Congenital diffuse infiltrating facial lipomatosis
A 3-year-old boy presented with a left side facial deformity at the outpatient department. Parents reported that the swelling started as an insidious and was slowly enlarging, remarkably noticeable since birth. [] On examination a soft mass infiltrating the left cheek skin from the inferior mandibular border to the orbital rim and left labial commissure. The boundaries of the lesion were ill defined and it was non-pulsatile. The underlying facial skeleton was not involved. Clinically, the patient had a prominent, large nevus on the left cheek. The oral mucosa appeared normal. A working diagnosis of lymphangioma was made, and it was decided to observe the lesion annually.\nAs the child continued to grow, a progressive accelerated growth in the size of the left cheek was observed. Radiologically there was hypertrophy of all facial bones in the region, including zygoma, maxilla and mandible. When he was 4 years, a biopsy of the upper lip was made for a diagnosis, revealing a diffuse, poorly circumscribed, unencapsulated, lobulated, fatty infiltration consisting of mature fat cells without any cytological abnormalities. Nerve and muscular fibers and thin-walled blood vessels were observed throughout the adipose tissue. Neither lipoblasts nor signs of malignancy were found. A diagnosis of CDIL- F was made based on the clinical, radiological and histopathological features. Surgical reduction of the lipomatosis area was planned and executed. In the process most of the nevus was also removed. Only a minor portion of the nevus remained along the surgical margin [].\nTwo more surgical debulking of the lipomatosis was carried out at age of 5 years and 7 years of age []. Currently the boy is 8.5 years old [] and presented with clinical evidence of hypertrophy of the left masseter, the left medial pterygoid muscle, the left hemi-tongue, and the soft tissues of the left cheek. The left zygoma and inferior orbital rim was also hypertrophied [Figures –]. The findings were confirmed by computed tomography. No ophthalmological, neurological (left cranial nerves V and VII were intact), vascular or respiratory problems were present. Psychomotor development till date was completely normal, and no other fatty infiltration was discovered on examination of the rest of the body. The patient is being followed up.