CLINICAL ARTICLE

A Surgical Conundrum: Tessier Number 4 Cleft

ATILLA CORUH, M.D.
GALIP K. GUNAY, M.D.

Objective: Among the rarest of the craniofacial clefts is the Tessier no. 4
cleft; and hence little has been published about its management and treatment.
Complete forms of the cleft yield poor surgical results because of the short-
ened oculoalar and oculo-oral distance and inadequate soft and bony tissue.
Multiple sequential corrective operations are required. A primary early concern
is the protection of the eye, particularly in severe forms in which corneal ex-
posure occurs. This article presents two cases of Tessier no. 4 clefts, one
unilateral and the other bilateral, and discusses the problems encountered dur-
ing their surgical and postoperative managements.

KEY WORDS: atypical cleft, cleft palate, craniofacial cleft, Tessier no. 4 cleft

The incidence of rare facial clefts is between 1.43 and 4.85
per 100,000 births and 9.5 and 34 per 1000 clefts (Kawamoto,
1976). The Tessier no. 4 cleft is one of the rarest craniofacial
clefts, with fewer than 50 cases having being reported in the
literature (Resnick and Kawamoto, 1990).
The Tessier no. 4 cleft is characterized by a cleft lip, de-
creased oculo-oral and oculoalar distance, orbital dystopia,
eyelid colobomas, inferiorly displaced medial canthus, and
cleft palate. This cleft may be found in two forms: complete
and incomplete forme frustetype clefting. The forme fruste
type cleft is characterized by minimal soft and bony tissue
deformities. In the complete form, the orbital and oral cavities
are confluent with the maxillary antrum. The soft tissue cleft
is located lateral to the cupids bow and the philtrum, midway
between the philtral ridge and the corner of the mouth. It con-
tinues on to the cheek, lateral to the nasal ala, ascending to
the medial portion of the lower eyelid. The lacrimal sac and
canalis nasolacrimalis are intact because the cleft passes lateral
to these structures; however, the inferior canaliculus generally
lies in the path of the cleft and is usually defective. The cleft-
side medial canthal ligament is almost normal. The alveolar
bony cleft is located between the lateral incisor and the canine
teeth sparing the piriform aperture. The cleft then continues
on to the anterior surface of the maxilla medial to the infra-
orbital foramen to terminate in the medial portion of the in-
ferior orbital rim and floor. The eye is usually present and
functional, although microphthalmos and anophthalmos may
Dr. Coruh is an Assistant Professor, and Dr. Gunay is a Professor, Depart-
ment of Plastic and Reconstructive Surgery, Medical Faculty, Erciyes Univer-
sity, Kayseri, Turkey.
Submitted September 2003; Accepted December 2003.
Address correspondence to: Dr. Atilla Coruh, Alpaslan Mahallesi, Esref Bitlis
Bulvari, Imaj Sitesi, 12/A, Kat: 13, Daire: 26, Kayseri, Turkey. E-mail atilla.
coruh@superonline.com.
also occur (Kawamoto, 1990; Stretch and Poole, 1990; Ka-
wamoto and Patel, 1998). Choanal atresiae may be associated
with craniofacial anomalies including various degrees of nasal
fossa malformation and may be part of paramedian facial clefts
(Kawamoto, 1990; Garabedian et al., 1996, 1999).
CASE REPORTS
Case 1
A 15-day-old boy presented with a bilateral Tessier no. 4
cleft. The left- and right-side clefts were a complete form of
Tessier no. 4 cleft. On the right side, the cleft began lateral to
the cupids bow, sparing the piriform aperture, passing lateral
to the alar groove and medial to the infraorbital foramen, ex-
tending to the infraorbital region as a wide cleft (Figs. 1 and
2). The orbital and oral cavities were confluent with the max-
illary antrum. Only the temporal one fourth of the lower eyelid
was present and the inferior punctum lacrimale could not be
detected. The bony and soft tissue clefts were alike in their
topographical anatomic landmarks. The left-side soft tissue
cleft was a notch, reducing the oculo-oral distance. The bony
alveolar cleft arose between the lateral incisor and the canine
tooth, passing through the alveolus, coursing lateral to the pir-
iform aperture, medial to the infraorbital foramen, extending
to the zygomaticomaxillary buttress to the medial orbital rim
and the floor all the way, as a fissure. The medial palpebral
ligaments were intact and displaced inferiorly. The eyes were
normal. There was a coloboma of the left upper eyelid situated
nasally. There was also complete cleft palate and bilaterally
situated choanal atresia; the left side was made of compact
bone and the right side was membranous in character (Fig. 3).
Ophthalmologic examination revealed serious bilateral cor-
neal exposure and ulceration. Urgent correction of the facial
cleft and the lower eyelids were planned using the technique

