risk intraoperatively had visible pulsations preoperatively, not all patients with preoperatively visible pulsations were assessed to be at risk. This finding may be related to alteration in carotid position from the typical sitting position for endoscopy to the supine neck–extended position for imaging or surgery.

The success rate with the specific procedure described in this report is acceptable, especially when one considers that the 2 failures occurred in the earliest of the surgical procedures performed. As is often true with any modification of a procedure, there is a learning curve, and once the goals and techniques of surgery are refined, implementation becomes easier over time. In the final 12 patients in the series, there were no failures, and the goal for elimination of hypernasality was achieved in all cases. Because almost all of these patients demonstrated poor or absent motion in 1 or both lateral pharyngeal walls (19 of 20 were bilaterally hypotonic), the goal in nearly all cases was to place a subobstructing flap. In 1 of the 2 cases in which VPI and hypernasality were not completely eliminated, it was noted that there was a prominent pulsatile vessel (the internal carotid artery) directly in the lateral port that persistently impinged on the lateral edge of the flap. It is unclear if the vessel caused that port to be stented open because of the constant pressure of the artery against the healing flap, or if the problem was some other unanticipated result of healing. However, in the later procedures in the series, the problem was not encountered. In both of the suboptimal cases, there was a unilateral insufficiency: 1 lateral port closing completely and 1 lateral port closing only partially with speech. In both of these cases, even though hypernasality was markedly diminished, it was not completely eliminated and therefore could not be categorized as a completely successful outcome.17 It should be noted that 5 of these 20 patients had already experienced surgical failure with other procedures.

There were essentially no significant postoperative complications in this series, including no evidence of obstructive sleep apnea either short or long term. Although respiration had initially been altered in many cases from predominantly nasal to oral, in most cases there was a gradual increase in the ability to exchange air nasally after 6 months. However, in no cases was the continued use of predominantly oral respiration accompanied by exercise intolerance or decreased vitality. The absence of postoperative complications in this sample is related to 2 factors that have been previously reported in the literature: the removal of tonsils and the confinement of the flap donor site to a short area almost entirely within the upper oropharynx and nasopharynx.9-10 This short flap donor site, closed vertically, prevents narrowing of the airway beneath the flap so that even if nasal respiration is partially compromised, oral respiration is unimpeded in the pharynx.

The elimination of VPI was followed by intensive articulation therapy that was checked frequently by speech evaluation for local patients and videotape for those coming from a distance. Speech therapy to eliminate abnormal articulatory compensations using specific techniques designed to eliminate glottal stop substitutions has been rapidly successful in nearly all cases and resulted in a complete normalization of speech intelligibility, articulation, and resonance.19

CONCLUSIONS

The specific type of pharyngeal flap surgery described in the present study has been highly successful in eliminating VPI in a patient population who have been considered to be at surgical risk and who have had a significant failure rate. Preoperative imaging of the major neck arteries is recommended for all patients with VCFS who are to undergo pharyngeal flap surgery.

Back to top
Article Information

Accepted for publication April 10, 2001.

This study was supported in part by funds from the Children's Miracle Network Telethon and by grants and donations to the Joseph and Annette Cooper Fund for Research in Velo-Cardio-Facial Syndrome at State University of New York Upstate Medical University and by grant 5PO1HD34980-03 from the National Institutes of Health, Bethesda, Md (Dr Shprintzen).

Corresponding author: Sherard A. Tatum III, MD, Division of Facial Plastic and Reconstructive Surgery, Departments of Otolaryngology and Pediatrics, State University of New York Upstate Medical University, 750 E Adams St, Syracuse, NY 13210 (e-mail: tatums@upstate.edu).

