Table of Contents  
Year : 2020  |  Volume : 16  |  Issue : 2  |  Page : 72-76

Tessier 4 right-sided orofacial cleft deformity with associated congenital limb anomalies − a case report and literature review

1 Department of Surgery, Lagos State University Teaching Hospital, Ikeja, Lagos, Nigeria
2 Lautech Teaching Hospital, Ogbomoso, Oyo State, Nigeria

Date of Submission02-Mar-2020
Date of Acceptance07-Aug-2020
Date of Web Publication18-Dec-2020

Correspondence Address:
Dr. S Ilori Oluwatosin
Lautech Teaching Hospital, Ogbomoso, Oyo State
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/njps.njps_3_20

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To report the case of a four year old girl with Tessier 4 right-sided orofacial cleft deformity, type I left cleft foot and hypoplastic right thumb. The patient presented with right-sided congenital orofacial defect, left foot defect and abnormally small right thumb noticed at birth. The pregnancy, labor and delivery periods were uneventful. She subsequently had repair of the orofacial cleft and the cleft foot defects. The post-operative period was uneventful. The patient is currently being followed up in the clinic. Tessier 4 cleft, though a rare orofacial cleft deformity may occasionally be associated with other congenital anomalies as it is found in the index case.

Keywords: Cleft foot, orofacial cleft, Tessier 4

How to cite this article:
Abdulwahab O. A, Fadeyibi I O, Omosebi D T, Oluwatosin S I. Tessier 4 right-sided orofacial cleft deformity with associated congenital limb anomalies − a case report and literature review. Nigerian J Plast Surg 2020;16:72-6

How to cite this URL:
Abdulwahab O. A, Fadeyibi I O, Omosebi D T, Oluwatosin S I. Tessier 4 right-sided orofacial cleft deformity with associated congenital limb anomalies − a case report and literature review. Nigerian J Plast Surg [serial online] 2020 [cited 2024 Mar 4];16:72-6. Available from:

  Introduction Top

Orofacial clefts are malformations of the face and the cranium with deficiency or excesses of tissues along an anatomical line based on embryological maldevelopment. They are among the most common malformations with incidence of 1.43 to 4.58 per 100,000 births.[1] They have variable clinical presentations with different levels of facial disfigurement. Their treatment is often complex and sometimes the evaluation of long-term result is difficult because of their low incidence. In 1976, Paul Tessier devised a classification system for craniofacial cleft wherein he assigned a specific number to the site of each malformation based on its relationship to the sagittal midline of the facial bone. The clefts are numbered 0–14 in an anticlockwise direction around the orbit. The orbit divides the face into upper and lower hemisphere and separates the cranial cleft from the facial clefts. The clefts are numbered such that the facial and cranial components of the cleft always add up to 14.[2]

Among all orofacial clefts classified by Tessier, the number 4 cleft is one of the most challenging and complex malformation.[3] It has demonstrated a wide clinical presentation ranging from unilateral simple notch to bilateral large tissue defect with bony fissure. Also, in some instances it can be associated with other congenital anomalies especially limb anomalies.[4] Surgical reconstruction of the deformity aims to restore the oro-ocular distance and close hard- and soft-tissue defects. The initial priority of management is to protect the cornea before other soft tissue and bony reconstructions are done. Thereafter, other components- the lip, nasal, alveolar, ocular and orbital deformities are further evaluated and managed.

Our search of the literature revealed that less than 100 cases of Tessier No. 4 clefts have been reported in the literature. This case report, therefore, deserves our attention as a result of the rare presentation and the rare associated limb anomalies found in the index case.

  Pathology Top

The Tessier no. 4 cleft is characterized by cleft of the lip and palate, decreased oculo-oral and oculo-alar distances, orbital dystopia, eyelid colobomas with inferiorly displaced medial canthus. This cleft may be found in two forms: complete and incomplete. The incomplete cleft is characterized by minimal soft and bony tissue deformities. In the complete form, the soft tissue cleft is located lateral to the Cupid’s bow and the philtrum, midway between the philtral ridge and the oral commissures. It continues to the cheek, lateral to the nasal ala, ascending to the medial aspect of the lower eyelid. The lacrimal sac and canalis nasolacrimalis are intact; the cleft passes lateral to these structures. The inferior canaliculus generally lies in the path of the cleft and is usually defective. The medial canthal ligament on the affected side 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 to the anterior surface of the maxilla medial to the infra-orbital foramen to terminate in the medial portion of the inferior orbital rim and floor. The eye is usually present and functional, although microphthalmos and/or anophthalmos may also occur.[3]

  Case report Top

A 4 year old girl was referred from a General Hospital to our facility on account of a right-sided facial deformity noticed from birth. The deformity involved the right lower eyelid and was associated with epiphoria and visual impairment of the right eye. She also had deformity of the left foot between the fourth and fifth digits and a poorly developed right thumb.

She was a product of term pregnancy delivered via spontaneous vertex delivery to a 19 year old woman. The pregnancy, labor and delivery histories were uneventful. There was no family history of similar deformity or other congenital anomalies.

