Cleft lip nose and secondary deformities of cleft lip, nose and palateDr Sumer YadavMch - Plastic and reconstructive surgeon
Introduction Improvement in lip repair results Less satisfactory in nasal deformity1. Cleft lip and palate abn studies more2. Lip line beneath the nose deemphasize the
lip, whereas nose is always more obvious
Total interrelated lip , nose ,maxilla & palate deformity poorly analysed
Brown and mcdowell – not to appear in public and retire
Not acceptable Multidisciplinary concept Interconnected so isloated operation give less
than optimal results Systematic evaluation , integrated plan and
quality surgical technique
Degree of deformity Original defect Method of repair Craniofacial growth patterns Orthodontic therapy Prosthodontic rehabilitation
Pathogenesis 1. Agenesis of tissue from deficiency of
mesoderm and ectoderm in primary palate region
2. Mechanical stresses as cleft widens in utero
After cleft is established the premaxilla segment begins to move forward at 6th week , pulled by growing nasal septum to which it is attached by septopremaxillary ligament.
The alar base region is retroposed because of lack of forward development of maxilla
There is increased widening of the distance between base of columella and alar base
When medial and lateral crura of alar cartilage are pulled apart --- lowering of alar arch in dorsal direction --- fascia nasalis is tightened
The infundibulum between two cartilages disappears and alar arch is forced to tilt downward in a caudal direction.
Lower edge of alar cartilage is also displaced dorsally
Shortening of columella in bilateral clefts is due to wide distraction of the alar cartilages. the alar domes are separated and ant parts of medial crura are displaced away from tip of nasal septum
The columella is therefore progressively shortened towards its base at its junction of prolabium
Severity is directly related to extent of lip deformity and alveolar cleft
Asc with high orbicularis defects , def of nostril sill , nasal spine and the maxilla
Components of nasal deformity 1. Defect of lower lateral cartilage on cleft side 2. Nasal septum3. Columella4. Nasal tip5. Nasal pyramid
Maxilla cleft and hypoplasia Incomplete rotation of alar cartilage Hypoplasia of lateral crus of alar cartilage Distortion of alar cartilage by mechanical forces Loss of orbicularis muscle continuity Abnormal muscle tension on nasal str esp alar base Malpositioning of maxillary segments
Etiology Intrinsic defect or def of growth and development of
the nasal structures Intrinsic hypoplasia of involved soft tissue and
cartilages Failure of neural crest cells to migrate results in
absence of mesodermal penetration of soft tissue in cleft region
Tissue def of cleft lip , a def of maxilla or abnormal muscular pull on nasal structures
Pathologic anatomy1. Nasal tip; alar cartilage and columella2. Lateral bony platform ; piriform aperture3. Midline supporting structures ;
cartilaginous septum and anterior nasal spine
Tilted tripod The tripod consists of dorsal portion of septum and
nasal bones and 2 alar arms Tilting result from maxillary hypoplasia with
secondary deformity of septum and cleft ala Convex deformity of septum and vertical bending of
septum posterior to junction of membranous and cartilaginous portions of septum
Restriction of caudal border of septum in ant thrust causes it to bend toward the normal nostril
When one of bony platform is def the tripod collapse on the ipsilateral ala and deflects the septum into the contralateral normal naris
With marked hypoplasia the septum is lifted out of the vomerine groove and encroaches on opposite nostril
Basic tenet of Cleft development Failure to reconstruct the nasal floor in
primary cleft repair leaves the nose attached directly to lip through the intact orbicularis and to palate through the lateral mucoperiosteum of alveolar cleft
Although the lip defect may improve with time the primary nasal deformity will never improve
Pathologic anatomy 1. Tip of nose is deviated toward noncleft side2. Dome on cleft side is retrodisplaced 3. Angle between medial and lateral crura on cleft side
is excessively obtused4. Buckling of alar cartilage5. Alar facial groove on cleft side is absent 6. Bony def of maxilla on cleft side7. Circumference of naris is greater on cleft side
Pathologic anatomy1. Naris is retrodisplaced2. Columella is shorter in AP dimension3. Medial crus is displaced 4. Columella is positioned obliquely 5. Nasolabial fistula6. Absence of nasal floor7. Hypertrophy of inferior turbinate on cleft side8. Displacement of noncleft maxillary segment
Caudal rotation of alar cartilage Importance Alar cartilage must be lifted to shorten on the side of
cleft and to level the nostril rims Elevation of the alar cartilage with the attached nasal
lining corrects the oblique fold within the vestibule When alar cartilage is lifted , the compound curve
that produces the typical flare of cleft lip nostril is avoided
Reasons for unsatisfactory result following primary repair Alar cartilage should be lifted at start of opn