References
1.
Shprintzen  RJSiegel-Sadewitz  VLAmato  JGoldberg  RB Retrospective diagnoses of previously missed syndromic disorders among 1,000 patients with cleft lip, cleft palate, or both. Birth Defects Orig Artic Ser. 1985;2185- 92
2.
Lipson  AHYuille  DAngel  MThompson  PGVandervoord  JGBeckenham  EJ Velocardiofacial (Shprintzen) syndrome: an important syndrome for the dysmorphologist to recognize. J Med Genet. 1991;28596- 604Article
3.
MacKenzie-Stepner  KWitzel  MAStringer  DALindsay  WKMunro  IRHughes  H Abnormal carotid arteries in the velocardiofacial syndrome: a report of three cases. Plast Reconstr Surg. 1987;80347- 351Article
4.
D'Antonio  LDMarsh  JL Abnormal carotid arteries in the velocardiofacial syndrome. Plast Reconstr Surg. 1987;80471- 472Article
5.
Mitnick  RJBello  JAGolding-Kushner  KJArgamaso  RVShprintzen  RJ The use of magnetic resonance angiography prior to pharyngeal flap surgery in patients with velo-cardio-facial syndrome. Plast Reconstr Surg. 1996;97908- 919Article
6.
Witt  PDMiller  DCMarsh  JLMuntz  HRGrames  LM Limited value of preoperative cervical vascular imaging in patients with velocardiofacial syndrome. Plast Reconstr Surg. 1998;1011184- 1195Article
7.
Shprintzen  RJ Discussion: limited value of preoperative cervical vascular imaging in patients with velocardiofacial syndrome. Plast Reconstr Surg. 1998;1011196- 1199Article
8.
Golding-Kushner  KJArgamaso  RVCotton  RT  et al.  Standardization for the reporting of nasopharyngoscopy and multiview videofluoroscopy: a report from an International Working Group. Cleft Palate J. 1990;27337- 347Article
9.
Shprintzen  RJ Pharyngeal flap surgery and the pediatric upper airway. Int Anesthesiol Clin. 1988;2679- 88Article
10.
Shprintzen  RJSinger  LSidoti  EJArgamaso  RV Pharyngeal flap surgery: postoperative complications. Int Anesthesiol Clin. 1992;30115- 124Article
11.
Arvystas  MShprintzen  RJ Craniofacial morphology in the velocardiofacial syndrome. J Craniofac Gen Dev Biol. 1984;439- 45
12.
Golding-Kushner  KJCraniofacial Morphology and Velopharyngeal Physiology in Four Syndromes of Clefting [dissertation] New York Graduate School and University Center, City University of New York1991;
13.
Shprintzen  RJSher  AECroft  CB Hypernasal speech caused by hypertrophic tonsils. Int J Pediatr Otorhinolaryngol. 1987;1445- 56Article
14.
MacKenzie-Stepner  KWitzel  MAStringer  DALaskin  R Velopharyngeal insufficiency due to hypertrophic tonsils: a report of two cases. Int J Pediatr Otorhinolaryngol. 1987;1457- 63Article
15.
Argamaso  RV The pharyngeal flap in cleft lip and palate. Kernihan  DARosenstein  SWeds.Cleft Lip and Palate: A System of Management Baltimore, Md Williams & Wilkins1990;263- 269
16.
Senders  CWEmery  BESykes  JMBrodie  HA A prospective, double-blind, randomized study of the effects of perioperative steroids on palatoplasty patients. Arch Otolaryngol Head Neck Surg. 1996;122267- 270Article
17.
Senders  CSEisele  JH Lingual pressure induced by mouthgags. Int J Pediatr Otorhinolaryngol. 1995;3353- 60Article
18.
Shprintzen  RJ Conceptual framework for pharyngeal flap surgery. Bardach  JMorris  Heds.Management of Unilateral Cleft Lip and Cleft Palate Philadelphia, Pa WB Saunders1990;806- 809
19.
Golding-Kushner  KJ Treatment of articulation and resonance disorders associated with cleft palate and VPI. Shprintzen  RJBardach  JCleft Palate Speech Management: A Multidisciplinary Approach St Louis, Mo Mosby–Year Book Inc1995;327- 351
×