The submucous cleft palate: Diagnosis and therapy

R. Reiter
a,
*, S. Brosch
a
, H. Wefel
b
, G. Schlo mer
b
, S. Haase
b
a
Department of Ear, Nose and Throat Surgery, Section of Phoniatrics and Pedaudiology, University of Ulm, Frauensteige 12, 89070 Ulm, Germany
b
Department of Cranio-Maxillo-Facial Surgery, University of Ulm, Albert-Einstein-Allee 11, 89081 Ulm, Germany
1. Introduction
The submucous cleft palate (SMCP) is a subgroup of cleft palates
with a prevalence of 1:1250 to 1:6000 [1]. Some of the SMCPs
present chronic ear-problems as a result of Eustachian tube
dysfunctions [2,3] and hyper nasal speech in 53% [4]. After
adenoidectomy a SMCP without symptoms can show symptoms
like hyper nasal speech [5]. However, up to 55%patients with SMCP
show no symptoms. In up to 17% SMCPs occur in syndromic
malformations, e.g. Down syndrome [6].
Early diagnosis of SMCP is necessary because palate closure
should be made at an early age to achieve an improved outcome in
speech and hearing [79]. SMCPs are a result of insufcient median
fusion of the muscles of the soft palate during palatogenesis.
Calnan dened in 1954, that there have to be the three main
morphological symptoms for diagnosing a SMCP [10]: (a) a bid
uvula, (b) absent posterior nasal spine, leading to a bony notch in
the posterior end of the hard palate and (c) a translucent zone
(zona pellucida) in the midline of the soft palate, which is due to
the separation of the musculus levator veli palatine (Fig. 1). The
levator veli palatine muscle is displaced anteriorly, which leads to
velopharyngeal insufciency, middle ear effusion and nasal reux
[10,11]. As the bony notch in the posterior end of the hard palate
and muscle defect is hidden under the mucosa, SMCP is often
missed [6,11]. Also occult variants of SMCPs (occult SMCP) need
to be mentioned. These show palatal underdevelopment, diastasis
of the palatal muscles and deciency in length or thickness leading
to velopharyngeal insufciency [1,11].
Therapy of patients with symptomatic (i.e. recurrent otitis
media with effusion and/or hyper nasal speech) SMCP and occult
SMCP is a veloplasty. The procedure has the aim of uniting the
levator veli palatine muscles and elongation of the soft palate (up
to 1 cm) [1,6].
The aimof this study was (a) to investigate the age of diagnosis,
symptoms and palate ndings in a huge collective of patients
(n = 439) with symptomatic SMCP/occult SMCP and (b) to evaluate
the efciency of a surgical technique developed by Haase. This
operative procedure consists in a lengthening of the soft palate by
uniting the musculi levator veli palatini with a buttery-suture.
2. Methods
2.1. Subjects/investigations
Patients records from439 individuals (244 males/195 females)
with symptomatic SMCP, who received veloplasty with the same
buttery-suture in the period from 01/1981 to 12/2009 in the
Department of Cranio-Maxillo-Facial Surgery of the University of
International Journal of Pediatric Otorhinolaryngology 75 (2011) 8588
A R T I C L E I N F O
Article history:
Received 20 July 2010
Received in revised form 13 September 2010
Accepted 6 October 2010
Available online 26 November 2010
Keywords:
Veloplasty
Velopharyngeal insufciency
Hyper nasal speech
Hearing loss
A B S T R A C T
Objectives: To investigate age of diagnosis, typical symptoms, nding of the palate, therapy options and
accompanying diseases.
Methods: A retrospective analysis of 439 patients with symptomatic submucous cleft palate (SMCP),
who received a veloplasty operation (buttery suture technique developed by Haase) was made.
Results: SMCP was initially diagnosed at the mean age of 4.9 years. Main symptoms were hyper nasal
speech (51%) and conductive hearing loss (45%), which resolved after veloplasty (often in combination
with adenotomy and insertion of ventilation tubes). Typical ndings of the palate were a lack of posterior
nasal spine (68%) and bid uvula (59%). Following surgery 17.1% required speech therapy and 5.5%
needed velopharyngoplasty due to continuing hyper nasal speech.
