Tessier 7—Macrostomia Repair

Patrick A. Gerety

Scott P. Bartlett

ANATOMY¹

Oral commissure (FIG 1)
- Position
  - Normal position: at a vertical line dropped from the medial limbus (some variability)
- Shape
  - Normal shape: The upper and lower lip vermilion comes to sharp corner.
  - Macrostomic commissure: Oblique angle, gaping at rest, round with smile

Tessier 7 cleft resulting in macrostomia

Variable in presentation—may be slightly wider oral commissure or severe cleft of skin, subcutaneous tissue, muscle, and bone

Trajectory—the cleft occurs along a line from the oral commissure to the zygomaticotemporal suture.

FIG 1 • Lateral facial cleft creates discontinuity of the orbicularis oris at the oral commissure. This in turn affects the location and insertion of the zygomaticus, risorius, and depressor anguli oris muscles.

Affected structures
- Commissure—laterally positioned, anatomically abnormal (ie, made of cleft tissue [lighter in color than normal vermilion])
- Orbicularis oris—interrupted and malinserted. The superior and inferior limbs contribute to the appearance of the lips on the macrostomic side (see FIG 1).

PATHOGENESIS

Development of normal facial anatomy is dependent upon early first and second branchial arch embryology.
The maxillary and mandibular prominences normally fuse in early embryologic development (weeks 4-6) forming the normal cheeks and lips.
Stapedial artery is the blood supply before the external carotid system has developed.
Stapedial disruption starves all tissue types—skin, fat, muscle, and bone.
Macrostomia results as a presumed failure of this process.
Interruption of embryologic development along the “orotragal” line can affect the oral commissure, muscles of mastication, muscles of facial expression, mandible, maxilla, and auricle.
Isolated macrostomia is a disorder of the first branchial arch only.

NATURAL HISTORY²

Incidence: rare, 1 in 225 000 live births
Unilateral 80%, bilateral 20%
As with cleft lip, the left side appears to be more commonly affected.
May be associated with syndromes including the following:
- Hemifacial microsomia
  - Including Goldenhar syndrome and variants of oculoauriculovertebral spectrum
  - May account for as many as 25% of macrostomia patients
- Treacher Collins syndrome
- Auriculocondylar syndrome
Tessier 7: the most common of the rare craniofacial clefts (5%-14%)

PATIENT HISTORY AND PHYSICAL FINDINGS

Laterally displaced oral commissure
Clefted/interrupted orbicularis oris
- Oral incompetence may be encountered.
- Gaping oral commissure in repose related to cleft orbicularis oris muscle
The cleft margin is demarcated. It does not contain normal lip elements (eg, white roll, dry vermilion), but it is mucosalized. The normal dark red vermilion can be seen to transition into a lighter colored cleft vermilion.
Spectrum of the cleft³
- Minor form—1 to 2 cm in length ending medial to the anterior border of the masseter muscle
- Major form—cleft ends lateral to the medial border of the masseter. It may terminate at the tragus or at the tonsillar pillar with disruption of superficial and deep structures.
The commissure is blunted/effaced, and when a baby with macrostomia cries, the commissural angle of an open mouth is more oblique than the normally formed commissure—this is thought to be related to disruption of the normally intact orbicularis ring.
Associated anomalies
- Preauricular tags
- Microtia/ear anomaly
- Mandibular hypoplasia
- Condylar abnormality
- Zygomatic arch abnormality
- Hearing loss

IMAGING

3D craniofacial CT—if bony involvement is suspected, this may be obtained to evaluate anatomy.

SURGICAL MANAGEMENT

Trilaminar repair—repair of oral mucosal, orbicularis muscle, and facial skin
Repair consists of
- Excision of abnormal cleft margin tissue (mucosa)
- Repair/repositioning of orbicularis muscle
- Recreation of the oral commissure
- Rearrangement of the cutaneous scar to avoid deforming contracture
Repair types (external cutaneous scar):
- Straight line
- Z-plasty
- W-plasty

Preoperative Planning

Standard facial photos should be obtained preoperatively including repose and animated (smiling) if possible.
Appropriate expectations should be created with the parents in terms of realistic commissure appearance and the risk of scar hypertrophy/contracture.
Evaluation for other anomalies and comorbidities with specialist consultation or multidisciplinary team as necessary
3D CT scan if cleft is thought to involve skeletal structures
Timing—as with cleft lip surgery, macrostomia repair may be done early in life. It is also reasonable to delay until late infancy if there is minimal functional or feeding issue. Late treatment reported in the literature is a function of later age at presentation and is usually not due to planned delay in treatment.