Cleft lip is a birth defect in which the upper lip is split vertically, extending into one or both nostrils.
- Cleft lip results from failure of merging of epithelial groove between the medial and lateral nasal process by penetration
of mesodermal cells.
- Most cleft cases are polygenic but the 5% of cleft cases associated with syndromes are said to be monogenic.
- Environmental factors like nutritional deficiencies, stress, infections, alcohol, drugs, toxins and ischemia may cause clefts.
Syndromes associated with cleft lip
- Beckwith–Wiedemann syndrome
- DiGeorge syndrome
- Fetal alcohol syndrome
- Goldenhar syndrome
- Gorlin syndrome
- Treacher Collins syndrome
- Van der Woude syndrome
- Waardenburg syndrome
Classification of Cleft lip
Veau system of classification is generally used where the emphasis is on the extent to which the clefting is seen. Submucosal clefts are not included here.
- Class I: Unilateral notching of vermillion border that does not extend into the lip.
- Class II: Unilateral notching of the vermillion extending into the lip but not involving the floor of the nose.
- Class III: Unilateral clefts of the vermillion border extending through the lip into the floor of the nose.
- Class IV: Any bilateral cleft of the lip exhibiting incomplete notching or a complete cleft.
Management of Cleft Lip
Timing of the Lip Surgery
The timing of the lip repair in infancy varies from first 48 hours of life to 6 months of age, depending on the surgeon’s judgement. Although, there is always a debate as to when the surgery should be performed, most surgeons follow ‘Millard’s rule of 10.’ 10 weeks of age with 10 gm of Hb and 10 pounds of weight. The trend is shifting toward early repair. In fact few centers like to repair the lip as early as 5-7 days post-birth.
Total paediatric and ENT check-up should be given to the child, though in a large number of cases this can be done by the operating surgeon himself. Wherever the need be, help may be sought of other specialists. One should particularly look for the following:
- Upper respiratory tract infection
- Ear infection
- Other congenital anomalies particularly cardiac.
- Mental status of the child
Parental counselling is extremely important and its role should never be underestimated.
Often the complaint made by parents is about the poor nutritional status of the child due to cleft palate deformity. This is a total fallacy. Though the child is not able to suck like a normal child and tends to take in more air with every sip of the milk, a proper training to the mother can easily overcome this handicap. Spoon feeding is preferred. It is more hygienic. Breast milk can be expressed and fed with a spoon. If desired, the child can be fed with a bottle with teat with a larger hole. In addition the child should be burped frequently to take the air out, which it ingests with the milk (due to large velopharyngeal port) and feels full without taking in adequate quantity of feed. Once the date of surgery is decided upon, the child should be weaned off the bottle/breastfeed and should be put on spoon feeding at least a week in advance to let him get adjusted to it.
Unilateral Cleft Lip Repair: Millard’s Rotation Advancement Principle
The operation is performed under general anaesthesia. For endotracheal intubation an oxford tube is preferred. It is then fixed with a stitch to the lower lip in its middle to prevent distortion. Head is placed on a rubber ring. Having draped the patient, the markings are done with pen and ink, usually a sterile toothpick is used dipped in ink (surgical grade). The important landmarks, which are usually present in unilateral cleft lip, are then tattooed with a no. 26 gauge needle.
The lip, ala and the adjoining cheeks are infiltrated with 1:100,000 saline-adrenaline solution, 1 c.c. or 5 to 10 micrograms per kg body weight can safely be injected. Five minutes wait after injection is desirable. For greater accuracy in incising and suturing and proper alignment of anatomical landmarks, use of magnification loops 1.5 to 2.5 is desirable. Skin incisions are then made with no. 15 blade. Incisions are completed through the muscle and the mucosa. Simply put, the difference in the height of two peaks of the cupid’s bow on the medial element is the exact distance that the higher peak must be lowered into normal position. This is also the exact distance that the interdigitation flap must measure across its widest point, so as to supply a mathematically sufficient amount of tissue to complete the release. The rotation incision provides flap ‘C’ which is cut free from the lip base attached to the columella. It is freed from the membranous septum to allow columellar advancement. This provides extra length to short columella and also reduces defect at backcut.
The difference in the vertical height of 2 and 3 = amount of release necessary from incision 3-5 + x = width of the point of flap 8-9 = 10, necessary to fill the rotation gap. To achieve requisite rotation, the incision may be carried well past the midbase of the columella. If rotation is still not sufficient, the incision can be carried further across toward the normal side. A “backcut” assures adequate closure without causing obliqueness of the scar or abnormal vertical lengthening of the lip and is a standard feature of all cleft lip repairs. Realignment of vertically attached muscle fibres is very important. Changing their direction from oblique to horizontal enables them to present their ends to the muscle of the lateral element. Wide undermining of the muscles on the non-cleft side should not be encouraged as such action will destroy the natural philtral dimple and philtral column. Freeing the muscle from its skin and mucosa along the edge, offers an advantage in the three-layer suturing.
Primary Nasal Correction
There is great controversy in regards to primary nasal correction at such an early age that it may interfere with the nasal growth. But the release of underdeveloped alar cartilage, correction of the wrongly directed ala and rotating it medially, upward and inward, bringing into its normal anatomical configuration does not interfere with the growth; in fact restoration of normal anatomy allows it to grow in more normal manner.
