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CASE REPORT |
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Intramedullary spinal cord abscess—rare complication of a congenital dermal sinus: Report of a case in a toddler
G Lakshmi Prasad
Department of Neurosurgery, Kasturba Medical College, Manipal Manipal Academy of Higher Education, Manipal, Karnataka, India
| Date of Submission | 27-Jan-2021 |
| Date of Decision | 13-Mar-2021 |
| Date of Acceptance | 11-Apr-2021 |
| Date of Web Publication | 11-Oct-2021 |
Correspondence Address:
G Lakshmi Prasad,
Department of Neurosurgery, Kasturba Medical College, Manipal Manipal Academy of Higher Education, OPD Block, Room 12, Kasturba Hospital, Manipal 576104, Karnataka.
India
 Source of Support: None, Conflict of Interest: None DOI: 10.4103/jpn.JPN_23_21
 Abstract | | |
Lumbosacral region is the commonest location for a dermal sinus (DS). The DS provides a natural pathway for infections of the deeper tissues such as the meninges or spinal cord, depending on its termination. One of the rarest and serious complications of a DS is an intramedullary spinal cord abscess. One such case has been reported here. A 2-year boy presented with high-grade fever, acute onset flaccid paraplegia and urinary retention. A L5 level DS was noted. Imaging showed a D12-L5 heterogeneously enhancing intradural lesion suggestive of an abscess. Multi-level laminectomy and drainage of abscess with removal of an inclusion epidermoid was performed. Complete recovery of all neurological functions was achieved that remained stable at 60 months follow up.
Keywords: Acute flaccid paraplegia, dermal sinus, intramedullary abscess, pediatric, spinal abscess
| Introduction | |  |
Congenital dermal sinuses (CDS) are uncommon lesions. Clinical presentation patterns may include asymptomatic ones, cutaneous findings, neurological dysfunction, or infections (meningitis or epidural/subdural/intramedullary abscess.[1],[2],[3],[4],[5] Intramedullary spinal cord abscess (IMSCA) is one of the rarest and ominous complications of CDS. Only 50 such cases have been reported.[1],[4],[6],[7],[8] In this paper, a case of a thoraco-lumbar intramedullary abscess with an infected epidermoid secondary has been reported.
| Case Illustration | | |
A 2-year-old boy was admitted with high-grade fever of 10 days duration. Two days prior, parents noticed an acute flaccid paralysis of right lower limb (LL), progressing to left LL within the next 24h. There was associated urinary retention and absent sensation below the umbilicus. Child had history of recurrent urinary infections for which he was hospitalized 6 months back. Dermal sinus was noted in the lumbar region. Neurological examination revealed LL flaccidity, paraplegia, sensory level at D10, and lax sphincters. MRI showed heterogeneously rim-enhancing intradural lesion from D12-L5 level with associated cord edema till D7 level. These lesions were hypointense on T1 and hyperintense on T2 sequences [Figure 1]A–C. The conus could not be discerned separately. Dermal sinus was seen at L5 vertebral level. D12-L5 laminectomy was performed and the sinus tract was excised [Figure 1]D. Greenish-yellow purulent content was obtained on incising the abscess wall. Myelotomy was performed over the conus to reveal pearly white epidermoid cyst contents. The cyst wall was densely adherent to the conus and the cauda equina nerve roots; hence, only partial decompression could be achieved. Culture grew Proteus mirabilis for which two antibiotics were given for 6 weeks (2 weeks intravenous plus 4 weeks oral). The child had gradual improvement in his LL power and was able to stand with support by POD 14. At 5 months follow up, he had achieved complete motor improvement with normal sphincter control that had remained stable at last follow up of 60 months. | Figure 1: Sagittal MRI showing a bulky conus and a heterogeneous ill-defined lesion extending from the D12-L5 levels, which appears predominantly isointense on T1WI (A) and iso-hyperintense on T2WI (B). The visualized cord cranially also edematous changes in the form of hyperintense signals on T2 images (B). The dermal sinus tract can be seen clearly at the L5 level (white arrow in B). Post-contrast images (C) showing heterogeneous rim enhancement with internal cystic areas. These findings were suggestive of a DST with intradural abscess. Intra-operative photograph (D) after laminectomy showing the DST (arrow) ending in dura (asterisk)
