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Tessier Number 30 Facial Cleft: A Rare Maxillofacial Anomaly.

INTRODUCTION

Number 30 facial cleft was first reported as a caudal extension of number 14 and number 0 facial clefts by Tessier in 1976. [1] The deformities vary in their presentation, ranging from minor clefting of the lower lip [2] to complete clefts of the mandible with the absence of the hyoid bone, thyroid cartilage, and manibrium. [3] In this article, we present a case of midline lower lip cleft that was repaired in late adolescent period and a review of the literature.

A 20-year-old male patient was referred to our clinic with a midline cleft and ulceration of the lower lip without any other involvement [Figure 1]. Several treatment methods such as antibiotic ointments and antihistaminic drugs had been tried for the ulcer by dermatologists; however, the pathology was not resolved.

In physical examination, the upper lip, tongue, nose, and frontal region were found normal. The mandible and the soft tissue of the neck were not involved. Radiographic confirmation was done. Written consent was obtained from the patient, and the surgical approach was planned.

Under local anesthesia, an elliptic incision was performed, ulcer was debrided, and the orbicularis oris muscle was identified. Free edges of the muscle were dissected and sutured together in the midline. Mucosa and the vermillion line were connected meticulously. No complications were observed in the early postoperative period. At 10-month follow-up, the patient was satisfied with the result [Figure 2].

DISCUSSION

The incidence of craniofacial clefts is estimated between 1.4 and 4.9/100,000 live births. [4] Although two genders are being affected by craniofacial clefts, girls are more commonly affected. The pathology can develop due to large doses of radiation, infection, vitamin deficiencies, metabolic syndromes, and Vitamin A toxicity. [5-7]

In Tessier Classification, clefts 0-14 radiate around the orbital bone rims [Figure 3 and Table 1]. Tessier number 0 and number 3 are seen to be the most commonly reported cleft types in both genders in the literature. [8,9] Tessier number 30 cleft of the face is a rare congenital anomaly. The deformity occurs as insufficient fusion of first branchial arc or the failure of mesodermal penetration in midline. [10] Anomaly can range from a lower vermillion notching to the involvement of the teeth, tongue, mandible, and neck. [11-14] Tafreshi et al. reported a Tessier number 30 cleft case with congenital heart defects. [13] One patient was reported by Adetayo and Martin [15] with a Tessier number 30 cleft concomitant with levocardia, ventricular septal defect, a patent foramen ovale, double outlet right ventricle, intestinal malrotation, and bilateral undescended testicles. Vendramini-Pittoli et al. published two Tessier number 30 cleft cases. One of them was accompanied by a Tessier number 7 cleft, and the other one had developed together with Tessier number 4, 7, and 9 clefts. [16] Moreover, nasal anomalies and cleft lip and palate can also be seen with Tessier number 30 facial clefts. [17-19]

Midline cleft of the lower lip and mandible can also be a sign of a syndrome, such as the Robinow syndrome which presents itself with extremity, genitalia, costa and vertebra anomalies, [20] or the Richieri-Costa syndrome which involves limb defects. [21]

The prenatal diagnosis of the Tessier number 30 cleft can be made through ultrasonography at 15-18 weeks of the pregnancy. [22]

Reports about this pathology are rare in the literature. [10] Less than 80 cases have been reported since the first article by Couronne. [10,23] There are a few cases of craniofacial clefts reported from Turkey; [24-27] moreover, only two papers reporting cases from Turkey that are accompanied by a simple lower lip defect. [24,28] Due to this rarity, it can be said that there is no consensus on the management of this deformity. Therefore, we decided to prepare an algorithm for the treatment of the pathology.

