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We have performed primary repair of bilateral cleft lip and nose on 169 patients in the past 15 years. During the first eight years, we used a small triangular flap skin design for the lip and for the nose correction, we used a corrective nasal cartilage lifting suture through rim incisions in order to bring the nasal dome cartilage toward the center and create the columella. The small triangular flap at the columella base was rotated 90° posteriorly to emphasize the contour of the nasolabial angle. In the subsequent 7-year period, the lip design was changed to the straight line method, and an inverted trapezoid suture was placed between the alar and nasal dorsum at four points. By this procedure displaced cartilages are moved into correct position and the alar groove became more distinct.
Long-term observations showed a favorable configuration of the nose, and eliminated the bilateral cleft nose stigma with only minimum degree of growth disturbance. The remaining problem is the somewhat superior faced nasal tip. To leave the bilateral cleft lip nasal deformity uncorrected for a long period places great psychosocial burden on the patient and the family. We believe that it is desirable to conduct early lip and nose repair synchronously in a minimally invasive manner, as a collaborative effort between plastic surgeons with specialized training in cleft lip repair and an interdisciplinary team.
We began conducting rhinoplasty simultaneously with primary bilateral cleft lip repair 15 years ago, and added some refinements seven years ago. There were minimum adverse effects on nasal growth in patients who underwent early stage lip and nose surgery. Following early lip surgery soon after birth (1–2 months of age), an inconspicuous white lip scar is obtained. To leave the bilateral cleft lip nasal deformity uncorrected surgically until puberty can be a great psychological burden on the patient and the family. Therefore, to avoid such problems, we prefer that nose correction be performed simultaneously with primary repair of bilateral cleft lip by well trained plastic surgeons in a well-organized multidisciplinary team.
1.1 Our original operative procedure
We have performed simultaneous repair of primary bilateral cleft lip and nose for 15 years. During the first eight years, the lip design involved a small triangular flap, which is raised at the prolabium of the columella base and rotated 90° in order to form a nasolabial angle as close to the natural angle as possible. For the nose, corrective suture of the nasal cartilage was performed through rim incisions and the widened nasal domes including alar cartilage are sutured together toward the center to form the columella
(Fig. 1) . The results achieved were favorable for the nostril and the nose. However, that shape of the Cupid's bow looked mountain-like, due to the use of the small triangular flap at the vermillion border and the alar groove became less apparent in many cases. We used these procedures for lip and nose repair in 67 cases. In this group, 39 patients had complete bilateral cleft lip. Since the first author changed his institutional affiliation, from Fujita Health University to Keio University long-term follow-up was not possible for many patients. We present two long-term follow-up cases (Figure 2, Figure 3) .
Figure 1Our initial operative procedure (first eight years). (A) Skin design is small triangular flap method. (B) A triangular flap at the columellar base is rotated 90° to create well defined columella-Labial angle. (C) Nasal dome cartilages are sutured together and lifted to opposite lateral cartilage through rim incisions. (D) Lip and nose repair is completed.
Figure 2(A) Patient with bilateral symmetric complete cleft lip and palate. Operation is performed 50 days after birth. (B) Seven years postoperative shape of Cupid's peak looks like a mountain (frontal view). (C) Profile, columella base is shifted caudally. At the age of 12 years, he underwent red lip revision and base of columella is moved cranial position using VY plasty. (D) 15 years after primary lip and nose operation (frontal view). (E) Profile.
Figure 2(A) Patient with bilateral symmetric complete cleft lip and palate. Operation is performed 50 days after birth. (B) Seven years postoperative shape of Cupid's peak looks like a mountain (frontal view). (C) Profile, columella base is shifted caudally. At the age of 12 years, he underwent red lip revision and base of columella is moved cranial position using VY plasty. (D) 15 years after primary lip and nose operation (frontal view). (E) Profile.
Figure 3(A) Patient with complete bilateral symmetric cleft lip and palate. Primary lip and nose repair is performed by triangular flap method at 1 month. At the age of eight, he underwent lip revision without nose correction. (B) 13 years after the primary operation. (C) Profile.
Immediately after birth a sleeved long nose retainer of our design is inserted into the nostril and sutured to a roll of silicon gauze at the back of the nose for seven days for preoperative nasal molding.
For reasons mentioned previously, during the latter seven years the lip design evolved toward the use of the straight line method, and for the nose correction, the inverted trapezoid suture was added together with suturing of displaced alar cartilage.
Operation is performed at 1–2 month of age. Our refined design for the treatment of symmetric bilateral cleft lip is depicted in Figure 5(A and B). This procedure is designed using the same principle for unilateral cleft lips which has been reported earlier.