Clinical examination showed a girl who was otherwise healthy looking with a defect between the medial and middle third of the right lower eyelid with exposure of the palpebral conjunctiva and the lacrima punctum. There was a furrow extending from the eyelid defect to just lateral to the peak of the cupid bow with associated reduced right oculo-oral distance [Figure 1]. Ocular examination revealed right eye microphthalmia, corneal opacity, coloboma of the iris and limbal dermoid. The left eye was however normal. Musculoskeletal examination revealed a cleft between the left fourth and fifth toes extending proximally towards the metatarso-tarsal joint with medial angulation of the fifth toe ([Figure 2]. The right thumb was hypoplastic.
Figure 1 Pre-operative picture of the patient showing the right sided orofacial cleft

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Figure 2 Pre-operative picture of the patient’s left cleft foot

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A diagnosis of incomplete Tessier 4 right-sided orofacial cleft with congenital ray anomalies was made.

A full blood count, electrolytes, urea, and creatinine levels were done and showed all values within normal range. The cephalometric skull X-ray done showed right maxillary hypoplasia [Figure 3]. The X-ray of the left foot revealed absent fourth metatarsal and a soft tissue defect between the fourth and fifth toes [Figure 4].
Figure 3 Anteroposterior view of the patient’s cephalometric skull X-ray

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Figure 4 The plain X-ray of the patient’s left foot

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A reconstructive surgery of the soft tissue defect was done. The margin of the right eyelid coloboma was infiltrated with 1:200,000 adrenaline solution. A V-shaped incision marking with a caudal apex was made at the margin of the eyelid coloboma. The incision was made and taken down to the embedded fibrous tissue which was excised to release the soft tissue. An increase in the oculo-nasal and oculo-oral distances were achieved. A Z-plasty was designed from the resulting skin and soft tissue defects. The flaps were interdigitated and closed with vicryl 5-0 suture. At the same sitting, the left cleft foot defect was also repaired. A V-shaped skin incison was made at the margins of the left cleft foot and dissected down to the subcutaneous tissue. Skin flaps were developed and excess skin excised. The resulting wound was thereafter sutured in layers [Figure 5].
Figure 5 Post-operative picture of the patient’s left foot

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The patient had post-operative partial wound dehiscence of the right eyelid with associated ectropion [Figure 6]. A revision surgery to correct the ectropion was done by excision of the scar and full thickness skin grafting performed [Figure 7]. She is currently being followed up in the clinic.
Figure 6 Picture showing post-operative scarring and ectropion of the right lower eyelid

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Figure 7 The patient’s picture following scar excision and FTSG

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  Discussion Top

Tessier no. 4 facial cleft is a rare congenital malformation. It has also been called medial oro-ocular cleft, medial maxillary dysplasia, and Morian II cleft.[3] In 2008, Alonso et al.[3] published the largest series of 21 patients managed in three specialized Brazillian centers over a twenty year period. Olusanya et al in 2014 also reported a case of Tessier 4 as part of a series of orofacial clefts managed in Ibadan.[5] Tshimbila in a tertiary hospital in Uganda reported seven cases of Tessier 4 orofacial cleft which were managed between 2005 and 2017.[6] In the Brazillian series, majority of the unilateral defects occurred on the left as opposed to the right sided defect found in the index case.[3] We have previously managed two other cases of Tessier 4 deformity in our centre. Our patient also had an incomplete defect which is similar to what was obtained in the majority of the patients reported by Alonso.[3] Amniotic band syndrome has been identified as the commonest congenital limb anomaly associated with oblique orofacial cleft.[4] Four of the patients reported by Alonso et al.[3] had amniotic band in the limb. The index case is however associated with a cleft foot deformity and hypoplastic thumb.

  Conclusion Top

Tessier 4 cleft, though a rare orofacial defect may also be associated with other congenital limb deformities as it is found in the index case.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kawamoto HK Jr. The kaleidoscope world of rare craniofacial clefts: order out of chaos (Tessier classification). Clin Plast Surg 1976;3:529-72.  Back to cited text no. 1
Tessier P. Anatomical classification facial, craniofacial and latero-facial clefts. J Maxillofac Surg 1976;4:69-92  Back to cited text no. 2
Alonso N, Freitas S, Oliveira G. A Tessier no. 4 facial cleft: evolution of surgical treatment in a large series of patients. Plast Reconstr Surg 2008;122:1505-13.  Back to cited text no. 3
Coady MS, Moore MH, Wallis K. Amniotic band syndrome: the association between rare craniofacial clefts and limb ring constrictions. Plast Reconstr Surg 1998;101:640Y649  Back to cited text no. 4
Olusanya AA, Michael AI, Olawoye OA, Akinmoladun VI, Ademola SA, Iyun AO et al. Distribution and morphological pattern of clefts in the craniofacial region seen in a sub-saharan tertiary hospital. Oral Surgery 2015;8:23-29.  Back to cited text no. 5
Tshimbila KJ, Hodges AM, Galiwango GW. Demographic and clinical profile ofcraniofacial clefts at Comprehensive Rehabilitation Service in Uganda. Afr J Health Issues 2018;1:6.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]


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