Annals of Clinical Case Reports Case Summary · dermoid, and teratoma), split-cord malformations and tethered spinal cords[3]. CDS may become symptomatic because of either infection

Remedy Publications LLC., | http://anncaserep.com/

Annals of Clinical Case Reports

2019 | Volume 4 | Article 15981

IntroductionWalker and Busy first coined the term 'congenital dermal sinus' in 1934; a rare form of spinal

dysraphism occurring during early embryonic life due to incomplete separation of neural ectoderm from epithelial ectoderm. This tract can be located in or near the midline anywhere from the coccyx to the cervical region, with 75% of the tracts observed in the lumbar and lumbo sacral areas. The tract is most often unique, associated with local skin marker.

Case PresentationA six years old female child was brought to us with the complaint of an abnormal opening in

the right posterior lumbar region since infancy noticed by the mother while giving bath (Figure 1A). There was no history of any discharge or back pain from the pit. Child was evaluated elsewhere with ultrasonography which revealed sinus tract in the posterior lumbar region without any communication to the spinal canal. Child was referred to our institute for further management. We evaluated further with Magnetic Resonance Imaging (MRI) which revealed, right posterior lumbar region showing subcutaneous sinus tract measuring 3.5 cm in length and 2 mm maximum thickness in the right posterior abdominal wall, adjacent to iliac crest region without any intra peritoneal or spinal connection (Figure 2). Underlying muscles were normal. External opening was noted posterolaterally in the subcutaneous plane with tract extending anteromedially and ending in the right posterior paraspinal muscle. Secondary tract was noted anteromedially extending along quadratus lumborum muscle. No intra abdominal or spinal or thecal connection was noted.

Per operatively, we found sinus tract measuring 4 cm × 3 mm, in the subcutaneous plane, ending blindly just above iliac crest (Figure 1B,1C). Surgical excision was done under general anesthesia uneventfully. Histopathology sections showed skin with subcutaneous tissue revealing sinus tract lined by squamous epithelium with the dermis showing a tiny cyst lined by stratified squamous epithelium surrounded by lymphocytes, plasma cells, neutrophils, eosinophils, histiocytes and multinucleate giant cells. Many hair shafts, sebaceous glands and sweat glands were also present in the dermis (Figure 1D,1E). Child is doing well at 8 months follow up.

DiscussionTrue incidence of congenital dermal sinus is not known; it is estimated to be 1in every 2500 live

births [1,2]. In order of frequency, it is localized most frequently in the lumbo sacral area (41%), followed by the thoracic (10%) and cervical (1%) areas [1,2]. CDS can also be found with other forms of dysraphism with or without any associated cutaneous markers. The CDS tracts can be associated with several pathological findings; including inclusion tumors (for example, epidermoid, dermoid, and teratoma), split-cord malformations and tethered spinal cords [3]. CDS may become symptomatic because of either infection or associated lesions.

An Unusual Variant of Congenital Dermal Sinus - A Rare Case Report

OPEN ACCESS

*Correspondence:Jayalaxmi Shripati Aihole,

Department of Paediatric Surgery, Indira Gandhi Institute of Child Health,

Bengaluru, Karnataka, India;E-mail: [email protected]

Received Date: 18 Jan 2019 Accepted Date: 11 Feb 2019

Published Date: 13 Feb 2019

Citation: Aihole JS. An Unusual Variant of

Congenital Dermal Sinus - A Rare Case Report. Ann Clin Case Rep. 2019; 4:

1598.ISSN: 2474-1655

Copyright © 2019 Jayalaxmi S Aihole. This is an open access

article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution,

and reproduction in any medium, provided the original work is properly

cited.

Case SummaryPublished: 13 Feb, 2019

AbstractCongenital Dermal Sinus tracts (CDS) are most commonly found over the midline and may or may not communicate with the central nervous system. We present a 6-year-old girl with a pit in the right posterior lumbar region noted since birth without any discharge. This location is quite unusual, and our report highlights the variability of congenital dermal sinus tract presentation and raises awareness that, the lesions prone to be having a connection with the central nervous system can present quite laterally.

