Unilateral cleft lip : primary deformities




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UNILATERAL CLEFT LIP : PRIMARY DEFORMITIES

  • Cleft lip is best referred to as cleft lip, nose and alveolar deformity as its all these structures of the primary palate that are affected to some degree in all but the most minor defects( even in form fruste clefts one can appreciate subtle differences in the nostril on the affected side)

ANATOMY




CLEFT (LATERAL) SIDE

premaxilla

  • The premaxilla is outwardly rotated and projecting, and the lateral maxillary element is retropositioned


Septum

  • The inferior edge of the septum is dislocated out of the vomer groove and presents with the nasal spine in the floor of the normal nostril


Columella

  • There is unilateral shortness in the vertical height of the columella, varying from three fourths to even one-half that of the normal side


Lower lateral cartilage

  • The lower lateral cartilage is attenuated, its medial crus lower in the columella and its dome separated from the opposite alar cartilage to rest below it. The lateral segment is flattened and spread across the cleft at an obtuse angle

  • The alar crease has no alar cartilage bulge to give way to and consequently continues obliquely across the tip just lateral to the joint of the columellar and through the rim of the ala. This is often responsible for an actual kink in the alar margin itself.

Alar base

  • The alar base is invariably rotated outwardly in a flare

Alar rim

  • The alar rim is invariably distorted by a skin curtain (without cartilage) that droops over the alar rim like a web and further reduces the apparent height of the columella

Vestibular lining

Orbicularis oris

  • The orbicularis muscle in the lateral lip element ends upward at the margin of the cleft to insert into the alar wing

Philtrum

  • The philtrum is short


NON-CLEFT (MEDIAL) SIDE
Philtrum

  • Shortened philtral height


Columella

  • Shortened columella

  • Preserved 2/3 of the Cupid's bow, one philtral column, and a dimple hollow

  • Hypoplastic musculature between the philtral midline and the cleft


CLEFT NOSE

  • Park showed that the lateral crus of the lower lateral cartilage on the cleft side is not hypoplastic.

  • During primary rhinoplasty of 55 unilateral cleft lip nose patients, the authors measured the lateral crus of the lower lateral cartilage on both sides.

  • On the cleft side, the midportion of the lateral crus appeared to be thicker and wider in comparison with the noncleft side.

  • No histologic differences were found between the lateral crura of the normal and the cleft side. They concluded that the cleft lip nasal deformity is caused by external factors rather than intrinsic factors.




  • the columella base was deviated to the noncleft side

  • the cleft side alar base was more posterior than the noncleft side alar base

  • the noncleft side alar base was farther from the midline than the cleft side alar base

  • the cleft side piriform margin was more posterior than the noncleft side piriform margin


Summary

  • unilateral cleft deformity is characterized by a wide nostril base and separated lip segments on the cleft side.

  • The affected lower lateral nasal cartilage is displaced laterally and inferiorly, which results in a depressed dome, the appearance of an increased alar rim, an oblique columella, and an overhanging nostril apex.

  • If there is a cleft of the palate, the nasal septum deviates to the noncleft side with an associated shift of the nasal base

  • The alar base width is significantly increased, and the lip segments are widely separated

  • The flattened nasal tip is tethered directly to the prolabium by a severely deficient or absent columella.

  • The lower lateral alar cartilages are flared and concave where they should be convex.

  • The greatest challenge for esthetic reconstruction is the absent or deficient columella.