Figure 5(A) Design for repair of bilateral symmetric complete cleft lip. (B) Distance resulting suture lines are 4 mm. (C) Red lip of prolabium is used to cover the oral vestibule (flap A). Cleft margin of lateral red lip are sutured zig-zag fashion to form the oral vestibule (flap B). Muscle suture is limited upper half of the white lip. (D) Tension reducing suture of the white lip.
Operation is usually performed in one stage, leaving two straight suture lines parallel to each other. The distance between the two is 4 mm.
Flaps A and B are used to reconstruct the gingival sulcus (Fig. 5(C)). Flap A is elevated from the bilateral mucosa of the prolabium and turned medially, thus forming the gingival side of the oral vestibule.
Flaps B are raised from the cleft margins of the bilateral mucosal lips, both are turned inward like hinges, and sutured to the corresponding portions of each other in a zig-zag line, forming the posterior aspect of the upper lip. We do not use intra-oral incisions along the lateral bucco-gingival sulous to avoid growth retardation of the maxilla.
Nostril floors are formed by advancing flaps C, which are raised from bilateral alar bases, medially toward the columella. Back-cuts are made at the bases of flaps C make advancement easier.
The top edges of flaps B and C are sutured to each other, to form the lining of the nostril floor.
At the prolabium of the left and right columella base, a triangular flap D (about 7 mm×10 mm) is created and rotated 90° posteriorly to achieve columella elongation and a columella labial angle that is close to the natural angle (Fig. 1(A)). The alar base is undermined to a point beyond the nasolabial fold, and 2-stitches of 4–0 white nylon are used as relaxation buried sutures to make the inter-alar base distance narrower and to reduce tension on the wound (Fig. 5(D)). Reconstruction of the muscular sling of orbicularis oris is performed by suturing muscles to their contralateral counterparts. In this process, the lower half of the muscle is left unsutured to prevent whistling deformity (Fig. 5(C)). The prolabial white skin roll was initially reconstructed with bilateral mucocutaneous flaps, but because this often resulted in a noticeable horizontal scar we now use the prolabial white skin roll as is, and only the red lip tubercle is created by a bilateral mucosal flaps sutured in a everted manner at the midline.
For the nose, bilateral rim incisions are made, and minimal subcutaneous undermining is performed (Fig. 6(A)) . After dissection and elevation of the soft tissue between the alar cartilages, an inverted trapezoid suture is placed at four points to pull the alar cartilage and widened nasal tip toward the center to form the columella and provide a pinched look of the nasal dorsum
(Fig. 6(B)). After approximating alar cartilages, the soft tissue present between the cartilages is placed above the united nostril cartilages. To ensure correction of the displaced cartilages, a lift-up sutures is placed between the alar cartilages and opposite lateral cartilages. For wound closure of the white lip, only a subcutaneous suture is used. The skin surface is covered by clear polyurethane tape (Perme-aid® Nitto Medical, Japan) (Fig. 6(C and D)). To maintain the correct shape of the nose, fixation suture are placed between the sleeved long nose retainer (Koken®, Japan) and rolled silicon gauze pads (Tampon prosthesis® Koken, Japan) on the dorsum of the nose. This retainer is twice as long as the conventional one, and the posterior edge reaches beyond the piriform margin, allowing for a stable fixation (Fig. 7(E)) . This not only improves the configuration of the columella and the nostril, but also allows the correction of the anterior portion of a deviated septal cartilage. There is a sleeve of about 5 mm located at the tip which prevents pressure sore formation at the nostril edge, even with a lift-up fixation of the nose. Long retainer fixation without a sleeve is continued for about one month, postoperatively.
Figure 6Nose correction. (A) Inverted trapezoid suture plus cartilage lifting. (B) Inverted trapezoid suture is placed at four point.
Figure 7(A) After the lip closure. (B) After the nose correction. (C) Wound surface is covered by clear polyurethane tape. (D) This sleeved long retainer is inserted into the nostril and fixed to the dorsum of the nose. It prevents pressure sores after this fixation. (E) Sleeved long retainer.
In the operation of the asymmetric bilateral cleft lip, differences in tissue volume between the incomplete cleft side and complete cleft side should be taken into consideration.
Namely, the former has ampler tissue than the latter. Therefore, to achieve symmetry, different operative design should be adopted on each side (Fig. 8) . On the complete cleft side, as the Cupid's peak is located superior to its counterpart on the incomplete cleft side and should be lowered using a small triangular flap at the vermilion border. On the incomplete cleft side, this flap is not necessary and a straight line method is used to raise the Cupid's peak. To correct the asymmetry of the columella base, triangular flaps C and D are raised only on the complete cleft side, for advancing alar base and elongating the columella unilaterally. On muscular reconstruction, the difference in tension on each side often shifts the prolabium to the complete cleft side. Therefore, the incision of the prolabium is initially performed along the vermillion border in the early stage of the operation and the final parallel incision design is decided on only after the completion of muscle repair. The positions of the incision lines are also selected to accommodate the small triangular flap on the complete side. Concerning the correction of the nose, more lifting of alar cartilage is required on the complete cleft side.