Conclusion: SMCP is often diagnosed very late, though symptoms of velopharyngeal insufciency (hyper
nasal speech, Eustachian tube dysfunction) and bid uvula are present. We therefore recommend that all
patients with such ndings are examined by an appropriate specialist such as Phoniatrics,
Otolaryngologist and Oral-Maxillofacial-Surgeon so that early diagnosis and palatoplasty can be
performed. The veloplasty operation (buttery suture technique) can be recommended as a safe therapy
for velopharyngeal insufciency for patients with symptomatic SMCP.
2010 Elsevier Ireland Ltd. All rights reserved.
* Corresponding author. Tel.: +49 0731 500 59705; fax: +49 0731 500 59702.
E-mail address: rudolf.reiter@uniklinik-ulm.de (R. Reiter).
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology
j our nal homepage: www. el sevi er . com/ l ocat e/ i j por l
0165-5876/$ see front matter 2010 Elsevier Ireland Ltd. All rights reserved.
doi:10.1016/j.ijporl.2010.10.015
Ulm, were retrospectively reviewed. In a subgroup of 140 patients
and in patients with remaining hyper nasal speech and/or
inadequate pressure consonants after veloplasty additional mal-
formations were assessed. Symptomatic SMCP means, that these
patients suffered from velopharyngeal insufciency with hyper
nasal speech and/or Eustachian tube dysfunction. All patients were
assessed by the ENT-Department, the Department of Cranio-
Maxillo-Facial Surgery (palatoplasty, if necessary velopharyngo-
plasty according to Sanvenero-Roselli [12]) and the Section of
Phoniatrics and Pedaudiology. Phoniatric-pedaudiological assess-
ment included otological examination, palate examination (bid
uvula, translucent zone, bony notch in the posterior end of the hard
palate) and audiological investigations (pure tone audiogram,
tympanometry, brain stemevoked response audiometry). Further-
more, articulation and speech sound was assessed perceptual by a
speech pathologist. Any hyper nasal speech was graded 1 (mild)
through 2 (moderate) to 3 (severe). Complete audiologic and
speech assessments were conducted pre-operatively as well as 6
weeks (standard deviation, SD 1.6 weeks; range, r 59 weeks) and
every 6 months (SD 2.8 months; r 512 months) post-operatively
for all patients till adolescence. For a subgroup of 92 patients a mean
follow up till the age of 14 years (SD 2.6, r 1116 years) has been
available already. The parents lled out a questionnaire with
information concerning birth, general development and development
of the language. Surgery was only offered to symptomatic patients
with history of recurrent otitis media with effusion and/or hyper
nasal speech. Patients without any symptoms did not receive any
surgery.
2.2. Surgical technique
The veloplasty with buttery-suture developed by Haase has
the aim of uniting the levator veli palatine muscles and the
technique will be described step by step: Step 1: a saggital scalpel
cut is made in the mucous membrane from the edge of the hard
palate to the base of the uvula. Step 2: the levator veli palatine
muscles are dissected with blunt scissors. Step 3: the dissected
muscles are united with a buttery-suture in the midline of the
soft palate using an absorbable material like Vicryl
1
2-0 (Fig. 2a).
The result is shown in Fig. 2b. Step 4: The surface layer is closed
with an absorbable suture, e.g. Vicryl
1
4-0. The result is that the
soft palate has been elongated, allowing velopharyngeal closure.
The operation is performed under general anaesthetics and
requires only about 10 min. It can therefore be usually performed
in an ambulatory setting and does not require a naso-gastric tube
to be passed.
The study was exempted from institutional review board
approval because the questionnaires and clinical physical exam-
inations were administered as part of standard clinical care to
assist diagnosis and treatment planning for SMCP. All patients gave
written informed consent for the veloplasty operation.