Important notes on the orbiicularis oris
2 well defined parts to the orbicularis oris

Deep and superficial parts - superficial component of the orbicularis muscle serves as a retractor, while the deep component serves as a constrictor of the lip.
Deep (intrinsic)

  • Functions in catching food with a sphincteric action

  • Consists of pars marginalis (forms the tubercle) and pars peripheralis

  • Deep orbicularis oris originates from the moidiolus on each side, it is horizontal with continuous fibres passing from one commissure to the other across the midline,

  • It lies close to the inner mucosal surface

  • At the commissure, the curled border of the upper lip muscle divides, and the curled border of the lower lip muscle inserts between these slips

  • When the fibres shorten to close the lips, the margins flatten out and the interdigitation provides a scissor-like motion which seals the angles of the mouth


Superficial (extrinsics)

  • Functions in facial expression

  • Upper (nasal bundles) and lower bundles (nasolabial bundle)

  • Lower bundle

    • decussate in the midline and insert into the skin lateral to the opposite philtral groove forming the philtral columns.

    • philtral dimple centrally is depressed as there are no muscle fibers that directly insert into the dermis in the midline

  • Upper bundle

    • common insertion of zygomaticus major and minor, levator labii superioris, levator labii superioris aaeque nasi, and nasalis (alar)

    • inserts into the anterior nasal spine, the septo-premaxillary ligament, and the nostril sill, passing deep to the alar base

Anatomical changes with the cleft lip

  • Intrinsic(deep) bundle not displaced but was interrupted by the cleft. The extrinsic bundle in the lateral side of both cleft types ran upward along the lateral cleft margin, whereas in the medial side it ran horizontally to terminate close to the medial cleft margin.

  • The naso labial bundle changes its direction and runs almost vertically becoming attached to the nostril and the periosteum of the nostril aperture. Contraction thus results in a marked lateral bulge

  • Cleft side nasal bundle pulls lateral alar laterally and down

  • Noncleft side nasal bundle inserting on to anterior nasal spine pulls septum towards noncleft nostril

  • In cases of incomplete clefting the muscle does not as a rule, cross the cleft unless the bridge is at least 1/3 the height of the lip




Fig 1: Normal anatomy; A=extrinsic, B=intrinsic

Fig 2: Microform cleft, no nasal deformity

Fig 4: Incomplete cleft lip

Fig 8: Complete cleft lip

Dado and Kernahan found no distinct muscle bundles parallel to the cleft margin

The muscle bulge in complete and incomplete cleft lips consisted of a haphazard arrangement of muscle fibres running transversely, obliquely, and anteroposteriorly
Simonarts band


  • tissue on the nostril floor in an incomplete cleft lip

  • Defined as soft tissue band <5mm wide and complete alveolar cleft

  • Millard states that “even the most minor Simonart’s band acting as a restrainer in utero greatly reduces the extent of maxillary and nasal distortion.”



Blood supply of cleft lip

  • Slaughter and coworkers detail the blood supply in the clefts

  • clefting interupts the normal anastomoses between the superior labial artery, anterior ethmoidal artery, posterior septal artery and greater palatine artery

  • In complete bilateral clefts the prolabium is based on the posterior septal artery

"White roll" of Gillies



  • Anterior projection of the pars marginalis of the orbicularis oris muscle

  • skin mucosal junction formed by ( on sagittal section beginning anteriorly and progressing post at the white roll), the vermillion mucosa exhibits increasing epidermal thickness and size of the rete ridges, decreasing melanin content, more superficial capillaries and an abrupt transition from a keratinized to non keratinized epithelium

  • "Red line" of Noordhoff abrupt transition from keratinized to nonkeratinized squamous epithelium

  • In the cleft lip

    • the white roll is absent

    • hypoplasia and disorientation of the underlying pars marginalis component of the orbicularis

    • Decreased vermillion width on the medial side of the cleft and normal to increased width of vermillion laterally

    • the entire prolabial vermilion component of bilateral cleft lip specimens is hypoplastic.

    • As a result, Noordhoff recommends use of a lateral vermilion flap to augment the deficient medial vermilion in cleft lip repair.


CLASSIFICATION







MANAGEMENT




Considerations


  1. Airways

  2. Feeding

  3. Facial

  4. Growth

  5. Speech

  6. Hearing

  7. Other anomalies

  8. Genetics

  9. Psychological



FEEDING

  • More cleft children tend to be underweight compared to normal children

  • Cleft palate children are more underweight than cleft lip children

  • Underweight cleft children had more fistulas post-op

  • Squeezable bottles shown to have a recommended because they are easier to use and have a beneficial impact on the babies’ growth.

