Transverse facial cleft: A series of 17 cases.

INTRODUCTION: Transverse facial cleft (Tessier type 7) or congenital macrostomia is a rare congenital anomaly seldom occurring alone and is frequently associated with deformities of the structures developing from the first and second branchial arches. The reported incidence of No. 7 cleft varies from 1 in 60,000 to 1 in 300,000 live births.
MATERIAL AND METHODS: Seventeen patients of transeverse facial cleft who presented to us in last 5 years were included in the study. Their history regarding familial and environmental predispositions was recorded. The cases were analysed on basis of sex, laterality, severity, associated anomalies and were graded according to severity. They were operated by z plasty technique and were followed up for 2 years to look for effectiveness of the technique and its complications. RESULT: Out of the seventeen patients of transverse cleft, none had familial predilection or any environmental etiology like antenatal radiological exposure or intake of drugs of teratogenic potential. Most of the patients (9/17) were associated with hemifacial microsomia and 1 patient was associated with Treacher Colin's Syndrome. Out of the 6 cases of Grade I clefts, 4 were isolated transverse clefts and of the 10 patients of Grade II clefts, 7 were associated with hemifacial microsomia. We encountered only one case of Grade III Transverse Cleft which was not only associated with hemifacial microsomia but also had cardiac anomaly. Out of the 17 cases, 15 were operated and in most of them the outcome was satisfactory.

INTRODUCTION

Transverse facial or Tessier No. 7 cleft or congenital macrostomia is a rare congenital anomaly.[1] This developmental aberration results from failure of fusion of the maxillary and mandibular processes of the first branchial arch.[23] This also explains the frequent association of transverse cleft with developmental anomalies of the first and second branchial arches.[2] Isolated transverse clefts are rare. The reported incidence of No. 7 cleft varies from 1 in 60,000 to 1 in 300,000 live births.[3] In most of the series in literature transverse cleft account for less than 0.5% of all clefts. The affliction can vary from slight widening of the mouth, to a cleft extending to the ear. But mostly these clefts are unilateral and do not extend beyond the anterior border of the masseter.[4]

Transverse clefts develop either due to failure of the maxillary and mandibular processes to fuse or a disruption in the processes after fusing. Although the former is a more acceptable theory, Gorlin and others claim that post-merging tear is the cause.[56]

MATERIALS AND METHODS

The age of the patients ranged from 2 years to 19 years. The cases were analysed on basis of sex, laterality, severity, associated anomalies operative result and complications.

The severity was graded as:

Grade I: Slight widening of angle of mouth

Grade II: Cleft extending till the anterior border of the masseter.

Grade III: Cleft extending beyond the anterior border of the masseter.

Transverse clefts repair

The Z-plasty technique of Longacre[7] was followed to correct these deformities. The reconstruction goals were creation of a normal commissure, which was symmetrical to the contralateral side, closure of the mucosal and skin defect lateral to the neo-commissure and restoration of the perioral orbicularis sphincter by reconstructing the modiolus. The position of the neo-commisure was determined by the mid-pupillary line in straight gaze and both commissures being equidistant from the midline [Figure 1a]. In addition, the neo-commissure was positioned just where the elevated mucocutaneous ridge of vermilion terminated at the normal anatomical limit of the lip [Figure 1b]. After the position of the neo-commissure was marked, the mucosa lining the cleft lateral to it was elevated as turnover flap and sutured to close the mucosal defect [Figure 1c]. The orbicularis muscle bellies of the upper and lower lip at the angle were dissected [Figure 1c]. The neo-modiolus was created by overlapping the bellies and suturing them just lateral to the proposed commissure [Figure 1d]. The skin was sutured with accurate alignment of the white line vermilion ridge [Figure 1e]. A Z was interposed in the skin suture line, the central limb of the Z in line with the nasolabial crease [Figure 1e]. During follow up scar contraction and hypertrophy and symmetry of the neocommissure with contralateral side were noted.

Figure 1a

Marking of neo-commissure and Z-plasty

Figure 1b

Note the elevated mucocutaneous ridge of vermilion terminates at the normal limit of the lip. The turnover flaps are marked for mucosal repair

Figure 1c

The orbicularis muscle bellies of the upper and lower lip at the angle dissected

Figure 1d

The neo-modiolus was created by overlapping the upper belly over the lower belly and suturing them just lateral to the proposed commissure

Figure 1e

Skin sutured with accurate alignment of the white line vermilion ridge and a Z interposed in the skin suture line

RESULTS

Seventeen patients of transverse left presented to us in last five years. None of the patients had a family history of cleft, history of antenatal radiological exposure or intake of drugs of teratogenic potential. The syndromic association was extrapolated with grade of affliction. Most of the transverse cleft patients (9/17) were associated with hemifacial microsomia and 1 patient was associated with Treacher Colin's Syndrome [Figure 3]. Only 7 patients were isolated transverse cleft [Figures 1a and 2a]. One case was associated with Treacher Colin's Syndrome and cleft palate [Figure 4]. We encountered only one case of Grade III Transverse Cleft which was not only associated with Hemifacial Microsomia but also a cardiac anomaly. Out of the 17 cases, 15 were operated. The Grade III transverse cleft with associated congenital cardiac anomaly succumbed to chest infection.