Simple notching of the lower lip can be treated with wedge excision or Z-plasty. [29] Lip deformity and bifid tongue should be corrected as early as possible due to functional deficits. Duplication of the tongue can be corrected with wedge excision or Z-plasty. In addition, the tongue should be released from the floor of the mouth. [30]

Clefts of the mandible can be treated with one- or two-stage procedure depending on the range of the defect. [31] Most surgeons begin the procedure when the child is about 10-year-old to avoid tooth bud damage and use interosseous wires or plates. [32] On the other hand, Oostrom et al. report that a carefully planned osteosynthesis of the base of the mandible does not harm the buds and may provide better occlusion. [33] If the mandibular cleft is too wide, the bones should be stabilized at an early age to avoid obstructing eating and breathing. [34] A bone graft harvested from the costa, calvarium, or iliac crest can be inserted around the age of 10 in the second stage. [28,35,36]

One of the largest case series was reported by Freitas et al. with 17 patients aged from 1 month to 30 years. [36] Early surgery was performed in two patients with sleep apnea and impaired nutrition using distraction osteogenesis to achieve better tongue movement. They used Z-plasty for mucosal bands, muscle repair for the contraction, and straight closure of the skin in a few patients. Early bone grafting was recommended in this report to establish mandibular continuity.

The strap muscles of the neck are often replaced with dense scar tissue and cause flexion contracture bands and can be repaired with multiple Z-plasties. [1] Facial anomalies can extend to the widened interclavicular space, bifid or absent manubrium, presternal skin tags, cleft lip and palate, hemifacial microsomia, dermoid cyst of the face, extremity anomalies, and cardiac deformities. [10] Treatment choices for all these anomalies should be determined according to the type of the deformity.

CONCLUSION

The lower lip notching was corrected with wedge resection and three-layer closure in our patient. No complications occurred. Functional and esthetic results were good in the postoperative 10-month follow-up period. Tessier number 30 cleft should be kept in mind in lower lip anomalies.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

REFERENCES

[1.] Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg 1976;4:69-92.

[2.] Arshad AR. Incomplete midline cleft of lower lip. Cleft Palate Craniofac J 1995;32:167-9.

[3.] Morton CD, Jordan HE. Median cleft of lower lip and mandible, cleft sternum and absence of basihyoid: Report of case. Arch Surg 1935;30:647-56.

[4.] Ozaki W, Kawamoto HK Jr. Craniofacial clefting. In: Lin KY, Ogle RC, Jane JA, editors. Craniofacial Surgery: Science and Surgical Technique. Philadelphia, PA: Saunders; 2002. p. 309-31.

[5.] Hunt JA, Hobar PC. Common craniofacial anomalies: Facial clefts and encephaloceles. Plast Reconstr Surg 2003;112:606-15.

[6.] Murray JC. Face facts: Genes, environment, and clefts. Am J Hum Genet 1995;57:227-32.

[7.] Moore LL, Singer MR, Bradlee ML, Rothman KJ, Milunsky A. A prospective study of the risk of congenital defects associated with maternal obesity and diabetes mellitus. Epidemiology 2000;11:689-94.

[8.] Kalantar-Hormozi A, Abbaszadeh-Kasbi A, Goravanchi F, Davai NR. Prevalence of rare craniofacial clefts. J Craniofac Surg 2017;28:e467-70.

[9.] Morian R. About oblique facial clefts. Arch Klin Chir 1887;35:245.

[10.] Rao AY. Complete midline cleft of lower lip, mandible, tongue, floor of mouth with neck contracture: A Case report and review of literature. Craniomaxillofac Trauma Reconstr 2015;8:363-9.

[11.] Millard DR Jr., Lehman JA Jr., Deane M, Garst WP. Median cleft of the lower lip and mandible: A case report. Br J Plast Surg 1971;24:391-5.

[12.] Rosenthal R. Aglossia congenital: Report of a case of the condition combined with other congenital malformations. J Dis Child 1932;44:383-5.

[13.] Tafreshi M, Aminolsharieh Najafi S, Hasheminejad R, Mirfazeli A, Shafiee A. Tessier number 30 clefts with congenital heart defects. Iran Red Crescent Med J 2015;17:e19078.

[14.] Ladani P, Sailer HF, Sabnis R. Tessier 30 symphyseal mandibular cleft: Early simultaneous soft and hard tissue correction--A case report. J Craniomaxillofac Surg 2013;41:735-9.

[15.] Adetayo OA, Martin MC. A unique case of tessier 30 and bilateral cleft lip and palate: A new clinical syndrome? Cleft Palate Craniofac J 2013;50:117-21.

