Indian Journal of Radiology Indian Journal of Radiology  

   Login   | Users online: 651

Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size     

 

HEAD & NECK RADIOLOGY Table of Contents   
Year : 2008  |  Volume : 18  |  Issue : 4  |  Page : 306-309
Case Report: Congenital infiltrating lipomatosis of face


1 Department of Radiology and Imaging Sciences, Sri Ramachandra Medical College and Research Institute, Sri Ramachandra University, Chennai - 600 116, India
2 Department of Plastic Surgery, Sri Ramachandra Medical College and Research Institute, Sri Ramachandra University, Chennai - 600 116, India

Click here for correspondence address and email
 

   Abstract 

Congenital infiltrating lipomatosis of the face is a rare condition characterized by diffuse fatty infiltration of the facial soft tissues. There may be muscle involvement along with associated bony hyperplasia. It is a type of lipomatous tumor that is congenital in origin; it is rare and seen usually in childhood. We recently saw an 11-year-old girl with this condition. She presented with a swelling of the right side of the face that had been present since birth; there were typical findings on plain radiographs, CT, and MRI. The patient underwent cosmetic surgery. Histopathological examination showed mature adipocytes without any capsule.

Keywords: Congenital; lipomatosis

How to cite this article:
Rajeswaran R, Murthy J, Chandrasekharan A, Joseph S. Case Report: Congenital infiltrating lipomatosis of face. Indian J Radiol Imaging 2008;18:306-9

How to cite this URL:
Rajeswaran R, Murthy J, Chandrasekharan A, Joseph S. Case Report: Congenital infiltrating lipomatosis of face. Indian J Radiol Imaging [serial online] 2008 [cited 2019 Jul 24];18:306-9. Available from: http://www.ijri.org/text.asp?2008/18/4/306/43847
Congenital infiltrating lipomatosis of the face is a rare entity characterized by collections of nonencapsulated, mature lipocytes that infiltrate local tissues, leading to craniofacial deformities. [1] These children have normal psychomotor development; the main concern is the esthetic appearance. Till date, fewer than 50 cases have been reported in the English literature. [2],[3] We report the radiological findings in one such patient.


   Case Report Top


An 11-year old girl presented with a swelling of the right side of the face since birth that had been progressively increasing in size [Figure 1]. There was no history of pain. On examination, a soft, nonpulsatile, noncompressible, ill-defined swelling was present over the face on the right side. The skin over the swelling was normal and there was no discoloration. There was no cervical lymphadenopathy.

Frontal skull radiographs [Figure 2] showed increased density of the right orbital and frontal bones. CT scan showed an ill-defined, inhomogeneous, fat-density infiltrative lesion involving the superficial as well as deep planes of the right hemiface and upper neck. The masticator, parapharyngeal, submandibular, and sublingual spaces on the right side were involved [Figure 3A]. The subcutaneous, muscular, and intermuscular planes were involved. The tongue and palate were also involved on the right side. The right orbital, maxillary, zygomatic, and frontal bones were thickened [Figure 3B] and [Figure 3C].

Subsequent MRI on a 1.5-T scanner showed a nonenhancing, ill-defined, infiltrative, inhomogeneous, hyperintense lesion (isointense to fat) on T1W images in the subcutaneous, muscular, and intermuscular planes of the face and upper neck on the right side [Figure 4A] and [Figure 4B]; the lesion appeared isointense to fat on T2W images [Figure 4C] and [Figure 4D]. The lesion involved the masticator, parapharyngeal, submandibular, and sublingual spaces on the right side. The tongue and palate on the right side were also mildly hyperintense, suggesting involvement. The lesion was hypointense on fat-suppressed images [Figure 5]. A radiological diagnosis of congenital infiltrating lipomatosis of the face was made.

The patient underwent cosmetic surgery. Histopathological examination showed mature adipocytes without any capsule. Diffuse infiltration of the intervening muscles was also identified. There was no cell atypia to suggest malignancy. These findings confirmed the radiological diagnosis.


