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Pharyngeal Flap and the Internal Carotid in Velocardiofacial Syndrome
Sherard A. Tatum III, MD;
JaKwei Chang, MD;
Natalie Havkin, MS;
Robert J. Shprintzen, PhD
Arch Facial Plast Surg. 2002;4:73-80.
ABSTRACT
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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.
INTRODUCTION
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.
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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*
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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.
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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*
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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:
- speech and language evaluation including a group
rating of nasal resonance on a 5-point scale (hyponasal, normal, mild, moderate,
and severe hypernasality)
- genetic/dysmorphologic evaluation
- cytogenetic/molecular genetic evaluation including
FISH
- flexible fiber optic nasopharyngoscopic evaluation
- multiview videofluoroscopic evaluation in at least
frontal and lateral views
- facial plastic surgery
- immunologic evaluation
- audiologic evaluation
- magnetic resonance angiography of the neck vessels
and magnetic resonance imaging of the brain and spine or CT scanning
- otolaryngologic evaluation
- 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.
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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.
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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.
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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).
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Figure 3. Hypertrophic tonsils (t) seen
in lateral view videofluoroscopy behind the velum (v) and positioned posteriorly
in the oropharynx.
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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).
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Figure 4. Wide pharyngeal flap as seen endoscopically
1 year following surgery. The lateral ports are patent, but small.
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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.
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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.
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Table 3. Carotid Pulsations as Seen on Endoscopy in Relation to Magnetic
Resonance Angiography or Computed Tomography Evaluation and Findings at Surgery*
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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.
AUTHOR 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{at}upstate.edu).
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.
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