[16.] Vendramini-Pittoli S, Guion-Almeida ML, Santos JM, Garcia Junior SM, Yoshida MM, Richieri-Costa A, et al. An unusual presentation of oculoauriculovertebral spectrum with a tessier 30 cleft: Report on two cases. Clin Dysmorphol 2015;24:144-50.

[17.] Ozek C, Gundogan H, Bilkay U, Cankayali R, Guner U, Tokat C, et al. A case of total nasal agenesis accompanied by tessier no 30 cleft. Ann Plast Surg 2001;46:663-4.

[18.] Murrad K, Aldaghri F, Mrad MA. Nasal septal agenesis and attenuated lower and upper lateral cartilages in a 5-year-old child: A Sporadic finding. Eplasty 2016;16:e38.

[19.] de Jong-Pleij EA, Pistorius LR, Ribbert LS, Breugem CC, Bakker M, Tromp E, et al. Premaxillary protrusion assessment by the maxilla-nasion-mandible angle in fetuses with facial clefts. Prenat Diagn 2013;33:354-9.

[20.] Kargi E, Acun C, Toma9 N, Savranlar A, Dursun A, Babuccu O, et al. Midline cleft of the lower lip associated with robinow syndrome. Scand J Plast Reconstr Surg Hand Surg 2004;38:361-4.

[21.] Raskin S, Souza M, Medeiros MC, Manfron M, Chong E Silva DC. Richieri-costa and Pereira syndrome: Severe phenotype. Am J Med Genet A 2013;161A: 1999-2003.

[22.] Kavak SB, Celik E, Firat C, Goktolga G, Ilhan R, Orak U. Tessier number 30 cleft: Report of an antenatally diagnosed case. J Perinat Med 2014;3:71-3.

[23.] Monroe CW. Midline cleft of the lower lip, mandible and tongue with flexion contracture of the neck: case report and review of the literature. Plast Reconstr Surg 1966;38:312-9.

[24.] Seyhan T, Kilin9 H. Tessier Number 30 cleft: A Case Report and Review of Literature. Turk J Plast Surg 1999;7:187-90.

[25.] Sade C, Kuran I, Ari C, Karasoy A, Bas L. A Case of Number 7 Left Unilateral Neck Cleft According to Tessier Classification. Turk J Plast Surg 2001; 9:37-9.

[26.] Akoz T, Erdogan B, Gorgii M, Kapucu R, Kara9al N, Kutlay R. A Different Approach on the Treatment of Tessier Number 0 Cleft (Evaluation of Two Cases). Turk J Plast Surg 1996;4:179-84.

[27.] Okur Ml, Kose R, Yildirim AM. Surgical Treatment of Macrostomia in Lateral Facial Clefts. Turk J Plast Surg 2009;17:4-7.

[28.] Seyhan T, Kilinc H. Median cleft of the lower lip: Report of two new cases and review of the literature. Ann Otol Rhinol Laryngol 2002;111(3 Pt 1):217-21.

[29.] Monroe CW. Midline cleft of the lower lip, mandible and tongue with flexion contracture of the neck: Case report and review of the literature. Plast Reconstr Surg 1966;38:312-9.

[30.] Goswami JK. Tessier 30 facial cleft with duplication of tongue. J Indian Assoc Pediatr Surg 2017;22:46-7.

[31.] Millard DR Jr., Wolfe SA, Berkowitz S. Median cleft of the lower lip and mandible: Correction of the mandibular defect. Br J Plast Surg 1979;32:345-7.

[32.] Armstrong AP, Waterhouse N. Tessier 30 median mandibular cleft: Case report and literature review. Br J Plast Surg 1996;49:536-8.

[33.] Oostrom CA, Vermeij-Keers C, Gilbert PM, van der Meulen JC. Median cleft of the lower lip and mandible: Case reports, a new embryologic hypothesis, and subdivision. Plast Reconstr Surg 1996;97:313-20.

[34.] Sherman JE, Goulian D. The successful one-stage surgical management of a midline cleft of the lower lip, mandible, and tongue. Plast Reconstr Surg 1980;66:756-9.