Introduction of flap `C’ into the short side of the columella adds length and contour bringing better symmetry to the central column. Lateral side of flap `C’ still serves as a portion of the nostril sill. The next step is to free the nasal and lip attachments to the maxilla on the cleft side. The extent of freeing depends on the width of the cleft. It must be quite radical in wide complete clefts. An incision along the upper labial sulcus on either side of the cleft with wide undermining and medial advancement of the mucosa depending upon the severity of the cleft, helps to bring the two lip elements (medial and lateral) together and suturing without tension with good buccal sulcus.
Flap ‘C’ is advanced into the columella on the cleft side and fixed with skin sutures of 6/0 ethilon. Then the vermilion pairings are sutured with 4/0 catgut or vicryl to line the sulcus by covering the raw area of the alveolus. Simultaneously the lip elements are advanced medially by suturing their upper lining edge to the maxillary mucosa along the labial sulcus on both sides.
Elbow splints are a must for 2 weeks postoperatively. The child is maintained on spoon feeds for 5 to 7 days and then can use a glass.
Post-operation, the lip is swollen from surgery and will look more natural within weeks. See photos in the section above.
Bilateral Cleft Lip
A complete and accurate assessment of the bilateral cleft lip is required before embarking on its surgical correction.
- Whether the cleft is complete or incomplete.
- The size and portion of the prolabium and the premaxilla.
- Presence of anomalies, i.e. lower lip pits, absence of the associated bifid nose, etc.
Principles and Objectives of the Surgical Correction
- Prolabium should be used to form the full vertical length of the middle of the lip.
- The vermilion ridge or white line of the inferior border of the prolabium should be preserved.
- The thin prolabial vermilion is turned down for lining.
- The thin central prolabial vermilion is immediately built up with the vermilion muscle flaps from the lateral lip segment.
- Vermilion ridge should come from the lateral lip segments.
- Upper buccal sulcus should be adequate and at no stage should the central portion of the lip look adherent and tethered to the alveolus.
- No lateral lip skin should be used below the prolabium.
- Lengthen the short columella.
- Premeditated adequate columella planning will avoid the need for later lip re-entry.
- The creation of continuity of the prolabium with the lateral lip elements joining mucosa for sulcus extension for muscles function. Scar is camouflaged within the philtrum column.
- Early and permanent alar base positioning.
- Correct disparity between premaxillary and maxillary segments of the alveolar arch.
- Repositioning of the severely protruding premaxilla must be done to avoid undue push on the lip.
- Prevention of collapse of maxillary processes behind the premaxilla.
- Bone grafting to stabilize the premaxilla.
Repair Both Sides Simultaneously
Simultaneous correction of the lip as well as the nose is done as it obviates the second anaesthesia and rehabilitation is faster. Repair in two stages should be reserved only in a small number of cases, where the general health of the child and anaesthesia risk overshadows the single stage repair.
- Freeing of the prolabium from premaxilla.
- Freeing of lateral lip elements from the maxilla.
- Forked flaps from the prolabium.
- Joining of mucosa and muscle to each other behind the prolabium.
- Lateral vermilion flaps to overlap prolabial vermilion.
- Banking of forked flaps in subalar incision. Join alar bases tip to tip. Correction of large defect is achieved by muscle to muscle union in the midline.
Technique of Bilateral Lip Repair
Almost all the methods of lip repair used in a unilateral cleft lip deformity can be used in bilateral lip repair as well. The simplest is essentially a straight-line closure and it can produce a satisfactory result. The most popular method for lateral lip repair is Veau’s technique, but the results achieved with Millard’s repair have been more pleasing.
While correcting a bilateral cleft lip with Millard’s rotation advancement, the only difference, but an important one, is that the flap ‘C’ is never allowed to cross the midline of the columella. This is to preserve the blood supply to columella. Point ‘A’ is located medial to lip of the alar base. Point `C’ is placed in the midline of the valley of the cupid’s bow on the vermilion ridge. Point `B’ is placed 3 mm lateral to point `C’ on vermilion ridge. If prolabium is wide, forked flaps can be developed and banked in nasal floor to be used later for columellar lengthening. Points A, B are marked on the lateral segments. After marking is completed, saline adrenaline infiltration is used and the operation is proceeded as described for the repair of the unilateral lip.
In the complete bilateral cleft, the premaxilla is unattached to either maxilla. Hence there are three components—two maxilla usually equal to each other in size and position and the third central premaxillary element protruding forward unhindered. The complete separation of the central component of prolabium and premaxilla influences the nose, philtrum, musculature, vascularity, nerve supply, growth and the development of all the three elements.
No matter which method of repair is used, certain basic principles remain the same : a. Observe symmetry of the lip. b. However small the prolabium, its vermilion must be retained. It should be used to form central part of the lip. c. Bring muscles into the prolabium. Release muscles in both the segments and try to suture these in the midline, if possible, if not at least bring these into the prolabium, so that the children with repaired bilateral lips can smile normally. d. Have adequate buccal sulcus. Prolabial lip or the central portion of the lip must be adherent to the alveolus. e. If for some reason, only one side of the lip is repaired at a time, the second side should be repaired 3 to 4 months later. In such a case one must try to match the previously repaired side.
In a unilateral cleft lip repair cleft-sided columella is lengthened with the use of the ‘C’ flap. In a bilateral cleft lip, vertical height of the columella always poses a problem unless the prolabium is quite ample. In such cases if Millard’s rotation advancement is used then the banked flaps in the nostril floors are used at second stage at the age of 2 to 3 years to lengthen the columella. If there are no banked flaps, then bilateral forked flaps as described by Millard is used, to lengthen the columella. This improves the lip scars of the previous operation as well.