2011.4-16.Amniotic

                                                                                                                            article in PDF  
Our Dermatol Online. 2011; 2(4): 232
Date of submission: 31.07.2011 / acceptance: 24.08.2011
Conflicts of interest: None
 
 

 

AMNIOTIC BANDS WITH INFANTILE DIGITAL FIBROMATOSIS

ZESPÓŁ PASM OWODNIOWYCH Z DZIECIĘCĄ FIBROMATOZĄ PALCÓW

Hariharasubramony Ambika1, Chankramath Sujatha1, Srinivasiah Santhosh2

1Department of Dermatology, M.V.J. Medical College & Research Hospital, Hoskote, Bangalore, India
2Department of Pediatrics, M.V.J. Medical College & Research Hospital, Hoskote, Bangalore, India

Corresponding author: Dr. Hariharasubramony Ambika    e-mail: Dr. Hariharasubramony Ambika


 
A Newborn baby presented with deformed digits of both hands since birth. Baby was born as full term normal baby of weight 2.2kg to non consanguineous parents. No history of any medications taken or infections during antenatal period. Mother’s. HIV and VDRL tests were non-reactive. There was no family history of similar illness. On examination child was healthy and alert. Vitals were WNL. Both hands showed multiple constriction bands over fingers with loss of distal phalanx of some digits. Portion of digit distal to bands showed diffuse swelling. There was a firm nodule of about 5mm diameter was present on right ring finger and left index finger. No other cutaneous or systemic abnormalities were observed. X-ray and Ultrasound scan of digital nodules revealed fibrous nature of digital nodule. Biopsy or FNAC of nodule could not be done due to lack of consent from parents. Child was referred to orthopaedic surgeon for release of adhesion bands and child is under regular follow up for nodular lesion which was diagnosed as Infantile Digital Fibromatosis clinically and confirmed by USS. Amniotic bands are congenital constriction bands which occur due to rupture of amniotic membrane which happens usually before 12 weeks of gestation [1]. Small strands of amnion encircle developing structures commonly digits causing constriction bands (pseudoainhum) pseudosyndactyly, auto amputation or if occlusion is partial leading to distal lymphoedema [2]. Large bands can cause decreased foetal movements which can be detected in utero by USS [3]. Bands in ankle joint can cause club foot or if it is in trunk can cause scoliosis. Facial clefts in association with amniotic bands are reported [4]. No two affected babies will have exactly the same features and there is no single feature that occurs consistently in all cases. Examination of placenta may reveal strands of amnion rolled up at the base of placenta. Results of amnion rupture are external so no internal anomalies are associated. Constriction bands can also be seen due to external forces like hair or thread. It can occur secondary to diseases like palmoplantarkeratoderma or after infection, trauma and some times seen associated with Michelin tyre baby [5]. Auto amputation occurring in utero has to be differentiated from hypoplasia aplasia and acromelia. Amniotic adhesions are also seen with limb body wall complex defects which is due to different pathomechanism. Infantile Digital Fibromatosis are rare cutaneous juvenile Fibromatosis [6]. They are also called as Rey tumours as they are first described by Rey in1965. They are usually present at birth as nodules over 3rd to 5th digits or may occur after birth. Histopathologically show paranuclear eosnophilic inclusion bodies inside interlacing bundles of myofibroblasts [7]. Inclusion bodies stain red with massons trichrome stain. Electron microscopically these are actin filaments. Infantile digital Fibromatosis has to be differentiated from conventional fibromatosis. So biopsy is mandatory. Conservative treatment is recommended due to benign nature and spontaneous regression. How ever rapid growth and functional impairment may necessitate surgery. 60% shows recurrence after surgery. Intralesional steroid and bleomycin has been found effective [8]. Association of amniotic bands with infantile digital fibroma has not been reported in literature to our knowledge.
 
Figure 1. Infantile digital fibromatosis 
Figure 2. Infantile digital fibromatosis 
 
REFERENCES
1. Editorial: Amniotic bands.Br Med J. 1973; 3: 651-652.
2. Seeds JW, Cefalo RC, Herbert WN: Amniotic band syndrome. Am J Obstet Gynecol. 1982; 144: 243-248.
3. Hata T, Tanaka H, Noguchi: 3D/4D sonographic evaluation of amniotic band syndrome in early pregnancy: A supplement to 2D ultrasound. J. Obstet Gynaecol Res. 2011; 37: 656-660.
4. Das D, Das G, Gayen S, Konar A: Median facial cleft in amniotic band syndrome. Middle East Afr J Ophthalmol. 2011; 18: 192-194.
5. Goldfarb CA, Sathienkijkanchai A, Robin NH: Amniotic constriction band: a multidisciplinary assessment of etiology and clinical presentation. Bone Joint Surg Am. 2009; 91 Suppl 4: 68-75.
6. Holmes WJ, Mishra A, McArthur P: Intra-lesional steroid for the management of symptomatic Infantile Digital Fibromatosis. J Plast Reconstr Aesthet Surg. 2011; 64: 632- 637.
7. Laskin WB, Miettinen M, Fetsch JF: Infantile digital fibroma/fibromatosis: A clinicopathologic and immunohistochemical study of 69 tumors from 57 patients with long-term follow-up..Am J Surg Pathol. 2009; 33: 1- 13.
8. Failla V, Wauters O, Nikkels-Tassoudji N, Carlier A, André J, Nikkels AF: Congenital infantile digital fibromatosis: A case report and review of the literature. Rare Tumors. 2009; 1: e47.


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