[35.] Ishii M, Ishii Y, Moriyama T, Enomoto S, Ono T, Ohyama K, et al. Seventeen-year follow-up of a patient with median cleft of the lower lip, mandible, and tongue with flexion contracture: A case report. Cleft Palate Craniofac J 2002;39:555-9.

[36.] Freitas RS, Cruz GA, Alonso N, Forte AJ, Busato L, Persing J. Tessier number 30 clefts: Surgical correction of a rare malformation. Rev Bras Cir Craniomaxillofac 2010;13:12-7.

Elif Sari

Department of Plastic, Reconstructive and Aesthetic Surgery, Faculty of Medicine, Kirikkale University, Kirikkale, Turkey

Address for correspondence: Dr. Elif Sari, Department of Plastic, Reconstructive and Aesthetic Surgery, Kirikkale, Turkey.

E-mail: drelifsanli@hotmail.com

10.4103/tjps.tjps_7_18
Table 1: Clinical features of the Tessier craniofacial clefts

                  Tessier classification
                  Synonyms

Tessier number 0  Median craniofacial
                  dysraphia
                  Centrofacial microsomia
                  Frontonasal dysplasia
                  Median cleft face
                  syndrome
                  Holoprosencephaly

Tessier number 1  Type 3 nasoschisis nasal
                  dysplasia

Tessier number 2  -

Tessier number 3  Oro-naso-ocular cleft

Tessier number 4  Meloschisis
                  Oro-ocular cleft
                  Orofacial cleft
                  Medial maxillary
                  dysplasia

Tessier number 5  Oculofacial cleft II
                  Morian III cleft
                  Lateral maxillary
                  dysplasia
                  Oro-ocular type 2 cleft

Tessier number 6  Zygomatic-maxillary
                  cleft
                  Maxillozygomatic
                  dysplasia

Tessier number 7  Temporozygomatic
                  facial cleft
                  Craniofacial microsomia
                  Hemifacial microsomia
                  Otomandibular
                  dysostosis
                  First and second
                  branchial arch syndrome
                  Auriculo- branchiogenic
                  dysplasia
                  Hemignathia and
                  microtia syndrome
                  Oro-aural cleft
                  Group B1 lateral
                  otocephalic
                  branchiogenic deformity
                  Zygotemporal dysplasia

Tessier number 8  Frontozygomatic cleft
                  The equator of the
                  Tessier craniofacial time
                  zones

Tessier number 9  Frontosphenoid
                  dysplasia

Tessier number    -
10

Tessier number    -
11

Tessier number    -
12

Tessier number    -
13

Tessier number    Median craniofacial
14                dysraphia

Tessier number    Mandibular process cleft
30                Midline branchiogenic
                  syndrome
                  Intermandibular
                  dysplasia

                  Tessier classification
                  Major clinical features of the pathology