102

FIGURE 1 Case 1. Frontal photograph at 15 days old. Orbital, maxillary
and oral cavities are confluent on the right-side complete cleft. In addition,
bilateral severe corneal exposure is observed.
described by Resnick and Kawamoto (1990) when the patient
was 15 days old. No bony reconstruction was carried out, in-
cluding the right infraorbital floor. The coloboma of the left
upper eyelid, localized nasally, was closed primarily. The right
nonfunctional purulence-filled lacrimal sac was also excised.
At the age of 12 months, the infant underwent repair of the
palatal cleft by V-Y push-back palatoplasty to separate the an-
tral and oral cavities. Because of inadequate reconstruction of
the right lower eyelid and orbital floor (Fig. 4), at the age of
3 years, a Mustarde cheek flap (Mustarde, 1980) with conchal
cartilage in place of tarsal tissue and oral mucosal graft for
palpebral conjunctiva was used for lower eyelid reconstruction.
The orbital floor and infraorbital rim were restored with bone
grafts that separated antral and orbital cavities. Bilateral trans-
nasal canthopexies were also performed at the same operative
session (Fig. 5). At the age of 6 years, otorhinolaryngology
surgeons repaired the bilateral choanal atresia. The bone grafts
used for the right-side orbital floor and infraorbital rim were
partially resorbed when the patient was 6 years old (Fig. 6).
FIGURE 2 Case 1. Coronal computed tomography scan of patient. The
right bony defect of the orbital floor, infraorbital rim, and cleft palate is
seen. Inferior displacement of the globe is also evident.
Coruh and Gunay, TESSIER NO. 4 CLEFT 103
FIGURE 3 Case 1. Choanal atresiae, membranous on the right, bony on
the left on axial computed tomography.
The orbital floor and rim were reconstructed again with bone
grafts, and at the same operative session, temporal scar revi-
sion was also performed when the patient was 8 years old (Fig.
7).
Case 2
A 7-year-old girl presented with a right-sided Tessier no. 4
cleft. Cleft site orbita was anophthalmic and orbital cavity was
hypoplastic (Fig. 8). The soft tissue cleft was only an embry-
onic scar, which passed between the philtral column and the
oral commissure and continued lateral to the nasal ala onto the
cheek and ascended to the medial portion of the lower eyelid.
The cleft was medial to the inferior lacrimal punctum and the
medial canthal ligament was unaffected because the path of
the fault lay temporal to it. The ipsilateral nasolacrimal canal
and sac were intact, although the lower canaliculus was de-
fective. The bony cleft was a groove lying between the lateral
incisor and the canine teeth and was coursing lateral to the
piriform aperture through the anterior wall of the maxillary
FIGURE 4 Case 1. Frontal photograph at 3 years old. Dystopia of the
globe to the maxillary antrum, epicanthal fold, insufficient lower lid, cor-
neal exposure on the right cleft side.
104 Cleft PalateCraniofacial Journal, January 2005, Vol. 42 No. 1

FIGURE 5 Case 1. Two years after first bone grafting. Frontal photo-
graph at 5 years of age. The right-side orbital floor reconstruction was
carried out with bone grafts, and lower eyelid reconstruction with Mus-
tarde cheek flap and transnasal canthopexies with medial canthal ligament
remnants were performed.
FIGURE 7 Case 1. Early postoperative view at 8 years of age. The orbital
floor and the rim were reconstructed with bone grafts for the second time,
and the scar at the temporal region was revised. The right upper lid is
edematous.