before nostril floor is closed so that vault of the vestibule is established and limning is in position
Scarring from incision made in nostril lining causes contraction and stenosis
Large changes in size and shape of nose that occur during growth spurts
Presurgical orthopedic procedures Displaced tissue should be returned to their
normal positions before a defect is repaired Maxillary segments are aligned and
displacement of nasal septum is reduced
Surgical procedures Hemirhinoplasty to reposition the displaced alar cartilage Elevate the alar cartilages with its attached vestibular lining
to recreate the vault of vestibule and to obliterate the vestibular ridge
Dissection through upper buccal sulcus deep to base of nostril . The alar base is separated from piriform aperture
Continue over ant surface of alar cartilage and extends to completely undermine the skin of nose till across the nasal tip over the lower part of upper lateral cartilage on non cleft side
Wide undermining for easy lift and contraction and shortening of lengthened skin on cleft side of nose
Scissors inserted through the upper buccal sulcus of ant nasal spine to liberate the medial crus of alar cartilage in columella from its attachment to overlying skin
If alveolar arch is cleft , a mucosal flap is preserved from the pared margin of lateral lip element based on ant buccal sulcus end
The nostril linining is freed from lower part of cartilaginous septum and from lateral wall of nose
The alar cartilage rotated upward and forward raising the nostril rim and reestablishing the vault of vestibule with obliteration of vestibular fold
The infundibulum is reestablished and upper edge of alar cartilage lies above and sup to caudal border of upper lateral cartilage
Correct site of dome and first lifting suture is selected by points of forceps lifting from inside the nasal vestibule
The suture pass through mucosa and alar cartilage upward and medially to emerge in nasion toward noncleft side
A second elevating suture is passed through lateral crus of alar cartilage
bolsters are used to lift and round out dome and lateral wall of vestibule
These make the nostril in level with contralateral side and established the vestibular vault
The nostril sill is augmented with local flaps and muscle union is established beneath the floor of nose
At completion of lip repair remove ,replace and realign the direction of lifting suture
Do not attempt to realign the cartilaginous septum completely – fibrosis and scarring
Bilateral cleft lip nose primary repair PSO alignment of bony platform ,premaxilla
is centraised and twisting is corrected 2 stages permit First columella so lengthened to release the
nasal tip and elevation of alar cartilages 6 wks latter simultaneous repair of lip and
nose
For minor deformities, a closed approach significant reconstruction, the open approach When significant inferior turbinate hypertrophy is present, turbinate reduction to
enable better visualization of the nasal cavity. then open rhinoplasty. V-shaped columella incision; the nose is skeletonized; and all cartilaginous and
bony deformities are visualized. The entire septal cartilage exposed by lateral reflection of the medial crura of the lower nasal cartilages.
dissect the mucoperichondrium on either side of the septal cartilage to fully expose the septum, the perpendicular plate of the ethmoid bone, the crest of the maxilla, the vomer, and the anterior nasal spine.
If only the caudal portion of the septum deviates from the midline, free this portion from the underlying maxilla and nasal spine.. The septum is secured in the midline with sutures to the nasal spine. Cartilage grafts and strut used to maintain the contour and provide support.. Symmetry of the domes with cartilage repositioning or augmentation using cartilage grafts.
Osteotomies when skeletal deformities and deviations are present and composite grafts from the ear are to correct significant lining deficiencies. The incision is closed in a V-Y fashion to provide additional columella length.
Finally, if the cleft side nostril is significantly smaller than that of the non-cleft side, corrected with composite graft from the conceal bowl of the ear.
Timing of the definitive nasal deformity correction It is deferred until 1. closure of the possibly coexisting
oronasal/palatal fistulas, 2. bone grafting of the alveolus and the
hypoplastic maxilla3. orthodontic alignment of the maxillary
dentition.
Reasons To achieve a symmetric result it is critical that the
alar bases start at a spatially symmetric level. depressed base on the cleft side is raised, through
alignment of the maxillary segments, alveolar bone grafting, or osteotomies during orthognathic surgery.
If the alar base is still depressed, onlay cortical bone grafting is done
When oronasal and/or palatal fistulas are present, saliva and food particles regurgitate into the nasal cavity, irritating the nasal mucosa and creating tissue thickening which exacerbates the airway obstruction.