   Discussion Top


Congenital infiltrating lipomatosis was first described by Slavin et al . [1] in 1983. The etiology is unclear. This is a rare entity [2] and is classified as a subgroup of lipoma. Benign lipomas [4] have five subgroups:

  1. Simple encapsulated lipoma
  2. Lipoma variants like angiomyolipoma
  3. Hamartomatous lesions
  4. Infiltrating or diffuse lipomatosis
  5. Benign tumor of brown fat - hibernoma


Infiltrating lipomas usually occur after the third decade of life [4] and involve subcutaneous tissue, muscles, and bones. When they have been present since birth or infancy, they are called congenital infiltrating lipomatosis and are commonly seen in the face.

Pathologically, these lesions show the following characteristics: [5] they are nonencapsulated congenital fatty tumors, they infiltrate adjacent muscles and soft tissues, there is an absence of lipoblasts and malignant characteristics, fibrous elements are present, and there is hypertrophy of subjacent bone. These lesions can be diagnosed based on the clinical and imaging features.

Plain radiographs show hypertrophy of facial bones and soft tissue swelling. USG may show adipose tissue but cannot delineate the real extent of the lesion. [2] CT scan and MRI are the modalities of choice as they can identify the fat content of the lesions and delineate their extent. CT scan shows fat-density lesions with exquisite detail. The intervening fibrous elements can give a feathery pattern or inhomogeneous character. [4],[6] MRI, with its multiplanar capability, is superior to CT. It can depict the exact extent of the lesion. The lesions are inhomogeneously hyperintense on T1W images. Muscle and bony involvement are better seen with MRI. Biopsy may not be required if the typical findings are seen on MRI. [2]

The differential diagnoses [7] include the Proteus syndrome, encephalocutaneous lipomatosis, and vascular malformations (hemangioma). Proteus syndrome is characterized by overgrowth of tissues from all three germ layers. The spectrum consists of hemihypertrophy, facial hamartomas, macrodactyly, and hyperkeratotic rugae of the soles. Encephalocutaneous lipomatosis is characterized by lipomas of the scalp and central nervous system as well as focal alopecia. Hemangiomas and other vascular malformations are compressible lesions that are easily differentiated clinically; they appear isointense on T1W and hyperintense on T2W images and can show flow voids.

The treatment modalities available are liposuction and excision. This is done for cosmetic reasons. Although these tumors are benign, the rate of recurrence is very high after surgical excision. [2],[5]

In conclusion, congenital infiltrating lipomatosis of the face is a rare benign condition occurring in childhood. It is characterized by diffuse infiltration of fat in the subcutaneous and muscle planes and bony hypertrophy. Clinical examination and imaging, especially MRI, can establish the diagnosis. Surgery is done for cosmetic purpose.

 
   References Top

1.Slavin SA, Baker DC, McCarthy JG, Muffarij A. Congenital infiltrating lipomatosis of the face: Clinicopathologic evaluation and treatment. Plast Reconstr Surg 1983;72:158-64.  Back to cited text no. 1    
2.Heymans O, Ronsmans C. Congenital infiltrating lipomatosis of the face. Eur J Plast Surg 2005;28:186-9.   Back to cited text no. 2    
3.Haloi AK, Ditchfield M, Penington A, Phillips R. Facial infiltrative lipomatosis. Pediatr Radiol 2006;36:1159-62.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Chen CM, Lo LJ, Wong HF. Congenital infiltrating lipomatosis of the face: Case report and literature review. Chang Gung Med J 2002;25:194-200.  Back to cited text no. 4  [PUBMED]  
5.Coffin CM. Adipose and myxoid tumors. Pediatric soft tissue tumors. In: A clinical, pathological, and therapeutic approach. Baltimore: Williams and Wilkins Co; 1997. p. 254.  Back to cited text no. 5    
6.Salvatore C, Antonio B, Del Vecchio W, Lanza A, Tartaro G, Giuseppe C. Giant infiltrating lipoma of the face: CT and MR Imaging Findings. AJNR Am J Neuroradiol 2003;24:283-6.   Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Padwa BL, Mulliken JB. Facial infiltrating lipomatosis. Plast Reconstr Surg 2001;108:1544-54.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]

