Median cleft lip refers to a vertical cleft on the midline of the upper lip. It is a rare congenital craniofacial anomaly brought about by a fusion failure in the medial nasal prominence. A novel surgical approach to median cleft lips and their repair is reported herein, with reference to a clinical case. The patient had a cleft in the lower half of the upper lip. There were no other craniofacial anomalies in this patient other than the cleft. Within the framework of the patient’s surgical treatment, a functional and cosmetically satisfactory result was achieved by performing a V–Y advancement flap on the columella base, Z-plasty in the vermillion zone, and Z pattern muscular tissue repair, without having to resort to any tissue excisions.
Median cleft lip is a rare congenital craniofacial anomaly. Its incidence has been reported to be between 0.43% and 0.73% among all patients with cleft lips. There are two theories explaining the formation of clefts: the existence of a defect in the mesodermal migration of the frontonasal and maxillary prominence, and a fusion failure of the medial nasal prominences. The severity of the cleft lip is variable; they range from a simple notch to a large cleft accompanied by hypertelorism.
Median cleft lip is a rare cleft, and since the level of the cleft varies, there are different approaches to surgical treatment. The case of a 4-month-old male patient with isolated incomplete median cleft lip and his treatment are presented herein. The aim in presenting this case is to demonstrate a flap technique as a new approach to surgical treatment that creates an acceptable cosmetic and functional lip structure without any vertical tissue excision.
A 4-month-old male patient was referred because of a median cleft on the upper lip ( Fig. 1 ). The family had no history of cleft. The pregnancy had gone to term and had been without any complications. The mother did not have any diseases and was not exposed to any environmental risk during pregnancy. The patient’s birth weight was normal and he had no additional anomalies. A physical examination revealed that he had a cleft on the lower half of the upper lip and a skin depression on the upper half. It was seen that there was a midline discontinuity through the whole orbicularis oris muscle. There was an insignificant shortness of the philtrum. The columella width was normal. There were no alveolar bone abnormalities. The nose and intercanthal distance were normal. The results of routine laboratory tests were within normal bounds.
Surgical treatment was planned as shown in Fig. 2 A . A skin incision was performed and the intact skin on the upper half lip was prepared in the form of a V–Y advancement flap (‘a’ flap). Philtral extension was provided by this flap. Triangular mucosal ‘b’ and ‘c’ flaps were created. No skin or mucosal excision was performed.
Following skin and mucosal incisions, the abnormal orbicularis oris muscle insertions at the alar margin and the nostril sills were released. The muscle layer was sutured midline in a Z pattern using 4–0 Vicryl. Mucosal flaps were crossed in the form of a Z-plasty ( Fig. 2 B). The tissue deficiency on the vermillion midline was resolved with this Z-plasty, and a lip tubercle was achieved.
Patient follow-up showed a cosmetically satisfactory result at 6 months post-operative ( Fig. 3 ). Sufficient philtral length, natural lip tubercle, and Cupid’s bow were achieved through the surgical technique used.
The median or midline cleft lip is a rare anomaly. It can be seen as an isolated cleft and also as part of a syndrome. The literature offers various classifications, although there is no clearly accepted classification. Veau defined three different variants of median cleft lip: notch on the vermillion, median cleft expanding into the columella base, and defect due to atrophy in the midline facial structures. The case presented here could be classified into the second category of the Veau classification, with total muscular disconnection and cleft in the lower half of the lip.
Another classification was put forward by DeMyer : complete median cleft accompanied by hypertelorism or hypotelorism. The case presented here had no hypo- or hypertelorism. Springer et al. , who reported a case with normal distance between the eyes and incomplete cleft lip, suggested that a third group should be added to this classification.
The Tessier 0–14 cleft classification includes the midline of the face and cranium. A number 0 cleft is the most common of the craniofacial clefts and relates to median craniofacial dysraphia. This includes the true or false median lip cleft, with or without associated hypertelorism or hypotelorism. The true median cleft lip is a splitting gap and results from the failure of fusion of two medial nasal processes between the fifth and eighth weeks of pregnancy. The false median cleft lip is a tissue defect. Agenesis is seen in the medial nasal processes in false cleft cases and it is a subtype of holoprosencephaly. In the management of the false median cleft, it is advisable to wait until the child is aged 1–2 years before proceeding with a corrective surgical procedure, because most patients with severe holoprosencephaly do not survive past this age.
Cupid’s bow, labial philtrum, vermillion, orbicularis oris muscle, and buccal mucosa repairs should be performed in the surgical treatment of the true median cleft. The literature offers different surgical repair options, since the severity of the cleft varies in many cases. The literature reports microform cleft cases treated with a reverse V excision or elliptic excision. Microform clefts can be treated by partial excision and repair because there is no distinctive tissue deficiency in the vermillion zone.
Median clefts related to the lower half of the upper lip have an abnormal philtrum complex. Ghildiyal et al. reported the surgical treatment of a patient with a cleft in the lower half of the upper lip and depression in the columella base. The authors repaired the lip with a double triangular flap following surgical excision. Jian et al. performed a Z-plasty on the columella base following vertical incision within the framework of their surgical treatment of a median cleft accompanied by teratoid polyp on the upper lip midline.
The case presented here also involved a median cleft, including the lower half of the upper lip. No vertical excision was performed because the upper lip, especially the vermillion and the lip tubercle area, had a tissue deficiency. A more acceptable scar beside the philtral extension was achieved by advancing the intact skin on the upper half of the lip in the V–Y form towards the columella base. Further, the tissue deficiency was overcome by placing the mucosal flaps, which had been obtained by protecting instead of excising in the vermillion zone, in the form of a Z-plasty. A natural lip tubercle and Cupid’s bow were achieved with these mucosal flaps.
In conclusion, the isolated median cleft lip is a rare anomaly with a varying level of cleft. The goal in surgical treatment is to obtain a lip structure that is close to the natural one. In incomplete clefts, a functional and cosmetically satisfactory result can be achieved by performing a V–Y advancement flap on the columella base and a Z-plasty in the vermillion zone without having to resort to tissue excision.