| Abstract|| |
Hemifacial hyperplasia (HH) is a rare congenital condition involving enlargement of one or more tissues of the face. The treatment is surgically challenging and requires expertise. This manuscript aims to report two similar appearing HH but warranting different surgical treatment. A 19-year-old female and a 14-year-old boy presented with right facial asymmetry since birth and sought correction of the same. Surgical treatment was planned. Based on clinical history, diagnosis and imaging, HH was diagnosed. The first case was entirely a soft tissue abnormality that was treated with debulking while the second case had involvement of facial bones, necessitating surgical recontouring. The facial asymmetry was addressed. Healing was uneventful. Though the aesthetical concern and appearance of the two cases of HH were same, the treatment vastly differed. This was based on the source of asymmetry. Proper diagnosis and informed decision are a key for successful surgical outcome.
Keywords: Craniofacial deformity, aesthetics, facial asymmetry, hemifacial hyperplasia, soft tissue correction
|How to cite this article:|
Balaji S M, Balaji P. Hemifacial hypertrophy – Report of 2 cases. Indian J Dent Res 2022;33:344-7
| Background|| |
Hemifacial hyperplasia (HH) is a rare developmental craniofacial malformation exhibited by a unilateral enlargement of hard and soft tissues of the face to varying degrees. The condition may be associated with syndromes like Beckwith–Wiedemann syndrome or appear isolated. Depending on the head and neck soft tissue involvement, hemifacial hyperplasia has been traditionally classified as (1) true hemifacial hyperplasia (with unilateral increased growth of one or more facial tissues) and (2) partial hemifacial hyperplasia (growth limited to one facial structure). There are several treatment approaches discussed in the literature. However, the treatment algorithm is largely dependent on the tissues involved, functional and anatomical planes involved and most importantly, the expectation of the patient for aesthetics and functional requirements.,,, This manuscript intends to present two cases of HH and discuss the spectrum of surgical challenges posed by this enigmatic condition from aesthetics concerns.
| Case-1|| |
A 19-year-old female patient presented to the author's centre with the chief complaint of asymptomatic, diffuse swelling of her right nares, upper and lower lip region, which had been present since birth. The swelling grew gradually to its current size. Family history had no bearing. The patient was alert and had stable vital signs.
Extra-oral examination revealed facial asymmetry with diffuse swelling on the right half of the nose and adjacent right upper and lower lips [Figure 1]a. The tip of the nose deviated due to unilateral enlargement, as did the nose's prominent asymmetry [Figure 1]b. The upper and lower lips were larger on the right side of the face than on the left. When compared to the left side, the right cheek was more prominent. The right and left nasolabial folds were significantly different. On palpation, the swelling was non-tender and did not differ from its counterpart on the left side. The skin over the affected area was normal, with no signs of secondary changes. There was no evidence of lymphadenopathy in the region. There were no abnormal structures intraorally. The right tonsil, on the other hand, was significantly enlarged, causing partial airway obstruction.
|Figure 1: (a and b) Preoperative image showing diffuse swelling of right nares, upper and lower lip region, (c-e) preoperative OPG and 3D CT scan, (f) intraoperative view showing significant enlargement of right tonsil, (g) closure done in layers|
Click here to view
Imaging substantiated the observations [Figure 1]c, [Figure 1]d, [Figure 1]e. The patient sought treatment for asymmetry as well as aesthetic concerns, but refused to have tonsil enlargement addressed [Figure 1]f. The goal of this patient's surgical treatment was to correct the facial asymmetry caused by nose and lip enlargement. As there were no obvious bone size abnormalities, it was not addressed. Lower lip debulking was performed under standard general anaesthesia and with adequate preparation. A sill and wedge incision was made in the right alar, and a right alar lift was performed. Later, haemostasis was achieved, and layer closure was performed with 6-0 Ethilon [Figure 1]g. Appropriate postoperative instructions, as well as antibiotic and anti-inflammatory coverage, are provided. Healing went smoothly. The patient was followed for a year, and there was no relapse.