  • At PMH, for 3 weeks post op – spoon fed only.



TIMING





  • Varies considerably between different units


PMH protocol

    • Lip repair at 3/12

    • Palate repair and grommets at 9/12





      • International trends

        1. early repair (<3 months) 33%

        2. conventional (3-6months) 65%

        3. late (>6 months) 0.7%

      • some still adhere to the rule of 10s: perform surgical repair of cleft lip when the child has a hemoglobin of 10 g, weight of 10 lb, and is aged 10 weeks.

- lip repair 3/12 vs earlier repair



    1. child is better able to withstand the stress of surgery and anaesthesia

    2. lip elements are larger and allow for a meticulous reconstruction.

    3. Allow maternal bonding

    4. Earlier repair gives better healing and better scar

- lip repair 3/12 vs late repair?



  1. Effect on the maxilla – growth is more favorable with early repair

  2. Scar – scar is more favorable with early repair

Mx of obstructed Piere Robin



  1. Monitored unit

  2. Position prone

  3. nasopharyngeal airway

  4. Lip and tongue adhesion

  5. Intubations

  6. Tracheotomy (difficult to decanulate down the track)

  7. some advocate early DOG to avoid tracheostomy or to allow for earlier removal



INTRAUTERINE REPAIR


see fetal healing


  • animal studies

  • incisional and excisional studies by Hendrick and associates

  • incisional clefts healed with regeneration had no scar formation

  • excisional defects healed without scar however without regeneration of the skin appendages

  • attendant risk of fetal death and as such is reserved for life threatening conditions where postnatal intervention can not help significantly

  • Stelnicki and coworkers analyzed the longterm functional and esthetic outcome of in-utero versus neonatal cleft lip repair in lambs. There was no evidence of maxillary growth impairment in the lambs repaired in utero. In addition, the clefts repaired in utero were scarless. Both the in-utero and neonatally repaired lambs had lips that were considerably shorter vertically on the repaired side than on the normal side. This was thought to be a function of the straight-line closure, and points to the need for similar comparisons in this ovine model using a Millard-type rotation advancement technique.



PRESURGICAL ORTHROPAEDICS





  • principle objective of presurgical nasoalveolar molding (NAM) is to reduce the severity of the initial cleft deformity. This enables the surgeon and the patient to enjoy the benefits associated with repair of cleft deformity that is of minimal severity.

  • Concept of the major and minor segments



The alveolar (maxillary) segments assume one of four positions

1. Narrow-no collapse

2. narrow-collapse

3. Wide-no collapse

4. wide-collapse



  • Wide is determined by an alveolus position lateral to the desired alar base position (ie with lip closure the alar base is sitting in the cleft)

  • clefts characterised as "narrow-no collapse" are prime candidates for lip repair with simultaneous correction of the nasal deformity. A static moulding appliance is useful to maintain the ideal arch relationships

  • Clefts characterized as "narrow-collapse" are ideal candidates for presurgical palatal orthopaedic expansion beginning at approx. 2 weeks of age and continuing to surgery, at which time definitive cheiloplasty is undertaken

  • clefts characterised as "wide-collapse" benefit from a combined approach: presurgical appliance that expands the collapse with external moulding through tapes to reduce the width of the interalveolar space

  • clefts characterised as "wide-no collapse" benefit from molding appliances that maintain width but allow external forces to guide the alveolar segments together




  • The primary benefit of a balanced non-collapsed arch configuration at the time of primary lip repair rests in the provision of a stable skeletal base on which the cleft nasal alar segment is positioned

  • In the infant with bilateral clefts of the lip alveolus and palate, the objective of presurgical NAM includes the nonsurgical elongation of the columella, centering of the premaxilla along the midsagittal plane, and retraction of the premaxilla in a slow and gentle process to achieve continuity with the posterior alveolar cleft segments.