3. Results
SMCP was rst diagnosed at a mean age of 4.9 years (range 0.6
66.1 years). All patients were identied by classic symptoms and
physical examination. The main symptoms were in 51.0% hyper
nasal speech(10.0%mild, 19.0%moderate, 22.0%severe), Eustachian
tube dysfunctions with conductive hearing loss (45.1%) and nasal
reux of meal and liquid (21.6%). All three symptoms together were
observed in8.0%(Table 1). Weak pressure consonants were foundin
28.9% and hyper nasal speech combined with weak pressure
consonants in 15.6% pre-operatively.
The preoperative anatomical ndings were assessed for all
patients. At least one nding was observed in every patient. In 299
cases (68.1%) a bony notch in the hard palate, in 259 cases (58.9%)
a bid uvula and in 198 cases (45.1%) a zona pellucida was
observed. Often a combination of 2 or 3 main symptoms was
detected (in 23.9% all 3 signs, Table 1).

Fig. 1. Submucous cleft palate with the three main morphological symptoms: (1)
bid uvula, (2) transluscent zone, and (3) lacking posterior nasal spine, leading to a
bony notch in the posterior end of the hard palate.

Fig. 2. Step 3: the dissected muscles are united with a buttery-suture in the
midline of the soft palate using an absorbable material like Vicryl
1
2-0 (a). The
result is shown in (b).
R. Reiter et al. / International Journal of Pediatric Otorhinolaryngology 75 (2011) 8588 86
A differentiation between SMCP and occult SMCP (6/439, 1.4%)
was not made for data analysis. The denitive diagnosis of occult
submucous cleft palate was dependent on the intraoperative
exploration of the soft palate muscles.
25 out of 140 [17.9%] investigated patients had additional
malformations (Table 2). The mean age of diagnosis in this
subgroup was equally high (4.6 years, range 0.69.8 years).
Before diagnosis of SMCP 50% received surgery one or more
times: 29% out of them an adenoidectomy, 12% out of them a
tonsillectomy and 32% myringotomy and/or insertion of ventila-
tion tubes (up to 4 times). After adenoidectomy or tonsillectomy
22% noticed a hyper nasal speech or enhancement of it.
Closure of the palate was usually done 3 months (SD 1.2; r 26
months) after diagnosis in the Department of Cranio-Maxillo-Facial
Surgery. 1.7%of operations were delayed as the patients suffered from
an internal disease (e.g. problems in blood coagulation).
Six months (SD 2.4; r 515 months) post-operatively in more
than50%of the patients a pre-operative diagnosedhyper nasal speech
resolved by the primary veloplasty. Concerning the degree of the
hyper nasal speech, it was noted that mild forms can be repaired in
84%. In case of a moderate form nearly 70% can be repaired to normal
ndings. Patients with a severe hyper nasal speech have the poorest
rate of success for a normal speech with only 22.7%. In the cases with
moderate hyper nasal speech additional postoperative speech
therapy for an average period of 6 months (SD 2.2 months; r 3
16 months) was necessary. In the severe cases additional speech
therapy and velopharyngoplasty had to be performed (24/97). After
these additional therapies 91% of all patients developed normal
speech after an average period of 12 months (SD 2.9; r 816
months). Hyper nasal speech remained in 5.0%, inadequate pressure
consonants in 4.0% and both in 3.2%. Twenty-ve percent of these
patients with remaining symptoms had a syndrome with mental
retardation.
A subgroup of 92 patients had available follow-up date through
the age of 14 years (SD 2.6, r 1116 years). Hyper nasal speech
became evident in only 4% of these patients when the adenoid tissue
atrophied during adolescence.
45.1% patients were found preoperatively to have Eustachian
tube dysfunctions with conductive hearing loss (dened as hearing
loss of 25 dB in both ears). From these patients with hearing
problems 35.9% (61/198) received a myringotomy (50 at the time
and 11 subsequent veloplasty), 64.1% (127/198) a ventilation tube
insertion on both sides (112 at the time and 15 subsequent
veloplasty) and 5.0% (10/198) needed a tympanoplasty because of
cholesteatoma subsequent veloplasty. Twelve months (SD 1.9; r
1014 months) after the operation 156 (78.8%) had normal hearing
(dened as hearing loss of <25 dB in the better hearing ear).