Keywords: Spinal dermal sinus tracts; Spinal dysraphism; Dermal sinus

Jayalaxmi Shripati Aihole*

Department of Paediatric Surgery, Indira Gandhi Institute of Child Health, India

Jayalaxmi S Aihole Annals of Clinical Case Reports - Paediatric Surgery

Remedy Publications LLC., | http://anncaserep.com/ 2019 | Volume 4 | Article 15982

Magnetic Resonance Imaging (MRI) is the most specific imaging method to evaluate the spinal canal in neonates and in children, allowing diagnostic confirmation and a more detailed analysis of dysraphism [2-4]. A CDS is lined by a stratified squamous epithelium and can be found in or near the midline, anywhere from the nasion to the coccyx [4].

CDS has both dermal and epidermal elements, since the primitive ectoderm has the capacity to form all elements of the skin. The epidermal component consists of a well differentiated stratified squamous epithelium that of the dermis includes hair follicles, sebaceous glands and sweat glands [4].

Figure 1: 1A: Clinical appearance of right posterior lumbar pit. 1B: Intra operative appearance-with probe in the sinus tract. 1C: Excision of sinus tract. 1D: HPE PICTURE-low power view – dermal cyst lined by stratified squamous epithelium with lumen containing keratin. 1E: HPE PICTURE-low power view showing CDS lined by Stratified squamous epithelium with dermis containing dermal appendages. Lumen filled with keratinous material.

Figure 2: MRI Pictures.2A: Coronal section-Curvilinear red arrow showing the opening of sinus tract at the skin surface. 2B: T2 axial section – sinus tract in the subcutaneous plane ending far away from the spine. 2C: Sagittal section curvilinear red arrow -the sinus tract seen in the subcutaneous plane just above the level of iliac crest. 2D: Coronal T2 image showing the sinus tract seen extending in the subcutaneous plane not communicating with spine.

We encountered a six year female child presenting with asymptomatic cutaneous pit at lumbar region, which on evaluation found to dermal sinus tract without any connection to spinal axis. Excision was done uneventfully with histology confirmed to be, the congenital dermal sinus tract lined by stratified squamous epithelium.

Laterally placed spinal dermal sinus is exceedingly rare. Since there are only very few case reports of laterally placed sinus tracts in the literature: Carrillo et al reported two cases, Nishimon one, khansa abderrehman reported one and yet another six cases by others, but they were all communicating with spinal canal presenting with symptoms [5-7].

Our case was unique with asymptomatic pit in the posterior lumbar region ending blindly in the muscular plane far away from spine and iliac crest.

The possible explanation in our case could be that the congenital dermal sinus which develops from primitive neuroectoderm might have ‘pinched off’ or severed off its connections from its origin with the in growth of dorsal body wall mesenchyma carrying it laterally hence ‘sequestered’ and ending blindly laterally without having any communication to the spine.

There are no published reports of CDS in children, presenting laterally in the lumbar region, without any symptoms, not communicating with spine, in the English literature so far. In view of its rarity, we are reporting this case.

AcknowledgementAuthors would like to thank the Director, pediatric surgical

colleagues, pediatric anesthetists, OT staffs, Radiologists of IGICH, Bengaluru, Karnataka, India.

References1. Wang KC, Yang HJ, Oh CW, Kim HJ, Cho BK. Spinal congenital dermal

sinus- experience of 5 cases over a period of 10 years. J Korean Med Sci. 1993;8:341-7.

2. Ikwueke I, Bandara S, Fishman SJ, Vargas SO. Congenital dermal sinus tract in the lateral buttock: unusual presentation of a typically midline lesion. J Pediatr Surg. 2008;43(6):1200-2.

3. Cox EM, Knudson KE, Manjila S, Cohen AR. Unusual presentation of congenital dermal sinus: tethered spinal cord with intradural epidermoid and dual paramedian cutaneous ostia. Neurosurg Focus. 2012;33(4):E5.

4. Frank GR, Rubin LG. Mixed meningitis with bacteroides ovatus caused by an occult congenital dermal sinus. Pediatr Infect Dis J. 1989;8(6):401-3.

5. Carrillo R, Carreira LM, Prada JJ, Rosas C. Lateral congenital spinal dermal sinus. A new clinical entity. Childs Nerv Syst. 1985;1(4):238-40.

6. Nishimon M, Shimizu Y, Ueno M, Iwanami A. Late-onset congenital lateral dermal sinus tract. BMJ Case Rep. 2014;22:2014.

7. Abderrahmen K, Kallel J, Jemel H. Unusual case of multiple lateral spinal dermal sinuses in a child. Pediatr Neonatol. 2017;58(2):189-90.