Appliances

  • Most appliances are designed to correct the alveolar cleft only, despite the fact that the cleft nasal deformity remains the greatest esthetic challenge.

  • Components

    • Alveolar plate

    • Nasal stent

    • External tapes

  • Latham appliance intraoral active pin-based appliance to simultaneously retract the premaxilla and expand the posterior segments over a period of days. Screw is turned 3/4 of a turn everyday until tight. The screw pushes on the back bracket to rotate the two side brackets upward and together.



  • In response to controversy associated with active retraction of the premaxilla, Hotz described the use of a passive orthopedic plate to slowly align the cleft segments. The premaxilla is not retracted, and by age 10, Hotz feels that the face has grown forward into appropriate

  • Matsuo’s research into cartilage moulding led to development of nasal splints – the infant cartilage was still under the influence of maternal oestrogens - this correlates with the increased hyaluronic acid, which inhibits the linking of the cartilage intercellular matrix.

  • Grayson appliance(1993) - correct the alveolus, lip, and nose in infants. Because the stent is extended from a molding plate, an intact nasal floor is not required.



Benefits

  • In the long term, studies indicate that the change in nasal shape is stable with less scar tissue and better lip and nasal form.

  • This improvement reduces the number of surgical revisions for excessive scar tissue, oronasal fistulas, and nasal and labial deformities.

  • With the alveolar segments in a better position and increased bony bridges across the cleft, the adult teeth have a better chance of erupting in a good position with adequate periodontal support




Complications

  1. mucosal ulceration

  2. skin irritation

  3. risk that the molding plate will become dislodged and obstruct the airway

International trends



  • 57% of centers routinely used presurgical orthopaedics (like at PMH)

  • 60% use NAM, 24% use Latham appliances


LIP ADHESION

  • The adhesion improves maxillary arch alignment and enables a more predictable correction of the cleft deformity

  • Reduces tension in the definitive closure and facilitates gentle molding

  • Initially advocated by Johanson and Ohlsson 1954

  • Randall interdigitates short broad triangular flaps

  • Millard high adhesion to avoid scarring in the area of the repair

  • Furnas used the straight-line repair

  • May be used instead of presurgical orthopaedics or in the event of failure.


Indications

    1. Presence of a wide cleft

    2. Poor compliance with appliances

    3. presence of a bilateral cleft

    4. a bilateral cleft with a prominent premaxilla

Opponents argue the scar introduced interferes with the results of the definitive repair
Timing (for centers that do this)

  • Interlip gaps >1cm should undergo preoperative taping

  • At 6 weeks to 3months, if gaps are still deemed wide, a lip adhesion is performed


Method

  • Mark out the routine cleft lip repair landmarks to ensure that these are not disturbed by the lip adhesion

Trends

    • 43% of centers use lip adhesion but only infrequently (<10% of patients)





DEFINITIVE LIP SURGERY


      • Warm theatre, patient warming

      • pediatric anaethetists

      • Supine, neck extended over end of pillow

        • Rose position - intended to prevent aspiration or swallowing of blood, as from an injured lip: the patient is supine with the head hanging over the end of the table in full extension so as to enable bleeding to be over the margins of the inverted upper incisors


Microform clefts




UNILATERAL CLEFT LIP REPAIR








Principles

  1. Lengthening of the shortened vertical height of the cleft side on the medial segment to match the non cleft side

  2. Bringing a flap of tissue from the lateral lip where it is abundant into the medial lip where it is missing

  3. Retaining the normal anatomic Cupid's bow

  4. The rotation-advancement flap

  5. Muscle reconstruction

  6. Restoration of the bony platform

  7. Reconstruction of the distorted nasal anatomy




  • a repaired unilateral cleft lip retains the configuration and length given at the initial repair.


