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5 The Unilateral Cleft Lip Nose Deformity
Fig. 5.7 Mild alar base
deformity in unilateral
cleft lip
Fig. 5.8 Moderate alar
base deformity in unilateral
cleft lip
135
purposes, the complete form including a Simonart’s band is managed as a complete
form; therefore, they may be considered a single type. Nasal deformity associated
with a unilateral cleft lip correlates well with these three types of deformity.
136
Fig. 5.9 Severe alar base
deformity in unilateral
cleft lip
Fig. 5.10 Left microform
unilateral cleft lip nose
deformity
P. Rossell-Perry
The Microform Unilateral Cleft Lip Nose Deformity (Figs.5.10,
5.11, and5.12)
Microforms are characterized by minor deciencies in lip height (less than 3mm),
and nose deformity is often associated even in the absence of an obvious cleft lip (a
muscular cleft may exist without skin or mucosa deciencies). These nasal abnormalities may vary in their presentation; however, they are mostly characterized by
alar base and nostril asymmetry and medial crus foreshortened. Septal and skeletal
deciencies are rare to be associated.
5 The Unilateral Cleft Lip Nose Deformity
Fig. 5.11 Right
microform unilateral cleft
lip nose deformity
Fig. 5.12 Right
microform unilateral cleft
lip nose deformity
137
Incomplete Unilateral Cleft Lip Nose Deformity (Figs.5.13,
5.14, and5.15)
The spectrum of incomplete cleft lips may vary from microforms to extended
incomplete clefts. Nose deformity usually correlates with the severity of an incomplete cleft lip. Sometimes the parents are unaware of their minimal malformations
in their children, especially when they have microforms. Based on the components
138
Fig. 5.13 Left incomplete
unilateral cleft lip nose
deformity
Fig. 5.14 Left incomplete
unilateral cleft lip nose
deformity
P. Rossell-Perry
of the nose, we may describe the characteristics of incomplete unilateral cleft lips
and their applications in surgical treatment.
Nasal oor The nasal oor has four sections as follows: nostril sill, primary palate
(alveolus), secondary palate (hard palate), and soft palate. The nostril sill and primary palate may be partially or not affected in this type of cleft lip.
Columella Even when the columellar component looks “short,” it is obliquely mal-
positioned and requires repositioning instead of lengthening. This concept is
5 The Unilateral Cleft Lip Nose Deformity
Fig. 5.15 Right
incomplete unilateral cleft
lip nose deformity
139
opposed to the traditional Millard’s philosophy that promotes the lengthening of the
columella using lip tissues.
Alar base Alar base distortion in incomplete cleft lips is characterized by alar base
asymmetry with alar hooding. In addition, nostril asymmetry, inward buckling of
the cleft side ala, and retrodisplacement of the cleft side alar dome may be observed.
Therefore, the need for alar base repair is conditional on the severity of the incomplete form.
Nasal tip Even in small deciencies of the lip, some abnormal changes can be
observed, as follows: the tip of the nose may be deected and the dome at the cleft
side is retrodisplaced resulting in decreased projection. The angle between the
medial and lateral crura is obtuse, and there may be some associated bone deciency of the maxilla on the clef side.
Nasal septum The nasal septum often deviates but not to the same extent as the
complete forms. The anterior nasal spine and the caudal septum are displaced to
different extents to the noncleft side.
Alar cartilage An obtuse angle exists between the medial and lateral crura on the
cleft side. In addition, inward buckling of the ala and retrodisplacement of the alar
dome on the cleft side affect the form and extension of the nasal vestibule. It is
important to mention that this structure may be congenital hypoplastic and may play
an important role in the quality of surgical outcomes.
140
P. Rossell-Perry
Nostril This component is asymmetric in comparison with the noncleft side. A
wider nostril is observed on the cleft side.
Maxilla The existence of a cleft may vary among patients with incomplete cleft
lips. Sometimes it exists as simple notch or as an alveolar cleft. Commonly, all of
these variants have an aligned maxillary arch. It is not rare to observe the association between incomplete cleft lip and incomplete cleft palate.