Figure 3

Unilateral syndromic transverse cleft, associated with hemifacial microsomia and facial palsy

Figure 2a

Unilateral transverse cleft, Pre-operative

Figure 4

Bilateral transverse cleft associated with Treacher Colin's syndrome

Unilateral transverse cleft, operated, Post-operative

Regarding complications, 1 patient had mild restriction of mouth opening due to excessive overlapping of the bellies of orbicularis oris. The patient was re-operated after 3 months and the problem was resolved by dividing a part of the neo-modiolus. One patient had partial suture line dehiscence which needed secondary suturing. One patient complained of a fold of excess mucosa on the repaired site, which he was biting repeatedly. The excess fold was excised and primarily closed.

Out of the 17 cases, 10 cases could be followed up for 2 years. The age of these patients ranged from 2 to 8 years. In all these cases, there was no migration of the neo-commissure and its symmetry with contralateral side was maintained. None of these patients had scar hypertrophy. In all the repaired patients, the oral sphincter functioned normally and no one had sialorrhea or any speech defect.

DISCUSSION

Transverse facial cleft (Tessier type 7) or congenital macrostomia is a rare congenital anomaly seldom occurring alone and is frequently associated with deformities of the structures developing from the first and second branchial arches. In Anderson's series of 3988 clefts over a period of thirty years, only 13 were transverse clefts accounting for 0.3% of the series.[8] Ibrahim reported that 3 of 121 patients with cleft palate-lip had a transverse facial cleft (2.48%).[9] In this context, our report of 17 cases over a period of 5 years is significant. Another notable aspect of our series is that 7 out of 17 were non-syndromic and isolated transverse clefts. This is at variance to most of the reported series where majority of the cases were associated with hemifacial microsomia. Transverse cleft are known to be more common in males and have left predilection. Contrary to this we had 9 females and 8 males. We had a right-sided predominance with 8 right and 6 left-sided clefts. Bilateral transverse cleft is an even rarer entity[10-14] and it is significant that there were 3 bilateral cases, out of which 2 were non-syndromic and 1 was associated with Treacher Colin's syndrome and cleft palate.

In addition to hemifacial microsomia it may be associated with pretragal skin tags, microtia, absence of the eustachian tube, temporomandibular joint, zygomatic arch, and eyelids, polydactyly, accessory maxillae and cardiac anomalies.[81015-19] In order to standardize the severity, cases of the series were categorized in three grades. It is significant to note Grade I cases were mostly isolated (4/6) and Grade II cases were mostly syndromic (7/10). The solitary Grade III cleft was also associated with hemifacial microsomia. The direct correlation of severity and associated anomaly can be explained by the fact that there is greater insult to facial development in cases of syndromic clefts.

As for any other facial congenital anomaly transverse facial cleft should also repaired at the earliest in order to relieve parents’ anxiety. In this series, we found that only 4 patients were repaired before 5 years of age, 8 before 15 years and 2 were operated after 15 years of age. In the literature, numerous operative techniques have been described. May first postulated the importance of recreating the orbiculais sphincter.[12] Longacre et al. described Z-plasty on the skin closure line to prevent scar contraction.[5] Nagai and Weinstein applied a modified Estlander-type flap to repair the oral commissure.[20] Onizuka, and Chen and Noordhoff inserted a small triangular flap into the mucosa at the lower part of the commissure in addition to Z-plasty.[2122]

All the 15 cases were repaired by the technique of Z interposition and all of them had satisfactory cosmetic and functional outcome. Although the need of Z interposition has been debated by some authors,[23] we found Z plasty technique of Longacre[5] satisfactory.

In conclusion, certain technical aspects of the repair need to be emphasized. While recreating the modiolus, care must be taken during suturing the bellies of orbicularis oris at the new angle of mouth. Suturing it medially or excessive overlapping leads to tightening of the of the orbicularis sphincter. It is a safe practice to open the mouth gently after repair while checking the tension of the muscle.