Unfavourable results, i.e. sleep apnea, airway comprise in
addition to unacceptable nasal resonance were not observed.
4. Discussion
4.1. Outcome comparison of palate ndings
Not every SMCP patient shows the typical Calnan triad [10]. In
our study it was only about 25% (see Table 2). In a Chinese study of
47 patients, 91%had a translucent zone, 85% a bid uvula and 80% a
bony notch on the end of the hard palate. The three symptoms
together were not investigated [13]. A different study of 108
patients showed the gures to be 85%, 93%, and 100% respectively
[4]. Lack of awareness of SMCP and its variability in presentation
may be the reason that it often goes undetected [6,11,13].
4.2. Outcome comparison of SMCP symptoms
Hyper nasal speech was the primary symptom in SMCP in
almost 51% of our patients and this was in accordance to other
studies [4,6,14]. After adenoidectomy or tonsillectomy 22% noticed
a hyper nasal speech for the rst time or an enhancement of it. This
was also described before [5,6,11].
Following successful palatal surgery nearly 50% of them
(mainly those with severe forms) still had remaining hyper nasal
speech, albeit improved. To gain a normal speech in this subgroup
the repair of velopharyngeal insufciency has a success rate
ranging from 28 to 97%, depending on the study [1,6,8,13]. An
important reason for the success might be the age at the time of
operation. In generally, younger patients have better outcomes
regarding velopharyngeal insufciency. The present literature
lays down the age between 12 and 18 months and below 7 years,
respectively for a good result in relation to hyper nasal speech [7
9,11,15]. Of course, this depends on early diagnosis, which does
not always occur. Our results conrmthe preliminary results that
SMCP is still late diagnosed rather late [6,11]. One reason
therefore might be that this malformation has a low prevalence
of 1:6000 [1]. Another reason is that especially the occult SMCP is
not known to every physician. Half of our patients had prior
received ENT-surgery for one or several times before SMCP was
diagnosed. It is known that children with craniofacial malforma-
tions often suffer from orofacial clefts. A prevalence of about 17%
has been reported [6,11] and was conrmed in our study.
Unfortunately, SMCP has not been considered previously in this
subgroup, a fact that is reected by the late mean age of diagnosis
(4.6 years).
Table 1
Preoperative symptoms.
Symptoms n [%] Preoperativ anatomical ndings n [%]
Nasal reux of meal and liquid 21.6% [92/439] Bid uvula 58.9% [259/439]
Hyper nasal speech 51.0% [224/439] Absent posterior nasal
spine/bony notch in the posterior
end of the hard palate
68.1% [299/439]
Impaired hearing due to middle ear effusion 45.1% [198/439] Translucent zone 45.0% [198/439]
All symptoms (nasal reux, hyper nasal
speech and middle ear effusion)
8.0% [35/439] All three signs (bid uvula,
bony notch, translucent zone)
23.9% [105/439]
Pre-operative anatomical ndings were available for all 439 patients. All patients had at least one of the three main symptoms (bid uvula, absent posterior nasal spine or
zona pellucida), except the 6 patients with occult SMCP. Often a combination of 2 or 3 main symptoms was observed (in 23.9% all 3 signs).
Table 2
List of associated syndromes/malformations.
Associated syndromes n=25/140 [17.9%]
Down syndrome 9
Pierre-Robin-Sequence 2
Ulrich-Turner-syndrome 1
Roberts-syndrome 1
Klippel-Feil-syndrome 1
Cornelia de Lange syndrome 1
Di George syndrome 1
Unknown syndrome 9
n=number; [%]: per cent.