Figure 8Design for repair of bilateral asymmetric complete cleft lip.
In the latter seven years, 92 cases were operated using the improved procedures, 54 cases had complete bilateral cleft lip and among them 17 cases were followed by the first author for five years or longer. We plan to report separately a statistical analysis with respect to the scar of white lip, red lip tubercle, nostril shape, inter-alar base distance, and nasolabial angle. The four cases presented here all underwent primary lip and nose surgery only, with no subsequent revision (Figure 9, Figure 10, Figure 11, Figure 12) .
Figure 9(A) Patient with bilateral complete symmetric cleft lip and palate. (B) Seven years postoperative (frontal view). (C) Worm's eye view. (D) Profile.
Our refined approach can achieve a fine white lip scar and better Cupid's bow contour compared to the small triangular flap method. However, it was difficult to create a philtrum dimple with a clear three-dimensional structure. The coloration of the red lip tubercle was favorable and matched the lateral red lip, but in those patients where the lateral mucocutaneous flap was used to form the white skin roll of the prolabium, the horizontal scar above the vermilion was sometimes visible. The inter alar base distance narrowed to some extent, most likely due to the use of subcutaneous relaxation suture. Favorable nasal and nostril configuration was achieved, with good formation of the columella and eliminated the stigmata of bilateral cleft lip and nose. However, possibly because the tissue in the nasal domes has been brought to the center to form the columella, with the lift-up suture of the nasal cartilage, there is a tendency for the protruded nasal tip and visible inner portion of the nostrils at frontal view. Despite having rotated triangular flap D at the columella base, the columella labial angle was still obtuse compared to normal. We believe that this may be because of the absolute insufficiency in the amount of tissue in the bilateral cleft lip and nose and because the attachment of the columella base to the premaxilla is sometimes shifted caudally. It may be necessary to use VY plasty to move the columella base in the cranial direction (Fig. 5). Despite these remaining problems, development of the nose was favorable overall, and long-term outcome is encouraging.
3. Discussion
Characteristics of bilateral cleft lip include protruding premaxilla, short columella, flat and widened nasal tip, anteriorly flared nostril rim, and widely displaced alar base. Previously, at the early stage bilateral cleft lip surgery, a widely used approach was to perform a staged operation in which the clefts were closed in two separate sessions and the surgical correction of nasal deformity was deferred to await the growth of the nose. However, recently it has become common place to conduct bilateral closure in one stage,
There are some differences such as the triangular flap raised at the prolabium of the columella base and moved 90° dorsally to emphasize the columella-labial angle; the prolabium tissue itself is used for the white skin roll of the prolabium; relaxation sutures are used to narrow the nostril base without using a vertical incision into the skin of the alar base; nostril floor is formed by advancing alar base flap C including tipped tissue toward alveolus, and in nasal reconstruction (Fig. 5(A)), an inverted trapezoid suture is introduced to reconstruct correct framework of the nasal cartilage and form a distinct alar groove. Since scars can readily become conspicuous in Asians, we believe that it is desirable to minimize the horizontal scar on the white lip. There are reports using an open approach and performing corrective suturing of the nasal cartilage under direct vision.
However, wide dissection leaving large areas of scar tissue in the nose can make secondary revision at a later date difficult. The long-term follow-up of our procedure showed good nostril configuration and columella formation. However, there are slight tendency for superior deviation of the nasal tip. Despite having moved the triangular flap at the columella base in the posterior direction, the columella labial angle was still obtuse. We believe that this may be due to an imbalance in the tissue distribution, because the tissues in the bilateral cleft lip and the nose are hypoplastic and attachment of the columella base to the premaxilla is located caudally. However, there is a report that development of the columella in the lower anterior direction becomes active beginning at around 12 years of age.
For this reason, we plan to continue follow-up and, if needed, perform VY plasty or other techniques on the columella base.
Our primary nose correction is performed by minimizing the dissection around the perinasal area through a rim incision, and the inverted trapezoid suture is used to form a correct cartilage frame and a distinct alar groove, followed by an additional lift-up suture for the cartilage. For this reason, to prevent relapse of nasal deformity, it is effective to use sleeved long nose retainer suspension. It also allows for effective correction of the curvature of the anterior portion of the septal cartilage, and avoid pressure sore around the nostril rim.
Acknowledgements
The author wishes to thank co-workers of the Cleft Lip and Palate Center at Fujita Health University. Where many of these cases were treated when first author was a member of the Department of Plastic and Reconstructive Surgery.
References
Nakajima T
Yoshimura Y
Nakanishi Y
Kuwahara M
Oka T
Comprehensive treatment of bilateral cleft lip by multidisciplinary team approach.
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