Top
Correspondence Address:
Rangasami Rajeswaran
Department of Radiology and Imaging Sciences, Sri Ramachandra Medical College and Research Institute, Chennai - 600 116
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.43847

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3A], [Figure 3B], [Figure 3C], [Figure 4A], [Figure 4B], [Figure 4C], [Figure 4D], [Figure 5]

This article has been cited by
1 Syndromic Lipomatosis of the Head and Neck: A Review of the Literature
Kashyap Komarraju Tadisina,Karolina S. Mlynek,Lyahn K. Hwang,Hooman Riazi,Francis A. Papay,James E. Zins
Aesthetic Plastic Surgery. 2015;
[Pubmed] | [DOI]
2 Congenital infiltrating lipomatosis of the face with exophytic temporomandibular joint ankylosis: a case report and review of the literature
S Sahai,S Rajan,N Singh,H Arora
Dentomaxillofacial Radiology. 2013; 42(3): 16128745
[Pubmed] | [DOI]
3 Imaging findings of lipomatosis: a comprehensive review
Seray Akcalar,Baris Turkbey,Tuncay Hazirolan,Musturay Karcaaltincaba,Iclal Ocak,Ustun Aydingoz,Erhan Akpinar
Japanese Journal of Radiology. 2013; 31(1): 1
[Pubmed] | [DOI]
4 Congenital infiltrating lipomatosis of the face with exophytic temporomandibular joint ankylosis: A case report and review of the literature
Sahai, S. and Rajan, S. and Singh, N. and Arora, H.
Dentomaxillofacial Radiology. 2013; 42(3)
[Pubmed]
5 Infiltrating lipomatosis of the face: A case series
Urs, A.B. and Augustine, J. and Kumar, P. and Arora, S. and Aggarwal, N. and Sultana, N.
Journal of Natural Science, Biology and Medicine. 2013; 4(1): 252-257
[Pubmed]
6 Imaging findings of lipomatosis: A comprehensive review
Akcalar, S. and Turkbey, B. and Hazirolan, T. and Karcaaltincaba, M. and Ocak, I. and Aydingoz, U. and Akpinar, E.
Japanese Journal of Radiology. 2013; 31(1): 1-8
[Pubmed]
7 Congenital infiltrating lipomatosis of the face with associated involvement of the TMJ structures. Case report and review of the literature
Keramidas, T. and Lagogiannis, G. and Vlachou, V. and Katsikeris, N.
Journal of Cranio-Maxillofacial Surgery. 2012; 40(8): 750-756
[Pubmed]
8 Congenital infiltrating lipomatosis of the face with associated involvement of the TMJ structures. Case report and review of the literature
T. Keramidas,G. Lagogiannis,V. Vlachou,N. Katsikeris
Journal of Cranio-Maxillofacial Surgery. 2012; 40(8): 750
[Pubmed] | [DOI]
9 Adipose and Myxoid Tumors of Childhood and Adolescence
Cheryl M. Coffin, Rita Alaggio
Pediatric and Developmental Pathology. 2012; 15(sp1): 239
[VIEW] | [DOI]
10 Congenital Infiltrating Lipomatosis of the Face: A Case Report
Asha Mahadevappa, Vanisri H. Raghavan, Sunila Ravishankar, Gubbanna V. Manjunath
Case Reports in Pediatrics. 2012; 2012: 1
[VIEW] | [DOI]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
    Case Report
    Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed3695    
    Printed174    
    Emailed2    
    PDF Downloaded378    
    Comments [Add]    
    Cited by others 10    

Recommend this journal