R. Reiter et al. / International Journal of Pediatric Otorhinolaryngology 75 (2011) 8588 87
The children who did not improve hyper nasal speech/
inadequate pressure consonants had a syndrome with mental
retardation (e.g. Down syndrome) in 25%. This circumstance might
be an additional cause for the persisting problems or little success
of additional speech therapy needed in moderate or severe cases.
4.3. Advantages of the surgical technique
Besides the time of palate repair, the surgical technique is also
important for the outcome concerning hyper nasal speech [16]. The
palatoplasty is a pushback-technique for lengthening the velum
rst described by Calnan [10]. This method slides the muco-
periosteumof the hard palate backward to lengthen the soft palate.
The average operation time is 60 min [17]. The big drawback is that
this approach is susceptible to intricacies, because it is invasive and
bloody. Calnan observed in a follow up study that 13/18 (72%) had
normal speech after surgery with the push-back technique [10]
whereas in a different study it was found that 9/12 (75%) had
residual velopharyngeal inadequacy with hyper nasal speech [18].
The pharyngeal ap technique has a success rate of about 80%, but
shows complications like sleep apnoe and airway comprise in
addition to unacceptable hyponasality. The sphincter pharyngo-
plasty with overlapping posterior tonsillar pillar myomucosal aps
documented resolution of symptomatic velopharyngeal dysfunc-
tion in 72% of 162 patients. Treatment of velopharyngeal
dysfunction by augmentation of the posterior pharyngeal wall
with autogenic materials such as cartilage, fat or pharyngeal
mucosal ap is not promising, because absorption of the material is
unavoidable [1,16]. Pensler and Bauer described a rat of normal
speech in 75% in patients that had surgery (levator repositioning
and palatal lengthening technique) before the age 2 years [8].
The velopharyngoplasty by Sanvenero-Rosselli uses a cranial
stemmed ap of the dorsal pharyngeal tissue for closure of the
SMCP. In this case the average operation time is nearly 30 min and
a postoperative nasogastric tube is needed [11,12].
In comparison to the previously mentioned methods the
method of Haase described here has the advantages of a shorter
operation time (about 10 min) and lacks the need of a nasogastric
tube [11,12]. The operation can be performed in an ambulant
setting increasing convenience and cutting costs. The results of a
normal speech were comparable or superior to others (91% of all
patients). In severe cases additional speech therapy and velophar-
yngoplasty was necessary [13]. Unfavourable results, i.e. sleep
apnea, airway comprise in addition to unacceptable nasal
resonance were not observed [16]. We additionally have an
experience with a huge collective (n = 439) in comparison to
different groups, who dealt with less than 10 patients [1,16].
4.4. Outcome comparison of hearing improvement
Conductive hearing loss was present in upto 96%of patients with
cleft palate. In comparison, healthy non-cleft palate children had a
prevalence of about 12.9% [19,20]. In our study 45.1% of the patients
were suffering hearing loss preoperatively. Twelve months postop-
eratively 78.8% of our patients had normal hearing. This nding is in
accordance with another study were 75% of the patients normalized
their Eustachiantube dysfunctionafter repair of the cleft palate [6]. A
shortcoming of the mentioned study was that there was no
differentiationabout the hearingimprovement due veloplastyalone.
5. Conclusions
Despite the presence of a bid uvula and symptoms of
velopharyngeal insufciency such as hyper nasal speech and
persisting Eustachian tube dysfunction SMCP is often diagnosed
very late. Children with craniofacial dysmorphic syndromes have
higher rates of submucous clefting. We therefore recommend that
all patients with such ndings are examined by appropriate
specialists such as Phoniatrics, ENT-Surgeon and Oral-Maxillofa-
cial Surgeon so that early diagnosis and palatoplasty can be
performed. The developed veloplasty with buttery-suture by
Haase is a safe and effective treatment for patients with
symptomatic SMCP that means in patients who suffer from
recurrent otitis media with effusion and/or hyper nasal speech due
to velopharyngeal incompetence. In patients with a history of
recurrent otitis media with effusion veloplasty is done in
combination with myringotomy and/or insertion of ventilation
tubes and adenoidectomy.
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