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dermal_sinus

Dermal sinus

A dermal sinus is a congenital defect arising from a closure failure of the neural tube that results in different degrees of communication between the skin and the central nervous system.

It is a scaly, multi-layered channel of tissue and is lined by epithelial cells and can end anywhere between subcutaneous planes to thecal sac.

Localization

see Cranial dermal sinus.


A dermal sinus can occur anywhere from the root of the nose to the conus medullaris, and the occipital location is the second most common.

In about 66%, dermoid cysts are associated with some form of dermal sinus 1), or epidermoid cysts and less frequently with teratomas.

Pathophysiology

Embryologically, they result from a failure of the surface ectoderm and dermal elements to separate from the neuroectoderm. This process likely occurs between the 3rd and 8th weeks of gestation.

Clinical features

May appear as a dimple or a sinus (open tract), with or without hairs, usually very close to the midline, with an opening of only 1 to 2 millimeters. The surrounding skin may be normal, pigmented or distorted by an underlying mass.

The tract may end just below the skin surface or may extend to portions of the spinal cord, skull base or nasal cavity.

These tracts may be accompanied with other pathologies such as lipomyelomeningocele, myelomeningocele, split cord malformation, tethered cord, filum abnormality and inclusion tumors.

Congenital dermal sinus (CDS) and occult spinal dysraphism are suspected when a cutaneous marker overlies the spine of a newborn. CDS can have the appearance of a simple dimple and occur within the gluteal cleft without any skin markers.

Diagnosis

If the tract is seen following birth, a magnetic resonance imaging (MRI) scan should be obtained. Images may show the tract and its point of attachment. MRI also shows masses within the canal.

Complications

Treatment

Sinuses that terminate on the tip of the tailbone rarely penetrate the dura and may not need to be treated unless local infection occurs.

Indications

Sinuses above the lumbosacral region should be surgically removed.

Although approximately 25 percent of presumed sacral sinuses seen at birth will regress to a deep dimple on follow-up, all dermal sinuses should be surgically explored and treated prior to development of neurologic symptoms or signs of infection.

The results of treatment following intradural infection are never as good as when undertaken prior to infection.

Technique

Their treatment in the past has consisted of either superficial resection or intradural surgical exploration. As these lesions often transgress the dura mater and are associated with significant intradural disease, the current literature would support complete resection and intradural exploration 2).

Case reports

2015

A 15-month-old female presented with high fever, severe right hemiparesis, difficulty breathing and cranial nerve deficits. Magnetic resonance imaging (MRI) of the spine revealed a congenital dermal sinus tract at the Th6 level, an intramedullary collection extending up to the brainstem and a probable intramedullary cystic lesion. The child was operated acutely with ligation of the sinus tract, drainage of the abscess and partial removal of the intramedullary lesion. Due to abscess recurrence, she was reoperated with complete excision of the dermal sinus tract, abscess redrainage and subtotal excision of the dermoid cyst (retaining a part of its capsule). Pus culture isolated Corynebacterium species and Peptococcus species and histology of the lesion showed a dermoid cyst. Postoperatively, after an initial neurologic deterioration, she progressively improved. An MRI scan at 15 months neither showed recurrence of the collection nor regrowth of the lesion. Spinal dermal sinus tracts that remain unnoticed or untreated can result in serious complications and should be operated as soon as possible to prevent undesirable sequelae 3)

1995

A 16-month-old male infant presented with paraparesis and a high thoracic skin dimple. After myelography the child underwent complete surgical removal of the dermal sinus and of an abscessed tumor located within the cervicothoracic cord. Histological study showed that the lesion was an intramedullary epidermoid cyst. Only four descriptions have previously been published of dermal sinuses associated with intramedullary epidermoids, three of them complicated by intramedullary abscesses. This appears to be the first report in the current literature of the association of a dermal sinus and an infected intramedullary epidermoid occurring out of the confines of the lumbosacral region 4).

1)
Caldarelli M, Massimi L, Kondageski C, Di Rocco C. Intracranial midline dermoid and epidermoid cysts in children. J Neurosurg. 2004;100(5 Suppl Pediatrics):473–80.
2)
Mete M, Umur AS, Duransoy YK, Barutcuo X011f Lu M, Umur N, Gurgen SG, Selçuki M. Congenital Dermal Sinus Tract of the Spine: Experience of 16 Patients. J Child Neurol. 2014 Feb 20. [Epub ahead of print] PubMed PMID: 24563472.
3)
Papaevangelou G, Tsitsopoulos PP, Flaris N, Iliadis C, Tsonidis C. Dermal Sinus Tract of the Thoracic Spine Presenting with Intramedullary Abscess and Cranial Nerve Deficits. Pediatr Neurosurg. 2015 Oct 13. [Epub ahead of print] PubMed PMID: 26458220.
4)
Martínez-Lage JF, Esteban JA, Poza M, Casas C. Congenital dermal sinus associated with an abscessed intramedullary epidermoid cyst in a child: case report and review of the literature. Childs Nerv Syst. 1995 May;11(5):301-5. PubMed PMID: 7648573.
dermal_sinus.txt · Last modified: 2019/07/10 11:57 by administrador