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CASE REPORT
Year : 2022 | Volume
: 30
| Issue : 3 | Page : 85-87
A rare cause of facial asymmetry: Recurrent hemifacıal lipomatosis
Yasin Celal Gunes, Deniz Sozmen Ciliz, Huseyin Cetin
Department of Radiology, Ankara City Hospital, Ankara, Turkey
Correspondence Address:
Dr. Yasin Celal Gunes
Ankara Bilkent Sehir Hastanesi, Radyoloji Bölümü, Universiteler Mah, 1604. Cad. No: 9, Bilkent, Cankaya, Ankara 06800
Turkey
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/tjps.tjps_2_22
The hemifacial lipomatosis (HL) is an extremely rare pathology that results in partial hemifacial hypertrophy. This case report presents a 16-year-old male patient who underwent surgery for swelling in the right hemifacial region 11 years ago and then presented to our hospital with a diagnosis of recurrent HL in the same area.
Keywords: Hemifacial, lipomatosis, recurrent
Hemifacial hypertrophy (HFH) is a rare congenital malformation characterized by marked unilateral overgrowth of the hard and soft tissues of the face. Involvement of the orofacial structures is associated with asymmetric morphogenesis of the teeth, bones, and soft tissues.[1] Hemifacial lipomatosis (HL) is also among the factors that may cause HFH.
Case ReportThe 16-year-old male patient without any known illnesses was admitted to our hospital with a swelling in the right half of his face. In the history of the patient, it was stated that when he was 5 years old, he was operated due to the same symptom in the mentioned region. The radiology imaging was performed for facial asymmetry and swelling in the right half of the face [Figure 1].
Figure 1: (a-d) Volume and surface rendering techniques showing asymmetrically hypertrophic right side of the face (arrows)The magnetic resonance imaging (MRI) revealed that the medullary bone marrow expanded more significantly and asymmetrically with respect to the left side of the face, on the posterior of the mandible corpus and ramus mandible, the small wing of the sphenoid bone, the zygotic arch and the right maxillary sinus anterior and lateral wall. Therefore, the isointense medullary bone marrow with adipose tissue was observed in the T1W and T2W sequence.
Significant asymmetric fatty signals and thickening were seen in the masseter, temporalis, buccinators, and orbicularis oris muscles on the right. Furthermore, the thickness of adipose tissue in the deep subcutaneous tissues significantly increased in the anterior of the parotid superficial lobe on the right, in the plane up to the mandible in the buccal region on the right.
The MRI also depicted that the fat plane was asymmetrically hypertrophic in the right orbital lateral and maxillary sinus anterolateral when compared to the left one [Figure 2] and [Figure 3]. Similar findings were detected in the neck computed tomography (CT) examination, but the destruction of the bone structure was not available [Figure 4]. The defined radiological findings were primarily evaluated in favor of the right HL. Besides, the pathology result of the patient's previous operation performed in an external center was obtained and found to be compatible with the HL.
Figure 2: Coronal view of CT scan (a) revealing significant asymmetric fatty replacement right side of the face (arrow) (b and c) facial artery, zygomatic arc and maxilla enlargement (arrows). CT: Computed tomography
Figure 3: Coronal view of MRI examination revealing (a-c) asymmetric fatty signals and thickening seen in the masseter, temporalis, buccinators, and orbicularis oris muscles on the right (arrows). MRI: Magnetic resonance imaging
Figure 4: Axial view of MRI examination revealing (a-c) zygomatic arc, perimaxillary fatty tissue and muscles, right parotis superficial lobe and maxilla enlargement which effects molar and premolar teeth (arrows). MRI: Magnetic resonance imaging Discussion The HFH is a pathology that was first described by Meckel in 1822.[2] HFH, a congenital morphological anomaly leading to facial asymmetry and thus affecting the soft tissue, bone, and teeth, is divided into two subgroups, as “true” and “partial.” Bou-Haidar et al. evaluated the HL as a subtype of the partial HFH.[2] Although several different theories about the underlying mechanisms have been proposed in literature, etiopathogenesis has not been clearly resolved.[3],[4]
The HL, a diagnosis of exclusion is concluded as a result of the exclusion of other diseases that lead to the hemifacial hyperplasia, through the clinical findings, and radiological and histopathological evaluation.[5]
The key histopathological criteria in the diagnosis of lipomatosis are the infiltration into the adjacent muscle and soft tissue, the absence of the lipoblasts, the presence of the fibrous elements, an increase in the vascularization and the thickening of the muscular layer of the wall structure, and the absence of any accompanying malignancy in the nonencapsulated lipocytes.[6]
Such fat cell-derived tumors as lipoma, liposarcoma, lipoblastomatosis, and congenital overgrowth syndromes that lead to progressive growth are essential in the differential diagnosis of HFD. The difference of HL from the liposarcoma is the lack of lipoblasts and pleomorphism.
Several diseases such as hemifacial microsomia (due to the development of the first or second arc) or progressive hemifacial atrophy ( Parry-Romberg syndrome More Details) that can stimulate an asymmetry in the contralateral facial half, may give the impression of HFH in the ipsilateral facial half and thus carry out a misdiagnosis.[7]
The soft tissue and extremity hypertrophy, the presence of the capillary hemangioma and the venous malformation on the face, the Neurofibromatosis cafe au lait spots, the detection of the fibrous dysplasia accompanying the endocrine disorders in McCune Albright syndrome, the calvarial thickening accompanying the typical skin findings in Proteus Syndrome are important findings in the Klippel-Trenaunay- Weber syndrome More Details.[8]
In addition, the Cloves Syndrome and Becwith-Wideman Syndrome, which can affect many parts of the body, are also included in the differential diagnosis. Wilm's tumor, hepatoblastoma, and adrenal carcinoma are the diseases that can be observed in the syndromes with HFH. The fact that the abdominal malignancy may accompany in patients diagnosed with the HFH should be taken into consideration at the time of the diagnosis.[9]
MRI is a very useful tool in determining the nature of the lesion that causes the HFH.
In particular, it ensures that the lymphatic and vascular malformations are ruled out. The lesion area without clear boundaries depicting a suppression in the T1- and T2-weighted images isointense with fat and suppression of fat-suppressed sequences is quite typical in the affected half of the face, in patients with the HL. The MRI examination is also very convenient in terms of evaluating the facial nerve trace and showing the intracranial lesions that may accompany. However, the distinction between the liposarcoma, which differentiates well with HL, cannot be performed clearly with this examination.[8],[9],[10]
CT is prioritized in the detailed evaluation of bone involvement in these patients. Expansile changes that do not cause a destruction in the bone structure of the maxilla and mandible are observed in the affected half of the face in patients with HL. In addition, it has been reported in the literature that they may cause ankylosis through affecting the temporomandibular joint in the affected face half.[11]
There is still controversy about the treatment of the HL in the medical literature. In the past, extensive surgical excision was preferred at an early period.[12] However, nowadays, it is recommended to delay the surgical approach as much as possible and prefer more conservative treatments such as liposuction.[13]
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 initial s 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
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