TENNISON-RANDALL TRIANGULAR FLAP REPAIR

Advantages

  1. Better for wide clefts and clefts with a relatively short lateral segment

  2. Less contracture than the Millard

  3. More geometrical

Disadvantages

  1. Scar crosses the lower aspect of the philtral column

  2. long lip


Method


  • Mark with a pen and ink before tumescent

  • Tattoo with ink and needle

  • Measure the vertical height of the on the normal side with calipers

  • Point X point on the non-cleft side midway between the alar base and the base of the columella

  • Mark the alar base and the columella

  • Mark the commissure

  • Point Y peak of cupids bow on the non-cleft side(X-Y = vertical height of the normal side)

  • Point Z is the trough of the philtrum

  • Point 1 is the same distance from point Z as distance Z-Y, marked on the vermillion

  • Point 2 is placed in the skin 1mm above point 1 but very close to the vermillion

  • Point 3 is placed 1mm from points 1 and 2 to form an equilateral triangle

  • Points 4 and 5 are located on the cleft side. 4 and 5 are located the same distance from point X to the alar base and columella (in reality preserve as much nostril floor as possible – Mr A Baker) - These points belong in the lip and not in the nasal vestibule. Placing a skin hook in the alar and lifting up helps to better define these points

  • Distance 4-2 is the major vertical distance (usually 7)

  • The repaired side is made 1 mm shorter then the non-cleft side because of a tendency for the cleft side to become too long with growth

  • The minor vertical distance for the side to be repaired is X-Y minus 1mm minus the major vertical height (usually 3)

  • Point 6 is located on the cleft side where

  1. white roll begins to disappear

  2. the vermillion starts to thin

  • point 7 is located using two calipers-one set for the major vertical distance and based from point 5 and the other is set for the minor vertical distance and based on point 6. The intersection needs to keep inside the vertical imaginary line from the alar base

  • Point 8 is the point of the triangular base and should be placed in the skin but close to the vermin

  • Distance 7-8 must equal length 2-9

  • traingular flap should be a equilateral triangle

  • Point 9 is placed medial to point 2 on a line through point 3 and should not encroach on the other philtril column

  • Angle 4-2 and 2-9 approaches 90 degrees

  • Point 10 is 1mm above point 6 so that the distance 10-8 equals the distance 3-9

  • 1mm offset above the vermilion is done so the oblique scar does not give the impression of the vermillion ridge extends to the skin of the lip

  • Local with adrenaline

  • Lateral lip element is incised first

  • Dissection around the alar base and columella

  • Need to release the muscle and skin of the laterally located alar base

  • Incisions across the vermillion are kept perpendicular


MILLARD REPAIR

Advantages

  1. cut as you go

  2. scar is located along the philtral column

  3. secondary revision is easier with reelevation of flaps

Disadvantages

  1. scar across philtral at the upper end (nasal sill)

  2. higher learning curve

  3. necessity for wide soft-tissue undermining

  4. tendency for a shortened upper lip causing notching

  5. tension across the nostril sill and tendency toward a constricted nostril on the side of repair.

  6. suggestion that it causes more hypertrophic scarring than the triangular flap repair




  • Back-cut may be made on the rotation flap but should not cross the midline

  • Technique allows the scar to follow the projected line of the philtral column except in its uppermost portion, where it arcs beneath the columella.

  • Millard emphasizes the importance of presurgical orthopedics, lip adhesion and nasal correction as part of the rotation-advancement procedure, the use of an L-flap to increase vestibular lining, and proper use of a C-flap to increase columellar length on the cleft side.


Adding a Z plasty to the flaps may be required if a length discrepancy exists despite maximum advancement-rotation.




SURGICAL COMPLICATIONS





    1. Haemorrhage

    2. Infection

    3. Dehiscence

    4. Respiratory compromise

    5. Interference with facial growth – maxillary growth shown to be hindered by cleft lip repair


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