A 14-year-old male patient presented to the author's centre with the chief complaint of asymptomatic, diffuse swelling of the right cheek since birth. The swelling gradually grew to its current size. Patient had previously undergone liposuction at the age of eight years. Due to his appearance, the patient is experiencing social isolation. Family history made no difference. The patient was alert, and his vital signs were stable. Extra-oral examination revealed facial asymmetry with diffuse swelling on the right half of the nose and adjacent right upper and lower lips. Asymmetry was caused by unilateral enlargement of the right half of the nose [Figure 2]a. Extraordinarily, the right cheek was more prominent than the left. There was a significant difference between the right and left nasolabial folds, and the right commissure of the mouth was lower than the left. On palpation, the swelling was non-tender and did not differ from its counterpart on the left side.
|Figure 2: (a) Preoperative image showing diffuse swelling of right cheek at the age of 14 years, (b) preoperative occlusion, (c) 3D CT showing expansion of bone in the right side of zygoma and mandible, (d) preoperative OPG showing enlarged right midfacial and mandibular jaw structures, (e and f) the excess bone is removed and sculptured with care to preserve the inferior alveolar nerve in the mandible and the infraorbital nerve in the maxilla, (g) excised bony specimens, (h) postoperative image after 1 month|
Click here to view
The skin over the affected area was normal, with no signs of secondary changes. There was no evidence of lymphadenopathy in the region. The intraorally right teeth were larger than the left side [Figure 2]b. Palpation revealed a large accumulation of fat and an associated mass of muscles. The muscle-to-bone insertion was thick. A radiological examination revealed that the right midfacial and mandibular jaw structures were uniformly enlarged when compared to the left side. Furthermore, the third molars were fully formed in the right maxilla and mandible while the left side was still developing. When compared to the left side, the right posterior teeth were slightly larger. This disparity has resulted in an occlusal slant [Figure 2]c and [Figure 2]d.
The goal of the surgical treatment in this patient was to address the facial asymmetry caused by cheek enlargement by removing hard tissue in the first stage and debulking soft tissue later, if necessary. A crevicular incision was made in the right mandibular posterior teeth region, and a full thickness mucoperiosteal flap was elevated under standard general anaesthesia and adequate preparation. Along with the transposition of the mental trunk, a portion of the thick bone was reduced. Haemostasis was achieved, and the wound was closed with 3-Vicryl. On the right posterior maxilla, a crevicular incision was made, a full thickness mucoperiosteal flap was raised, and thick bone was reduced. Malar prominence was also diminished. Haemostasis was achieved, and the wound was closed with 3-Vicryl.
The anterior wall of the maxilla and the zygoma was debulked through a right trans-conjunctival incision [Figure 2]e, [Figure 2]f, [Figure 2]g. A standard lateral tarsal strip procedure was performed by suturing the periosteum at the lateral orbital wall with 4-Prolene and closing the incision in layers with 6-Vicryl to adjust the height and tension of the right lateral canthus of eye. Suction drain is installed. Appropriate postoperative instructions, as well as antibiotic and anti-inflammatory coverage, are provided. Healing went smoothly. After surgery, the patient experienced mild sagging of soft tissue and is scheduled for debulking once growth has stabilized [Figure 2]h.
| Discussion|| |
Hemifacial hyperplasia (HH) is a rare developmental entity showing asymmetric facial growth by unilateral localized enlargement to varying degrees.,,, In the present cases, clinical appearance overlaps; though in the first case, it was a pure soft tissue abnormality while in the latter it was more of a bone abnormality. The surgical rehabilitation approach widely varies due this anatomical variation. Also, the patient's aesthetic concern partly drives the extent of surgical rehabilitation.
The prevalence of HH is approximately 1 in 86,000 live births with female and right half of the face predilection. The HH becomes evident in late infancy and continues to grow in an exponential, synchronized fashion as compared to the uninvolved side. This relative asymmetry is taken forward throughout the growth until adulthood. Several aetiological factors such as heredity, chromosomal abnormalities, altered intrauterine development, endocrine dysfunction, and vascular and lymphatic abnormalities are implicated in HH. The clinical differential diagnosis of HH includes fibrous dysplasia, dyschondroplasia, congenital lymph oedema, arteriovenous aneurysm, haemangioma, lymphangioma, neurofibromatosis, and benign and malignant lesions. However, the diagnosis was arrived based on clinical imaging and supplemented by laboratory diagnosis.,,,
In the first case, the hard tissues were not much involved, while in the second case, hard tissue was extensively involved and responsible for the asymmetry. The dental apparatus was relatively normal in the first case, while molar macrodontia, precocious tooth development and eruption (of the third molar) were noted in second case. Also in the second case, the size of the right half of zygoma, maxilla and mandible was accentuated. Tooth size abnormalities and accelerated jaw growth appear to contribute to the developing malocclusion. In addition, the muscle attachment was prominent needing shaving of bone and repositioning of muscle attachment. A similar approach has been described recently. Accumulation of fat in second case contributes significantly to the asymmetry, and such lipomatosis has been described as a subtype of HH. Also, the tonsillar enlargement in the first case is very interesting and has been described recently in literature.