Complete Unilateral Cleft Lip Nose Deformity (Figs.5.16, 5.17,
and5.18)
This complex deformity involves distortion of the skeleton and soft tissues of the
nose. The existence of a hypoplastic segment (cleft side) limits lip and nose reconstruction. Even when clear evidence exists regarding the hypoplasia of the lateral
segment of the lip, there is not evident deciency of the cartilage structures of the
nose; therefore, total reconstruction of the nose is possible despite of the severity of
the cleft. Based on the nose components, we may describe characteristics of complete unilateral cleft lips and their applications to the surgical treatment. They
include the following:
Grossly asymmetric nose
Columella deviated toward the noncleft side
Nostril wider and retrodisplaced on the cleft side
Nostril margin buckles inward on the cleft side. This is because of bowing by the
internal vestibular web.
Maxilla deciency on the cleft side
Posterolaterally displaced alar base and piriform aperture on cleft side
Deviated caudal septum, premaxilla, and columella toward noncleft side
Fig. 5.16 Right complete
unilateral cleft lip nose
deformity
5 The Unilateral Cleft Lip Nose Deformity
Fig. 5.17 Left complete
unilateral cleft lip nose
deformity
Fig. 5.18 Left complete
unilateral cleft lip nose
deformity
141
Posterolaterally displaced alar dome
Increased angle between the medial and lateral crura
Shortened medial crus and lengthened lateral crus
Malpositioned upper and lower lateral cartilage
Nasal oor The nasal oor has four sections as follows (Figs.5.19):
142
Fig. 5.19 The nasal oor
in the unilateral cleft lip
and palate. This is an
intraoral view of a patient
with unilateral cleft lip and
palate illustrating the
absence of segments of the
nasal oor. (A) Nostril sill
(nasal vestibule). (B)
Primary palate (alveolus).
(C) Secondary palate (hard
palate). (D) Secondary
palate (soft palate)
(E) Nasal septum
P. Rossell-Perry
(a) Nostril sill. This is the most anterior portion of the nasal oor and represents the
nasal vestibule oor; this is repaired primarily using the lateral and medial elements of the cleft during lip repair and supported by the muscular reconstruction. The presence of Simonart’s band is not relevant for reconstructive
purposes, and it is usually resected during primary lip and nose repair.
(b) Primary palate (alveolus). This is the most anterior portion of the nasal skele-
ton. It is attached to the vomer (as the vomero premaxillary junction), the lower
segment of the nasal segment. It is primarily repaired using the Oslo protocol
[3]. A concern exists regarding the potential effect of the gingivoperiosteoplasty
(GPP) on the growth of the maxilla, which is why it is not used in different
protocols [4].
(c) Secondary palate (hard palate). It is represented by the posterior section of the
nasal skeleton and is composed of two bones: the upper maxilla (palatine process) and the palatine bone (horizontal plate). This section is repaired during the
primary palatoplasty and depends on the type of protocol used.
(d) Secondary palate (soft palate). This portion includes the palatine muscles cov-
ered by mucosa, is functional, and creates the anterior part of velopharyngeal
sphincter.
The primary and secondary palates are repaired depending on the protocol used,
for example, the primary and hard palate are closed together rst using the Oslo
protocol and the soft palate is closed rst using the Goteborg protocol.
All these elements in complete unilateral cleft lip and palates are absent and
should be repaired during primary surgery through the lip and palate surgery.
Alar base The lateral element of the cleft is attached to the skeletal structure (pyriform aperture of the maxilla). It should be released for proper alignment of alar
bases during primary lip repair. Muscular repair is also important providing support
for the nasal sill. Malposition of the alar base after primary cheilorhinoplasty is not
related to a lack of skeletal support (a common misconception). In fact, this occurs
because of a poor repair of the oral muscles.
5 The Unilateral Cleft Lip Nose Deformity
143
Columella The concepts used for columellar repair are the same as those used for
incomplete cleft lip repair. The columella should be repositioned instead of lengthened. The medial crus and dome medial repositioning (independent of the technique
used) allowed us to correct the columellar position.
