Surgical Management of Facial Clefts

David Matthews

Samer Abouzeid

Edward W. Kubek

DEFINITION

Craniofacial clefts are rare congenital anomalies of facial development involving the facial bones, overlying soft tissue, and temporomandibular joint.
Facial clefts are classified by their involvement of the orbitomaxillary anatomy according to the Tessier classification.¹
Each craniofacial cleft is associated with characteristic, yet highly variable, bony and soft tissue changes ranging from mild hypoplasia to total agenesis.
Facial clefts and labiopalatine clefts are separate entities with key differences in surgical management and incidence.
The extreme variation of facial cleft anatomy mandates a unique surgical approach for every patient.²

ANATOMY

Dr. Paul Tessier published his 0 to 14 organizational system in 1976 (FIG 1).¹

Because of its clinical utility and relative simplicity, Tessier’s nominal system continues to be used widely.

FIG 1 • Tessier classification of clefts. A. Paths of various clefts on the face. B. Location of the clefts on the facial skeleton. (From Tessier P. Anatomical Classification of facial, craniofacial and lateral-facial clefts. J Maxillofac Surg. 1976;4:69-92.)

For purposes of nomenclature, the palpebral fissure/orbit separates lower facial clefts from their cranial extensions.
- When they occur together, the numerology of the lower facial cleft plus its cranial extension always equals 14.
Tessier’s 0 to 14 system is based on clinical experience, not embryologic study (Table 1).

PATHOGENESIS

The etiology of facial clefting is incompletely understood but likely involves both genetic and epigenetic factors.²
Abnormalities or premature resolution of the stapedial artery during fetal development is known to cause facial clefting.
The severity of the deformity relates to the timing of stapedial artery regression in utero.

PATIENT HISTORY AND PHYSICAL FINDINGS

In facial clefts, the bone and soft tissue are rarely affected to the same degree.