Timing of repair1. Simultaneous primary lip and alveolar repair Interfere with nasal and maxillary growth due to
postoperative scarringTechnical difficult – small and fragile cartilages2 preschool age Social pressure at 4 to 6 yr
Bardach and salyer delayed till 8 to 12 yrs To allow completion of orthodontic correction of
skeletal base To allow growth and development of lower lateral
cartilages for support stable stronger support for reconstructed nasal tip
To allow bone grafting of hypoplastic maxillary segment on cleft side --- more symmetric alar base , improving conditions for nasal deformity correction at latter stage
Nasal growth is completed by 16yr in females and 18 yr in females
Highest growth activity was in suprapremaxillary and anterior border of septal cartilage between 6 -10 yrs.so no septal resection or revision before 20 yr
Vomer is essential for general nasal growth and downward and forward growth of maxilla till 7 to 8 yrs
Rational approach is rotation advancement lip repair with primary closure of cleft nostril floor and repositioning of alar base .
Onlay bone grafts or surgical augmentation of hypoplastic piriform aperture to elevate the cleft alar platform should represent the extent of primary procedure
Secondary correction of residual nasal deformity by limited septoplasty, reconstruction of nasal tip and alar cartilages and cartilage grafts is also appropriate
3 Puberty / adolescenceBy 16 to 18 yr canine teeth have erupted and
bone grafting has been performed providing bony support for nasal base with augmentation of hypoplastic maxilla
Osteotomies of maxilla and correction of skeletal or occlusal abnormalities should precede def rhinoplasty as advancement of maxilla may alter nasal contour
Salyer principles of CLN deformity1. The more severe the deformity , the earlier and more radical
the secondary procedure should be 2. Correction of nasal deformity is designed to improve form
and function and to alleviate psychological stress3. Correction of nasal deformities include Skeletal base Septum Alae 4 Bone grafting and cartilage augmentation may be indicated5 Definitive rhinoplasty at 14 yr or more6 Severe asymmetry of skeletal base is a contraindication to
definitive rhinoplasty [email protected]
Corrective surgery techniques To restore nasal symmetry , the alar cartilage must be
modified by repositioning , suspension, alteration in size or augmentation with graft
Techniques1. External approach 2. Alar cartilage mobilization and suspension 3. Alar cartilage incision and repositioning4. Graft augmentation5. Orthognathic procedures6. Bone grafting7. Vestibular web revisions8. Nostril hood modification
Rotation of cleft lip lobule and external incisions Blair excision and rotation advancement –
superior and medial rotation of alar base to correct abnormal orientation of nares and advanced the downwardly displaced medial crura by a midcolumellar incision that extended under the alar base . Wedge excision for caudal dislocation of alar margin
External scarring so abandoned Various modification described
Joseph – semilunar excision of dorsal skin to correct the downward displacement of ala
brought the dome of alar cartilage into a more normal position
Crickelair – justified external incisions in marked abnormalities . Medial advancement of alar base is done in all these procedure
Gilles and killner Extended Blair procedure by lengthening
the midcolumellar incisions upward over the cleft side of dome
Willie correction– Joseph dorsal incision was part of rotation advancement of alar columella
A separate rim incision corrected downward displacement of ala
Berkeley Extensive rotation upward and medially of
entire half of nose on cleft side Extensive mobilization of lobular complex abandoned
Hugo and Tumbusch Instead of rotation the nostril floor into
columella , they incorporated lip skin and scar to lengthen the columella on cleft side
Dibbel – excision of excess alar rim skin ,mobilization of alar cartilage from the skin and rotation of nostril peripherally rather than rotation of half of columella
Disadvantage is fresh lip scar
Black Deglove the lower nasal skeleton and enhance
exposure through rim and upper labial sulcus incisions
Tajima “C” flap extension for simultaneous exposure and skin tailoring
Advantages of external incision1. Wide exposure2. Increased alar mobilisation 3. Stability4. Superior correction of severe deformitiesDisadvantage1. Scar on nasal tip indications1. Severe deformities 2. Thick alar skin3. Previous unsuccessful intranasal procedures
External approach for nasal tip 1. Erich flying wing incision2. Figi combination of flying wing and midcolumellar
incision 3. Gilles extended the columella incision into the cleft
floor 4. Potter – only acceptable ext approach Incision at columella base and dev of columella flapLess scar and excellent access to alar cartilage
Alar cartilage mobilisation and suspension Potter – Complete exposure of deformed alar cartilage,
delivery of lateral crus and suturing of domes. Columellar flaps raised exposing both lateral crura
Mcindoe exposing both ala and securing alar domes to each
other and to septal angle . The lateral cartilage and crura secured to septum and skin .raw defect closed with composite graft of cartilage and skin or left to epithelize
Spira excised noncleft crus to fill lateral vestibular defect on cleft side . A suture from alar base passing thought the nasal spine and caudal septum provide maintenance of nostril sill width
Stenstrom – z plasty to narrow alar base with a buried suture anchoring the alar base to septum
Rees dissected the entire lateral crus on the cleft side from
the nasal skin and mucosa and weakened the cartilage by scoring to establish a contour similar to noncleft dome.
