Congenital Infiltrating Lipomatosis of the Face

Abstract

RationaleCongenital infiltrating lipomatosis of the face (CILF) is a rare disorder characterized by collections of nonencapsulated mature lipocytes that infiltrate surrounding tissues. In this article, we would report a new case of CILF, which may be one of the first few cases reported in China.Patient ConcernsAn 8-year-old boy presented with a hyperplasia of subcutaneous tissue of his left face, which had been gradually progressing since birth, resulting in a marked facial asymmetry. Then he underwent an operation of resection of the subcutaneous mass, and the postoperative pathological analysis reported a mature adipose tissue.Diagnoses and OutcomesThe diagnosis of CILF was finally made according to a comprehensive consideration of the patient's situation. We then searched different databases for studies that had investigated CILF, reviewed those literatures, and gave our summaries for such a rare disease.LessonsCongenital infiltrating lipomatosis of the face is an extremely rare disease. There is so much unknown about it, and the gradual progress and recurrence make it even harder to cure. Besides, the psychological impact on such patients must be considered. Thus, a proper collection and analysis of the reports of such a disease are very important.

Congenital infiltrating lipomatosis of the face with hyperplastic mandibular, maxillary and pterygoid bones: case report and a review of literature

“… 30 – 33 These changes in bone appear to be associated with periosteal irritation associated with the overlying mass, regional malformation of mesenchyme affecting bone and soft tissues or increased vascularity. 30 , 33 , 34 Li et al 35 in their review of 59 reported cases of CILF noted that the bony asymmetry appears to increase with age, with the adipose tissue infiltrating into surrounding tissues and causing pterygoid gland and muscles (such as pterygoid plate and massetter and temporalis muscles) involvement, as reported in our cases.…”

“… 1 , 4 – 7 The manifestation is always unilateral, and reported cases that described the site of the facial mass have shown that ~64% occur on the left. 10 In CILF patients who present for treatment at the adult stage, the condition progresses with gradual hyperplasia, while there is extensive hyperplasia in childhood patients (≤1 year of age). 11 …”

“…The major concern in the treatment of CILF is the high rate of recurrence after surgical excision although the tumor is benign, with a recent study reporting as high as 79% rate of recurrence. 1 , 8 , 35 As a result, multiple surgeries are needed for cosmetic reasons, and when necessary, osseous reduction operations of the maxilla and mandible are performed. 21 , 35 , 36 Based on the esthetic requirement of the patient, a multidisciplinary approach that involves surgeons, clinicians, dentists, psychologists and geneticists may be ideal.…”

“… 1 , 8 , 35 As a result, multiple surgeries are needed for cosmetic reasons, and when necessary, osseous reduction operations of the maxilla and mandible are performed. 21 , 35 , 36 Based on the esthetic requirement of the patient, a multidisciplinary approach that involves surgeons, clinicians, dentists, psychologists and geneticists may be ideal. 22 , 29 , 37 The high rate of recurrence may be the result of incomplete resection, as these fatty lesions are histologically benign, and as such, the value of aggressive extirpation needs to be weighed against the facial nerve damage and the likelihood of the resultant deformity.…”

Macrodystrophia Lipomatosa Extending Into the Abdominal Wall and Breast: A Case Report

“…Histopathological examination of this case could see abnormal bro-fatty tissue hyperplasia, the adipocytes were invasive, and part of muscles presented fat degeneration, which is also similar to congenital in ltrating lipomatosis (CIL) , a rare congenital disease initiating from infant [16] . Slavin et al…”