Table 1 Tessier System of Cleft Identification

Tessier Cleft No.	Description	Cranial Extension Equivalent Cleft No.	Bony Abnormalities	Effects on Soft Tissue and Appearance	Defining Features
0	Median craniofacial dysraphia	14	Similar to labiopalatine cleft but located at the midline (teeth fall into cleft)² Wide nasal bones with enlargement of the sphenoid and ethmoid sinuses Duplication of the crista galli Cleft of the primary and secondary palate Maxilla lacking vertical height Hypertelorism	Broad philtral columns Duplication of midline nasal structures, including the nasal spine and septum Bifid nose and labial frenulum	Duplication of midline nasal structures (nasal spine and septum) Operating after eruption of the permanent central incisors correlates with nearly uninterrupted anterior cranial fossa growth
1	Paramedian craniofacial cleft	13	Traverses nasal bone and maxilla into the pyriform Results in an anterior open bite Effect on the ethmoids results in orbital hypertelorism	Traverses the dome of the alar cartilage, giving a notched appearance Widening of the columella and nasal tip	Vertical orbital dystopia Telecanthus results secondary to the cleft traversing structures medial to medial canthus
2	Paramedian craniofacial cleft	12	Traverses the lateral mass of the ethmoid with malformation of ethmoidal labyrinth Localization on the frontal bone (corresponding with no. 12 cleft) is anatomically imprecise Flat glabella with enlarged frontal sinus	Traverses between the nasal tip and the base of the alar cartilage Associated with cleft lip Ipsilateral absence of the lacrimal apparatus Hypertelorism	Cleft is transnasal, not paranasal Can see ipsilateral absence of lacrimal apparatus, normal lacrimal function, or a spectrum of malfunction due to hypoplasia (eg, dacrocyst formation) Telecanthus secondary to the cleft traversing structures medial to medial canthus
3	Medial orbitomaxillary cleft or paranasal cleft	11	The oblique course of the cleft traverses the lacrimal groove Frontal process of maxilla is often absent Medial wall of maxillary sinus is often absent	Traverses the alar base, nasolabial fold, and alveolus, causing a cleft lip	Moving lateral, the no. 3 cleft is the first and most medial paranasal cleft It is characterized by lateral nasal shortness, malformation of the nasal ala, and absence of the frontal process; communication of the maxillary sinus with the nasal septum makes the no. 3 cleft one of the most difficult to repair Cleft no. 3 traverses the lacrimal groove and frontomaxillary process Therefore, in a cleft no. 3, the nasal cavity communicates with the maxillary sinus Cleft no. 3 is the most common Tessier cleft
4	Median orbitomaxillary cleft	10	Traverses infraorbital rim and floor medial to infraorbital nerve Traverses the maxillary sinus leading to sinus exstrophy Associated alveolar cleft appears similar to the typical alveolar cleft	Vertically traverses the lacrimal segment of the lower lid Cleft lip is located between philtral crest and commissure	Cleft no. 4 traverses the infraorbital rim medial to the infraorbital foramen Superior displacement of nasal ala causes soft tissue deficit between medial aspect of the lower lid and lip Normal nasolacrimal system (this cleft is lateral to these structures)
5	Lateral orbitomaxillary cleft	9	Traverses the infraorbital rim lateral to infraorbital foramen Results in an alveolar cleft of the premolar region	Traverses the medial third of the lower lid Soft tissue groove is visible on the cheek from lower lid to lateral lip near the commissure	Soft tissue groove in the cheek from lower lid to ipsilateral oral commissure The sclerotic furrow of the cheek inferiorly displaces the lateral canthus and palpebral fissure
6	Intermaxillozygomatic cleft	8	Located between the maxilla and zygoma; opens into the inferior orbital fissure	Laterally located coloboma of the lower lid	When clefts 6, 7, and 8 occur simultaneously, they constitute Treacher Collins syndrome
7	Temporozygomatic cleft	7	Absent or atrophic zygomatic arch Shortening of the ascending ramus and reduced height of the maxilla Absence of the coronoid and mandibular condyle is common	Absence of the coronoid is accompanied by ipsilateral absence of the temporalis muscle Microtia and pretragal enchondromas Macrostomia	Cleft no. 7 is associated with hemifacial microsomia (otomandibular syndrome) Most laterally located cleft Possible parotid gland malfunction and abnormalities of cranial nerves 5 and 7
8	Frontozygomatic cleft	6	When the malar portion of zygoma is absent, the greater wing of sphenoid becomes the lateral-most border of the orbit	A true cleft of the lateral canthus is associated with Goldenhar syndrome	Associated with Goldenhar syndrome (with the soft tissue deformity predominating) Also associated with Treacher Collins syndrome (with the bony deformity predominating)
9	Superolateral orbital cleft	5	Traverses the superolateral orbit	Traverses the lateral third of the upper lid	Cleft no. 9 is the rarest facial cleft
10	Central superior orbital cleft	4	Traverses medial third of supraorbital rim and orbital roof Lateral to the supraorbital nerve	Coloboma of the upper lid can be severe (clinical ablepharia)	Often associated with fronto-orbital encephalocele, orbital dystopia, and hypertelorism Potential for total ablepharia
11	Superomedial orbital cleft	3	Traverses the ethmoid or supraorbital rim lateral to the ethmoid	Coloboma of the medial third of the upper lid Cleft traverses the medial brow	Normal cranial base
12	Paramedian craniofacial cleft	2	Traverses frontal bone and lateral mass of the ethmoid Enlargement of the frontal sinus	Coloboma at the root of the eyebrow	Not associated with encephalocele
13	Paramedian craniofacial cleft	1	Traverses the frontal bone and olfactory groove of the cribriform plate Absence of the ipsilateral nasal bone	Cleft is medial to the eyebrow Associated with encephalocele and hypertelorism	Often occurs bilaterally Bilateral no. 13 clefts are associated with severe hypertelorism
14	Median craniofacial dysraphia	0	As with cleft no. 0, often see duplication or widening of midline structures Bifid crista galli and ethmoid plate may be present Rotation of the sphenoid wings causes attenuation of the middle cranial fossa	Widening or duplication of midline anatomy, including frontonasal encephalocele, hypertelorism, and telecanthus	Frontonasal encephalocele, hypertelorism, and telecanthus