The columella should not be elongated using lip tissues as Millard proposed. The
distal portion of caudal septum can be corrected by muscular action when it is properly done.
Nasal tip This structure is commonly affected in complete forms and to different
degrees. Its distortion is related to maxillary collapse; however, maxilla correction
is not necessary for nasal structure repositioning, and maxillary alignment occurs
spontaneously as a consequence of nose and oral muscle reconstruction providing
adequate support for the nose.
Nasal septum The nasal septum often deviates to different degrees and generates
aesthetic and functional distortions of the nose. Even when some authors promote
its primary correction as the key point of the primary cleft rhinoplasty, more studies
are required to conrm its long-term effect in cleft lip patients.
Alar cartilage Between the medial and lateral crura on the cleft side, an obtuse
angle exists depending on the severity of the nose deformity in unilateral cleft lips.
Its role in the nasal vestibule determines the extent of the corrective surgery. The
orthopedic nasoalveolar molding corrects the nasal vestibule but only temporarily
which is why the primary surgery is always needed. The author creates the surgical
nasal molding concept producing a surgical extension of the vestibule but without
presurgical treatment.
Nostril This component is asymmetric in comparison with noncleft side, and wider
nostril is observed on the cleft side; therefore, the objective of the primary repair is
to create symmetric nostrils.
Maxilla Sometimes the surgical repair of the lip is sufcient to produce an align-
ment of the maxillary segments independent of the width of the cleft or maxillary
collapse. The author named this effect as the “surgical nasoalveolar molding” which
is evidence of the lack of need for plates. In addition, there is a lack of evidence
supporting the effect of orthopedic devices for nasal and maxillary molding, and
probably the best therapeutic molding is the one produced by repaired muscular action.
Surgical Protocol
Primary cleft rhinoplasty is an important step in cleft lip and palate reconstruction,
and it is performed actually during the primary lip repair for all types of cleft lips;
however, this complex deformity requires soft tissue and skeleton management at
144
Table 5.1 Unilateral cleft lip nasal repair surgical protocol
Age
3–6months Microform Nasal base shortening
5years All complete Alveolar cleft closure
7–9years All complete Alveolar bone graft
16–18years Secondary rhinoplasty
Degree of severity
Incomplete Rotational composite ap technique
Mild complete Rotational composite ap technique
Moderate complete VYZ cleft rhinoplasty
Severe complete VYZ cleft rhinoplasty + septoplasty
Surgery
Intermediate rhinoplasty or revision
P. Rossell-Perry
different surgical times. Since the neonatal presurgical management to the secondary rhinoplasty renement during the adulthood, the nose deformity associated with
cleft lip requires different interventions from those of the interdisciplinary cleft
team to achieve good functional and aesthetic outcomes. Management during the
timeline of the patient’s life is presented in Table5.1. The presented protocol for
primary unilateral cleft lip nose repair is indicated according to the type of unilateral
cleft lip [5, 6].
References
1. Rossell-Perry P.The surgical nasoalveolar molding: a rational treatment for unilateral cleft lip
nose deformity and literature review. Plast Reconstr Surg Glob Open. 2020;8(8):e3044.
2. Millard DR.A rhinoplasty tetralogy: corrective, secondary, congenital, reconstructive. Boston:
Little Brown & Company; 1996.
3. Åbyholm FE, Borchgrevink HC, Eskeland G.Cleft lip and palate in Norway: III.Surgical
treatment of CLP patients in Oslo 1954–75. Scand J Plast Reconstr Surg. 1981;15(1):15–28.
4. Berkowitz S.Gingivoperiosteoplasty as well as early palatal cleft closure is unproductive. J
Craniofac Surg. 2009;20(8):1747–58.
5. Rossell-Perry P.Rotational composite ap technique for primary incomplete cleft nose defor-
mity. Plast Reconstr Surg Glob Open. 2020;8(6):e2870.
6. Rossell-Perry P.Primary unilateral cleft lip nasal deformity repair using VYZ plasty: an anthro-
pometric study. Indian J Plast Surg. 2017;50(02):180–6.