Tessier number 0  Deficiency of midline structures
                   Developmental arrest range from hypoplasia of the
                   nasomaxillary region and hypotelorism to a severe
                   cyclopia, ethmocephaly, or cebocephaly
                   False median cleft lip and absence of philtral
                   columns
                   Narrowed columella or totally absence of columella
                   Lack of septal support
                   Skeletal deficiencies range from separation between
                   the upper central canines to absence of the
                   premaxilla and cleft of the secondary palate
                   Partial or total absence of nasal bones and septal
                   cartilages
                   Hypotelorism or cyclopia
                   Encephalocele
                  Excess of midline tissue
                   True median cleft lip with broad philtral columns
                   Duplication of the labial frenulum
                   Bifid nose with a broad columella
                   Laterally displaced alae and upper lateral
                   cartilages
                   Skeletal excess can be seen as a diastema between
                   the upper central incisors
                   Duplicate nasal spine
                   Keel-shaped maxillary alveolus
                   Anterior open bite
                   Shortened central midface height
                   Cartilaginous and bony nasal septum is thickened or
                   duplicated
                   Nasal bones and nasal process of the maxilla are
                   broad, flattened, and displaced laterally
                   Ethmoid and sphenoid sinuses may be enlarged,
                   contributing to symmetric widening of the anterior
                   cranial fossa and hypertelorism
                   Displacement of the pterygoid plates away from the
                   midline
Tessier number 1  Soft tissue involvement
                   It is similar to the common cleft lip
                   Notching on soft triangle of the nose
                   Short and broad columella
                   The nasal tip and nasal septum deviate away from
                   the cleft
                   Malpositioned medial canthus, and telecanthus
                  Skeletal involvement
                   A keel-shaped maxilla and anterior open bite
                   An alveolar cleft that passes between the central
                   and lateral incisors
                   The cleft may extend posteriorly as a complete
                   cleft of the hard and soft palate
                   The nasal bones are displaced and flattened
                   Hypertelorism
Tessier number 2  Soft tissue involvement
                   Ala nasi is hypoplastic, lateral aspect of nose is
                   flattened and dorsum is broad
                   Cleft passes medially to the palpebral fissure and
                   eyelid is not involved
                   Lacrimal duct is usually intact
                  Skeletal involvement
                   Cleft begins between lateral incisor and canine,
                   extends into apertura piriformis
                   Hard or soft palate cleft can be accompanied with
                   the pathology
                   Cleft passes between nasal bone and frontal
                   process of maxilla
                   Orbital hypertelorism can be occurred
                   Asymmetry of the sphenoid wings and anterior
                   cranial base
Tessier number 3  Soft tissue involvement
                   Begins like number 1 and number 2 clefts
                   Nose is shortened at the effected side
                   Cleft extends between medial canthus and lacrimal
                   punctum, lacrimal system is affected
                   Recurrent ductal infection is common
                   Medial canthus is displaced inferiorly, colobomas
                   of lower eyelid are medial to inferior punctum
                   Microphthalmia may occur, eye is malpositioned
                   inferiorly and laterally
                  Skeletal involvement
                   This is an oro-naso-ocular cleft characterized by
                   communication of these 3 cavities
                   Cleft begins between lateral incisor and canine
                   Anterior maxillary arch is flattened
                   If the cleft is bilateral, skeletal disruption
                   is significant
                   Ethmoid and sphenoid sinuses are narrowed and
                   anterior cranial base is displaced inferiorly
Tessier number 4  Soft tissue involvement
                   Begins lateral to the Cupid's bow, between
                   tubercle and oral commissure
                   Orbicularis aris muscle is located laterally to
                   cleft and makes a mass-like appearance
                   Cleft passes lateral to the ala
                   Although nose is intact, alar base can be displaced
                   superiorly
                   Cleft extends to lower eyelid lateral to inferior
                   lacrimal punctum
                   Medial canthus and lacrimal system are intact
                   Globe is normal, however microphthalmia or
                   anophthalmia can be seen
                  Skeletal involvement
                   Alveolar cleft begins between lateral incisor and
                   canine
                   Extends lateral to piriform aperture and involves
                   maxillary sinus
                   Medial wall of maxillary sinus is intact
                   Cleft passes through inferior orbital rim medial to
                   infraorbital foramen
                   Globe can prolapse inferiorly due to absence of
                   medial aspect of inferior orbital wall
                   Sphenoid body is asymmetric and pterygoid plates
                   are displaced
                   Anterior cranial base is unaffected
Tessier number 5  Soft tissue involvement
                   Begins just medial to oral commissure, courses
                   along cheek lateral to alar rim
