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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 4  |  Issue : 3  |  Page : 201-204

Congenital hemifacial hyperplasia with “locked jaw”: Rare anomaly with a rare presentation


Department of Plastic Surgery, Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

Date of Web Publication21-Nov-2017

Correspondence Address:
Parvathi Ravula
Flat No. 501, Sri Ganesh Siri Sampada Apt., Dwarakapuri Colony, Panjagutta, Hyderabad - 500 082, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jclpca.jclpca_66_17

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  Abstract 

Congenital hemifacial hypertrophy is a rare developmental disorder characterized by unilateral enlargement of all the facial tissues of viscerocranium involving the teeth, bone, and soft tissues. Many case reports have been reported in the literature describing the characteristic clinical and radiological features, etiology, and conditions mimicking the hemifacial hypertrophy. In general, treatment is considered for the esthetic improvement in the form of soft tissue debulking, osteotomies, or orthognathic procedures. We report a case of true hemifacial hypertrophy presented with a severe temporomandibular joint ankylosis with the jaws locked in an anterior open bite deformity and its management.

Keywords: Facial asymmetry, hemifacial hypertrophy, temporomandibular joint ankylosis


How to cite this article:
Ravula P, Rangachari S, Swathi K. Congenital hemifacial hyperplasia with “locked jaw”: Rare anomaly with a rare presentation. J Cleft Lip Palate Craniofac Anomal 2017;4, Suppl S1:201-4

How to cite this URL:
Ravula P, Rangachari S, Swathi K. Congenital hemifacial hyperplasia with “locked jaw”: Rare anomaly with a rare presentation. J Cleft Lip Palate Craniofac Anomal [serial online] 2017 [cited 2019 Aug 26];4, Suppl S1:201-4. Available from: http://www.jclpca.org/text.asp?2017/4/3/201/218883


  Introduction Top


Congenital hemifacial hyperplasia (CHFH) is a rare developmental disorder characterized by unilateral viscerocranial enlargement involving the facial soft tissues, bones, and even the teeth. Clinical examination and imaging conclude the diagnosis and treatment is indicated only for cosmetic considerations. We report a case of true CHFH with a rare presentation of temporomandibular joint (TMJ) ankylosis. A review of literature revealed that there have been no reports of such a presentation previously.


  Case Report Top


A 54-year-old female presented with inability to open or close the mouth for 2 years and history of gross enlargement of the left side of the face since birth. She had undergone multiple soft tissue surgeries for the correction of facial asymmetry, since childhood. There was no history of trauma or ear discharge. There was no family history.

Clinically, left half of the face is grossly enlarged, vertically extending from the superior orbital ridge to inferior border of the mandible and from the midline to left preauricular region laterally. The left half of the upper and lower lips were enlarged with drooping of the left oral commissure. There was a limitation of downward gaze in the left eye. The skin on the left side was coarser with multiple previous surgical scars. Malar prominence and zygomatic arch in the pre auricular region are grossly thickened and enlarged. There was no movement at the left TMJ.

On intraoral examination, there was a lack of occlusion with a fixed anterior open bite, with a gap of 2mm between the right upper and lower molars. Patient was neither able to close nor open her mouth. There was loss of teeth on the left half of both the jaws except for first premolars which were locked with each other. The maxillary and mandibular alveolar ridges, tongue, and gingiva were grossly enlarged and deformed. Left buccal mucosa was folded and pendulous [Figure 1].
Figure 1: A 54-year-old female with gross facial asymmetry, jaw fixed in anterior open bite deformity with no mobility at left temporomandibular joint, hypertrophy of the left half of the tongue

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The patient was evaluated with computed tomography (CT) angiogram and magnetic resonance imaging looking for the involvement of facial bones, soft tissue component, and relation to underlying vascular tree. Imaging revealed gross three-dimensional (3D) enlargement of the maxilla, mandible, zygoma, greater wing of sphenoid and pterygoid plates on the left side with enlarged foramina. There was bony ankylosis of left TMJ. Bony orbit on the left side was deformed; however, globe, intra-, and extra-orbital structures were normal. All the muscles of mastication and facial muscles on the left side were enlarged with fatty infiltration. The left carotid vessels were larger. Based on these clinical and radiologic findings, a diagnosis of CHFH with left TMJ ankylosis was concluded [Figure 2].
Figure 2: Computed tomography scan showing gross three-dimensional enlargement of the left side facial bones as described, coronal section showing left temporomandibular joint ankylosis

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The patient had requested for only the release of TMJ ankylosis and removal of the malar prominence to improve her visual field. 3D model aided in presurgical planning [Figure 3]. An elective tracheostomy was opted for general anesthesia. Through submandibular incision extending onto the preauricular region, debulking of soft tissue in the submandibular region and resection of about 2.5 cm of extra capscular condyle along with posterior border of ascending ramus and coronoid were performed. Through a Weber-Ferguson incision, shaving of the malar prominence and anterior zygomatic arch was done [Figure 4]. Intraoperatively, mouth opening was achieved with an interincisal distance of 4 cm. Subsequently, the patient needed partial resection of thickened alveolar margin of the mandible and maxilla to get the molar contact on the right side and also debulking of the left half of the tongue. At the end of 2 years, the patient is satisfied with adequate mouth opening and able to take soft diet [Figure 5].
Figure 3: Preoperative surgical planning on three-dimensional model as described in the text, relation to vascular structures is also highlighted

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Figure 4: Resected segments of the extra capsular condyle, coronoid process and the posterior border of the ascending ramus. Partially resected malar prominence is also shown in the picture