FIGURE 6 Case 1. Three-dimensional computed tomography at the age of 6 years. Bone grafts used for right-side orbital floor and infraorbital rim
reconstruction are partially resorbed.
 Coruh and Gunay, TESSIER NO. 4 CLEFT 105

FIGURE 8 Case 2. Periorbital, three-dimensional computed tomography

FIGURE 9 Case 2. Frontal photograph at 7 years of age with a right-
of patient. The hypoplasia of the right anophthalmic orbital cavity is clear-
sided Tessier no. 4 forme fruste cleft.
ly seen.

antrum and medial to the inferior orbital foramen, ending at used to reconstruct this deformity may be inadequate. Recon-
the medial orbital rim and floor (Fig. 9). struction of the lower eyelid especially constitutes a difficult
Soft tissue closure was carried out with a superiorly based problem and is often an urgent procedure when exposure of
nasolabial flap to fit it along the lower eyelid to increase the the cornea occurs. Using well-known corrective procedures for
distance between the oculoalar region. The redundant skin be- Tessier no. 4 cleft may not suffice, as in the outcome of the
tween the philtral column and the cleft was excised and the first operation of case 1. The reconstructed lower eyelid on the
nasal complex was rotated in a counterclockwise direction ac- right side was inadequate and protected the eye poorly (Fig.
cording to a rotation flap principle. The right upper lip was 4). When the soft tissue defect of the infraorbital region and
repaired with advancement cheek flap (Longaker et al., 1997). especially the lower eyelid is extensive, use of a Mustarde
No bony reconstruction was performed for the fissure-like cheek flap may be an alternative for reconstruction of the low-
groove located on the anterior surface of the maxilla and the er eyelid. Tissue expansion seems to be an appropriate method
alveolar ridge. in which there is a shortage of soft tissues. Tissue expansion
In the future, with the aid of sequential orbital confirmers, also adds ideal similar skin color, texture, appearance, and aids
the hypoplastic orbital cavity will be expanded and an ocular in a perfect outcome of scar revisions (Toth et al., 1990; Men-
prosthesis will be placed after suitable orbital cavity expansion. ard et al., 1999).
If required, multiple surgical revisions may be performed in In case 1, orbital dystopia was minimal, which may be ex-
the future (Fig. 10). plained by early bone grafting. The necessity of secondary
bone grafting to the orbit indicates the difficult problems of
DISCUSSION bony correction in this anatomical region. The patient had in-
telligible speech with quite good occlusion of the upper and
Tessier classifications brought about simplicity in the un- lower jaw but still requires orthodontic treatment of anterior
derstanding and classification of rare facial clefts and also pi-
oneered their successful surgical treatment (Tessier, 1976). The
surgical treatment of this congenital anomaly includes: (1) me-
dial canthopexy and lower eyelid reconstruction to protect the
eye, which may be an emergency procedure; (2) bone grafting
for orbital bony continuity and maxillary bony deformities; (3)
reconstruction of the soft tissue defects of the cheek (if local
flaps do not suffice, tissue expansion may be a versatile tech-
nique for reconstruction); (4) cleft lip repair; (5) serial orbital
conformers for orbital expansion of anophthalmic or microph-
thalmic orbits; and (6) if required, multiple surgical revisions
may be performed for bone and soft tissue deformities as cor-
rection of medial and lateral canthal ligament malposition,
treatment of lower eyelid defects, and augmentation of anterior
maxillary region and the zygoma (Resnick and Kawamato,
1990).
When bony and soft tissue deformity is severe, the methods FIGURE 10 Case 2. Frontal photograph 1 year after the surgery.
106 Cleft PalateCraniofacial Journal, January 2005, Vol. 42 No. 1
open bite deformity and surgical treatment of the alveolar cleft.
The patient also received psychological help when he attended
primary school.
In case 2, a superiorly based nasolabial flap below the lower
eyelid was used to increase the distance between the oculo-
oral and oculoalar regions. During the 1-year follow-up period,
it was clearly seen that this technique brought about acceptable
aesthetic units and less color discrepancy between the flap, and
the neighboring tissues placed scars in less conspicuous areas
on the face and gave sufficient support to the lower eyelid.
If the soft tissue deficiency is severe, conventional tech-
niques using flaps or Z-plasties, which are designed to replace
the missing tissues, are far from being ideal. Mustarde cheek
flaps for lower eyelid reconstruction may be an alternative for
extensive facial clefts.
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