                   Terminates in lateral half of lower eyelid
                   Globe is normal but microphthalmia can be seen
                  Skeletal involvement
                   Alveolar cleft begins lateral to canines
                   Extends lateral to infraorbital foramen
                   Cleft terminates in lateral orbital rim and floor
                   Cleft does not affect the inferior orbital fissure
                   Maxillary sinus can be hypoplastic
                   Vertical orbital dystopia can be seen
                   Lateral orbital rim and wall may be thickened;
                   greater sphenoid wing is affected but cranial base
                   is normal
Tessier number 6  Soft tissue involvement
                   Can be seen as a hypoplastic soft tissue that
                   begins from orbital commissure and extends
                   to lateral lower
                   eyelid
                   Lateral palpebral fissure is pulled down, lateral
                   canthus is displaced inferiorly
                   Pathologies can cause severe ectropion of lower
                   eyelid and an antimongoloid appearance
                   Colobomas are located on lateral lower eyelid and
                   mark the cephalic end of the cleft
                  Skeletal involvement
                   Cleft separates zygoma and maxilla via affecting
                   the zygomaticomaxillary suture
                   No alveolar cleft but shortened posterior maxilla
                   can cause occlusal tilt
                   Choanal atresia is common
                   Cleft end in lateral third of the orbital rim and
                   floor
                   Connects to inferior orbital fissure
                   Zygoma is hypoplastic but zygomatic arc is intact
                   Anterior cranial fossa is narrow but sphenoid is
                   normal
Tessier number 7  Soft tissue involvement
                   Cleft begins from oral commissure and extends to
                   preauricular hairline
                   Pathology can range from a mild broadening of oral
                   commissure with a preauricular skin tag to a
                   complete fissure extending toward the microtic ear
                   Cleft does not extend beyond anterior border of
                   masseter muscle
                   Ipsilateral tongue, soft palate and muscles and
                   mastication can be affected
                   Parotid gland and duct can be absent
                   Facial nerve weakness may occur
                   External ear absence or microtia or only
                   preauricular skin tag can be seen depending on the
                   level of pathology
                   Middle ear can also be affected
                   Preauricular hair is usually absent in craniofacial
                   microsomia patients
                   Hairy skin between oral commissure and temporal
                   region can be seen in Teacher Collins Syndrome
                   The ipsilateral soft palate and tongue are often
                   hypoplastic
                  Skeletal involvement
                   Skeletal cleft passes through pterygomaxillary
                   junction
                   Cleft is centered in the region of temporozygomatic
                   suture according to Tessier
                   Posterior maxilla and mandibular ramus are
                   vertically hypoplastic and cause abnormal occlusal
                   plane
                   Coronoid process and condyle are often hypoplastic,
                   contributing to a posterior open bite
                   Zygomatic body is hypoplastic and displaced
                   In severe pathology, zygomatic arch represents as a
                   small stump
                   In severe form, true orbital dystopia can occur
                   Zygomatic process of temporal bone is normal
                   Cranial base is asymmetric and glenoid fossa is
                   malpositioned
                   There can be a rudimentary medial and a lateral
                   pterygoid plate
                   Sphenoid bone is usually abnormal
Tessier number 8  Soft tissue involvement
                   Cleft extends between lateral canthus and temporal
                   region
                   Hair markers can be seen along a line between
                   temporal area and lateral canthus
                   True lateral commissure coloboma with absence of
                   lateral canthus is seen
                   Epibulbar dermoids are also presented
                  Skeletal involvement
                   Cleft is located at frontozygomatic suture
                   The only support of lateral palpebral fissure is
                   the greater wing of the sphenoid bone
                   There is soft-tissue continuity of the orbit and
                   temporal fossa
Tessier number 9  Soft-tissue involvement
                   Lateral third of the upper eyelid and eyebrow are
                   abnormal
                   Lateral canthus is distorted
                   In severe pathology, microphthalmia is presented
                   Superolateral bone defect causes lateral
                   displacement of globe
                   Cleft extends to temporoparietal hair-bearing scalp
                   Temporal hairline is anteriorly displaced, and a
                   temporal hair projection is often seen
                   Nervus facialis palsy in forehead and upper eyelid
                   is common
                  Skeletal involvement
                   Bone defect extends through superolateral aspect of
                   the orbit, involving supraorbital rim and roof
                   Upper part of greater wing of sphenoid, squamous
                   portion of temporal bone, and surrounding parietal
                   bones are distorted
                   Posterolateral rotation of lateral orbital wall
                   can be seen
                   Pterygoid plates are hypoplastic
                   There may be a reduction in anteroposterior
                   dimension of anterior cranial fossa