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Figure 5: Two-year follow-up with molar contact and mouth opening. Picture shows follow-up computed tomography scan

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  Discussion Top


Hemifacial hyperplasia is a rare congenital anomaly characterized by gross enlargement of one-half of the face involving all the tissues, seen in 1 in 86,000 live births.[1] Usually, it presents at birth with a male predominance, more commonly on the right side. It was first described by Meckel[2] in 1822. Rowe[3] in 1962 classified hemihypertrophy into (1) complex (entire half of the body), (2) simple (one or both limbs), and (3) hemifacial hypertrophy. Depending on the characteristic pattern of involvement, it is further subclassified into (a) true and (b) partial hemifacial hypertrophy. Histologically, all tissues show an increase in the number of cells rather than an increase in cell size;[4] hence, it is hyperplasia rather than hypertrophy. The asymmetry grows at a rate proportional to the uninvolved side so that the disproportion is maintained constantly and the condition stabilizes[5] at the end of adolescence. Asymmetry is not a feature of deciduous dentition but early eruption of permanent dentitions is seen. The etiology is unknown, and various theories have been proposed.[4],[6],[7],[8],[9] Different conditions that mimic CHFH should be distinguished on the basis of clinical, radiological, and laboratory findings.[10] Unilateral dental abnormalities and the tongue enlargement are the striking features of hemifacial hypertrophy.[3]

Treatment is only indicated for cosmetic considerations, in the form of debulking, osteotomies, and orthognathic procedures[11],[12],[13] when physiological growth ceases.[11]

In the present case, cause of TMJ ankylosis was not known. The possible mechanism may have been grossly enlarged alveolar ridges causing the premature contact and prolonged anterior open bite deformity leading to articular changes compounded by extra-articular mechanical obstruction; repeated surgeries associated with inflammation may have contributed too. Conventional resection of bony ankylotic block would have been very difficult and unsafe in view of gross enlargement of all the regional bones. Use of 3D models enabled understanding the anatomy and preoperative planning. Intraoperatively, the facial vessels were confused for external carotids because of its displacement and increase in size. We hence stress the role of a CT angiogram.

Aim of this presentation is to report a rare presentation of CHFH and the importance of clinical and radiological evidence establishing the diagnosis, appropriate surgical planning in view of grossly enlarged and distorted anatomy in relation to vascular structures.

CHFH is a benign disease, and there were no reports on malignant transformation in the literature. Our clinical experience of TMJ ankylosis with CHFH seems to be unique.


  Conclusion Top


Hemifacial hyperplasia creates a diagnostic dilemma and needs a thorough clinical and radiological evaluation. Appropriate evaluation and surgical planning are essential to achieve a desired result in an unfamiliar condition.

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 Top

1.
Marx RE, Stern D. Oral and Maxillofacial Pathology: A Rationale for Diagnosis and Treatment. 1st ed. Chicago, Illinois, USA: Quintessence; 2003.  Back to cited text no. 1
    
2.
Meckel JF. On the Lateral Asymmetry in the Animal Body, in Anatomical Physiological Observations and Investigations, R. Halle, Ed., p. 147, 1822.  Back to cited text no. 2
    
3.
Rowe NH. Hemifacial hypertrophy. Review of the literature and addition of four cases. Oral Surg Oral Med Oral Pathol 1962;15:572-87.  Back to cited text no. 3
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4.
Pollock RA, Newman MH, Burdi AR, Condit DP. Congenital hemifacial hyperplasia: An embryologic hypothesis and case report. Cleft Palate J 1985;22:173-84.  Back to cited text no. 4
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5.
Antoniades K, Letsis I, Karakasis D. Congenital hemifacial hyperplasia. Br J Oral Maxillofac Surg 1988;26:344-8.  Back to cited text no. 5
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6.
Islam MN, Bhattacharyya I, Ojha J, Bober K, Cohen DM, Green JG, et al. Comparison between true and partial hemifacial hypertrophy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:501-9.  Back to cited text no. 6
    
7.
Gesell A. Hemihypertrophy and twinning. Am J Med Sci 1927;173:542-55.  Back to cited text no. 7
    
8.
Noe O, Berman HH. The etiology of congenital hemihypertrophy and one case report. Arch Pediatr 1962;79:278-88.  Back to cited text no. 8
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9.
Yoshimoto H, Yano H, Kobayashi K, Hirano A, Motomura K, Ohtsuru A, et al. Increased proliferative activity of osteoblasts in congenital hemifacial hypertrophy. Plast Reconstr Surg 1998;102:1605-10.  Back to cited text no. 9
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10.
Bhuta BA, Yadav A, Desai RS, Bansal SP, Chemburkar VV, Dev PV, et al. Clinical and imaging findings of true hemifacial hyperplasia. Case Rep Dent 2013;2013:152528.  Back to cited text no. 10
    
11.
Horswell BB, Holmes AD, Barnett JS, Hookey SR. Primary hemihypertrophy of the face: Review and report of two cases. J Oral Maxillofac Surg 1987;45:217-22.  Back to cited text no. 11
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12.
Khanna JN, Andrade NN. Hemifacial hypertrophy. Report of two cases. Int J Oral Maxillofac Surg 1989;18:294-7.  Back to cited text no. 12
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13.
Hall HD. An improved method for treatment of facial asymmetry secondary to jaw deformity. J Oral Maxillofac Surg 1984;42:673-9.  Back to cited text no. 13
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    Figures

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



 

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