There is a spectrum of treatment for HH, and this is often done when physiological growth ceases, as in the first case. To prevent developing malocclusion and psychological issues, camouflage surgery would be required, as in the second case. The surgical treatment spectrum extends from simple soft tissue contouring to extensive surgeries to correct the underlying bony defect. In the latter part, reshaping the overlying soft tissues may be required as a second surgery. In the present case, we proceeded with recontouring only. In extreme cases, osteotomies and ostectomies may be required. Such cases were reported by Pollock et al., Khanna and Andrade., The latter case involved three-stage surgeries.
Literature does not provide sufficient reports on long-term results of these types of intervention. Complications include likelihood of aggravating bone remodelling, limitations in recontouring, especially in anatomically complex areas and suboptimal postoperative aesthetics. In the second case, liposuction has been unsuccessfully attempted. Liposuction requires sufficient elasticity, marginally excess skin and sufficient subcutaneous adipose tissue to be suctioned. In the second case, probably there had been only limited adipose tissue contributing to its failure. As there is a developing malocclusion as well as social isolation, partial relief by recontouring the facial bones was done. After the physiological growth ceases, the final corrective surgery would be carried out.
Both cases presented with similar aesthetic concern, but the treatment approach widely varied. The first case was an adult, requiring tissue debulking, while the second case was an adolescent, and here the first surgery was to prevent the developing malocclusion as well as to address social concerns. The complete correction would be provided after the growth ceases.
| Conclusion|| |
HH cases are rare; hence, treatment algorithm is not described and requires customization. The success rates and long-term follow-up remain under-reported. The treatment is dictated by the type of tissue involved. When hard tissues are involved, the muscle attachment becomes prominent but not the associated fat content, while when only soft tissues are involved, the associated fat also drastically increases. The surgical approach for both conditions is diverse and needs different approaches. This article presents two identically presenting cases while the treatment planning was vastly different and challenging. The challenges are presented, and the need for documentation of treatment algorithm development is highlighted.
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Dattani A, Heggie A. Hemifacial hyperplasia: A case series and review of the literature. Int J Oral Maxillofac Surg 2021;50:341-8.
Bhuta BA, Yadav A, Desai RS, Bansal SP, Chemburkar VV, Dev PV. Clinical and imaging findings of true hemifacial hyperplasia. Case Rep Dent 2013;2013:152528. doi: 10.1155/2013/152528.
Shanmugasundaram K, Vedam VK, Ganapathy S, Sathish S, Satti P. Congenital hemifacial hyperplasia: Clinical presentation and literature review. Case Rep Dent 2016;2016. doi: 10.1155/2016/5260645.
Zhu P, Li XY. Congenital hemifacial hypertrophy: A case with unilateral tonsillar hypertrophy and parapharyngeal space lipoma. Ear Nose Throat J 2022:01455613221120730. doi: 10.1177/01455613221120730.
Zissman S, Cooperman Y, Leshem D, Gur E. Progressive surgical management of hemifacialmyohyperplasia for improved functional and aesthetic results. Plastic Reconstr Surg Global Open 2020;8:e2724.
Sun R, Sun L, Li G, Sun Z, Zhao Y, Ma X, Sun C. Congenital infiltrating lipomatosis of the face: A subtype of hemifacial hyperplasia. Int J Pediatr Otorhinolaryngol 2019;125:107-12.
Nandimath SA, Rajkumar GC, Nayak T, Ashwin DP, Rudresh KB, Prashanth R. Hemifacial hypertrophy: Exploring new avenues of treatment modalities. Natl J Maxillofac Surg 2016;7:100-4.
] [Full text]
Pollock RA, Newman MH, Burdi AR, Condit DP. Congenital hemifacial hyperplasia: An embryologic hypothesis and case report. Cleft Palate J 1985;22:173-84.
Khanna JN, Andrade NN. Hemifacial hypertrophy. Report of two cases. Int J Oral Maxillofac Surg 1989;18:294-7.
Dr. S M Balaji
Director & Consultant Oral and Maxillofacial Surgeon, Balaji Dental and Craniofacial Hospital, 30, KB Dasan Road, Teynampet, Chennai - 600 018, Tamil Nadu
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]