Tessier number    Soft tissue involvement
10                 Begins at the middle third of upper eyelid and
                   eyebrow
                   Lateral eyebrow may angulate temporally
                   Palpebral fissure may be elongated with an
                   amblyopic eye displaced inferolaterally
                   In severe pathology, entire upper eyelid can be
                   absent
                   Colobomas can be seen
                   Frontal hair projection may connect temporoparietal
                   region to lateral brow
                  Skeletal involvement
                   Begins in the middle of upper orbital rim just
                   lateral to supraorbital foramen
                   A prominent mass can be seen in forehead due to
                   encephalocele
                   Orbit rotated lateroinferiorly
                   In severe disease, hypertelorism can be seen
                   Anterior cranial base is affected
Tessier number    Soft tissue involvement
11                 Medial third of upper eyelid is affected and upper
                   eyebrow is disrupted
                   Pathology can involve a tongue-like projection of
                   the frontal hairline
                  Skeletal involvement
                   A notch can be seen in the medial third of the
                   supraorbital rim
                   If cleft passes through ethmoid air cells, orbital
                   hypertelorism can be seen
                   Cranial base and sphenoid anatomy are normal
Tessier number  - Soft tissue involvement
12                 Cleft lies medial to medial canthus
                   There is a lateral displacement of the canthus
                   that causes telecanthus
                   There is no eyelid clefting
                   Aplasia of medial eyebrow can occur
                   Downward projection of paramedian frontal hairline
                   possible, but forehead skin is normal
                  Skeletal involvement
                   Passes through frontal process of maxilla
                   Transverse dimension of ethmoid cells are increased
                   and orbital hypertelorism occurs
                   Frontal and sphenoid sinuses are usually enlarged
                   Cleft is lateral to olfactory groove, so cribriform
                   plate is normal
                   Encephaloceles have not been observed in this cleft
                   Anterior and middle cranial fossae are widened
Tessier number  - Soft tissue involvement
13                 There is typically a paramedian encephalocele
                   located between nasal bone and frontal process of
                   maxilla
                   Upper eyelid and eyebrow are intact
                   Medial end of eyebrow can be displaced inferiorly
                   A V-shaped frontal hair line can be seen
                  Skeletal involvement
                   Anomaly in cribriform plate is characteristic in
                   this cleft
                   There is widening in olfactory groove
                   Cribriform plate and ethmoid sinus are enlarged,
                   thus hypertelorism can be seen
                   A paramedian encephalocele can relocate the
                   cribriform plate inferiorly leading to orbital
                   dystopia
Tessier number    Soft tissue and skeletal tissue deficiency
14                 Hypotelorism can be seen
                   A spectrum of holoprosencephalic disorders such as
                   cyclopia, ethmocephaly and cebocephaly may also
                   be seen
                   Cranium is typically microcephalic
                   Cranial base components are absent which causes
                   orbital fusion
                  Soft tissue excess
                   Hypertelorism can also be seen with number 14 cleft
                   Frontonasal encephalocele or a midline frontal
                   encephalocele can cause lateral displacement of the
                   orbits
                   Forebrain remains in a low position during
                   embryologic development
                   Glabella is flattened
                   Periorbita, eyelids and eyebrow are normal
                   Long midline projection of frontal hairline can be
                   seen
                  Skeletal tissue excess
                   Caudal frontal bone is flattened
                   While frontal sinus is nonpneumatized, sphenoid
                   sinus is over-pneumatized
                   Bifid crista galli and perpendicular plate can be
                   seen
                   Crista galli and ethmoid bone are widened and
                   caudally displaced
                   Cribriform plate is displaced inferiorly
                   Relative shortening of middle cranial fossa is
                   caused by rotation of sphenoid wings
                   Harlequin eye deformity can be seen on X-ray
Tessier number    Soft tissue involvement
30                 Pathology can range from a notch in the lower lip
                   to an entire lower lip and chin cleft
                   Anterior tongue can be bifid and attached to
                   split mandible
                   Ankyloglossia and total absence of tongue have also
                   been reported
                  Skeletal involvement
                   Cleft is located between central incisors and
                   extends to mandibular symphysis
                   Anomaly is thought to be caused by failure of
                   fusion of first branchial arch
                   If lower branchial arches are affected, neck
                   anomalies can be included in the pathology
                   Hyoid bone is absent in most cases and thyroid
                   cartilages are underformed
                   Flexion contracture of neck due to atrophia of
                   neck strap muscles
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Title Annotation:Original Article
Author:Sari, Elif
Publication:Turkish Journal of Plastic Surgery
Article Type:Report
Date:Jan 1, 2018
Words:4131
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