suture to contralateral upper lateral cartilage and medial crura maintained the corrected height of dome
Lateral vestibular defect closed with comp graft [email protected]
Reynolds and horton Suspension of cleft alar lateral crus to both
ipsilateral and contralateral upper lateral cartilage. Elevation and suspending the cleft of alar cartilage are facilitated by excision of a portion of alar cartilage
Tajima and maruyama reverse U incision with suture suspension of
repositioned alar cartilages . On nostril on cleft side a reverse U incision begins in membranous septum curving forward slightly over nostril rim parallel to dome of them cartilage and reentering the nose to end just lateral to fold in nasal vestibule
chondromuccutaneous flap of alar cartilage is raised and widely undermined .
Additional undermining over contralateral alar cartilage & upper lateral cartilage frees entire nasal skin for redraping .
The deformed alar cartilage flap is properly positioned and sutured to contralateral alar cartilage of noncleft side and lateral cartilages of both sides by rotationg the reverse u flap medially and superiorly
Nakajima added a z plasty in lateral nasal vestibule Straith – correction of alar columella web by modified Z
plasty technique
Incision and relocation of alar cartilage Humby – incision and transposition of the upper portion of
unaffected lateral crus across midline to augment lateral crus of cleft side
Kazanjian elevation of medial crura of both alar cartilage as medially based flaps ; sutured together vertically after division from the lateral crus . Excision of alar base weges and semilunar excision of skin from alar web area also modified nostril width and projection
Brown and McDowell divided cleft lateral crus and repositioned it across the midline over its own medial crus and dome ;suspended to contralateral dome through an intranasal incision
Incision and relocation of alar cartilage Barsky relocated and suspended the cephalic border of lateral
crus of cleft side to dorsum of the septum divided the medial crus on the cleft side through an external incision and suspended the dome area to contralateral dome
Whitlow-constable – figi type external incision and crossed bilateral alar winged flaps suspended through the skin by pull out bolster sutures
These hinged flaps depend on cartilage integrity to maintain the elevation and position of remaining alar cartilage. Prerequisites are strong well developed cartilaginous component that can withstand stresses necessary to move attached soft tissue
Graft augmentation Lamont cephalic margin of uninvolved ala to augment the
cleft alar dome Fomon ear cartilage graft over lateral crus in columella and
ant nasal spine Musgrave and dupertuis multitiered cartilage graft Millard columella strut graft Gorney & falces gull wing conchal graft formed by suturing
conchal grafts together with their convexities apposing one another
Dibbell shaped costal cartilage into a bowie knife strut for placement in a pocket created in columella and membranous septum
Tessier Minerva helmet or lily conchal cartilage Chait – C shaped cartilage inserted through an
incision in columella rim extends into floor of nose . Support and augments nasal sill. Placed sup to alar cartilage & secured to medial crura of both alar cartilage
Thomson – incision of alar to produce medially based flap resulting in lengthening of columella . Nasal tip and perialar sulcus are augmented with a conchal cartilage graft
Lengthening of columella Always shortened columella At 6 yrs Early repair results in downward slippage of columella
and lip over the premaxilla . Advancing skin from the floor of nose and base of ala
into columella Converse used skin from floor of nose Millard forked flaps from the prolabium Brauer and Foerester V Y principle in wide tip
Bipedicle flaps are formed based medially on columella and laterally on alae
The medial incision that separates the columella form the septum is continued laterally and posteriorly across the floor of the nose to make flaps progressively wider.
If alae are excess remove half thickness wedge and remaining half is advanced medially
The flaps are sutured in midline [email protected]
Adjacent tissue of cheek are freed form the maxilla Columella septal incision is sutured with the
columella in a more forward position Cronin technique Z plasty Ear cartilage elliptical piece sutured convex to
convex surface ends are left in spreading position posteriorly against spinous process
The ant ends sutured to medial [email protected]