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| Discussion | | |
CDS are squamous epithelium-lined neural dysjunction disorders.[1],[9],[10] Two theories have been proposed for the occurrence of a DS. Firstly, it occurs because of an incomplete separation of cutaneous ectoderm from neuroectoderm and secondly due to persistence of the cutaneous-endomesenchymal fistula due to an error in the notochord formation with sagittal splitting of spinal canal.[4],[5],[11],[12] The lumbosacral and occipital regions are the most common locations. Coexistence with inclusion tumors (dermoid/epidermoid cysts/teratomas) is often seen.[4],[5]
IMSCA is rare and etiology differs in adults and children. In adults, hematogeneous dissemination (from urogenital, pulmonary infections) is the commonest source, while in children, CDS is the most common etiology.[1],[13] Although, the first case of IMSCA due to a DS was first reported in 1877 by Dubreuilh, only 50 cases have been reported till date.[4],[6],[7],[8],[10],[12],[13],[14],[15],[16],[17] The clinical presentation may be acute in onset consisting of fever, backache, and neurological deficits or it may present like an intramedullary neoplasm in the chronic ones.[4],[17],[18] Meningitis alone or in combination can be seen in one-thirds of cases.[4]
The imaging modality of choice is MRI. Ultrasonography is sensitive as a screening tool for detecting CDS in the infantile period in children with atypical dimples.[3],[6],[10] Spinal abscesses tend to spread over multiple levels and some authors have recommended whole spine imaging. On MRI, the cord looks expanded and the abscesses appear as a heterogeneous hypointense lesion on T1WI, hyperintense on T2WI and showing peripheral enhancement.[17],[19],[20] Prasad et al.[4] observed that the mean levels of spinal involvement was 6.1. With regards to the organisms, Staph, aureus, and E.coli  are the commonest. Polymicrobial growth is seen in around a quarter of cases.[4],[7]
A combined medical and surgical treatment is necessary for optimizing the outcome. For the CDS, a prophylactic and early excision of the sinus tract has been shown to give better outcomes in terms of preventing future infections.[3],[16] For the IMSCA, early surgical drainage and extended antibiotic treatment has been recommended. Complete excision of the sinus tract, achieving adequate neural decompression, abscess drainage with wall removal, maximal safe resection of the associated inclusion tumors, and obtaining adequate specimens for microbiological/histopathological examinations are the goals of surgery.[4]
A multilevel laminectomy/laminoplasty followed by myelotomy centered at the maximum swelling of the abscess has been advocated by most authors.[6],[13],[21] However, irrigation through a feeding tube or catheter appears to be a good option in long-segment holocord involvement.[13],[22] Further, the inclusion tumor should also be removed simultaneously and since they may be adherent to the cord, radical removal may endanger the nervous tissue. In such circumstances, a conservative debulking approach will suffice.[4],[6],[23]
Of equal importance is the prolonged broad-spectrum antibiotic therapy. This is for the treatment of the residual infection and to prevent recurrences /relapses.[1],[6] Clinicians need to be aware of the common etiological organisms ( S.aureus , E.coli, and Prot.mirabilis). A minimum of 4–6 weeks of parenteral antibiotic therapy forms the standard antibiotic regimen.[6],[16],[24] In addition, 2–3 months of oral antimicrobial therapy may be considered.[4],[8] A regimen consisting of 2 weeks parenteral and 4 weeks oral antibiotics is equally efficacious as seen in the present case.
Multiple factors have been proposed to be of importance in determining the outcomes of such cases. These include duration of presentation, timing of intervention, duration of antibiotics, type of surgery, fever, and limb weakness.[4],[25]
In comparison to the outcomes of IMSCA in adults (20%), those due to a CDS have good to excellent outcomes in almost 60% of cases. Rarely, mortality has been reported, principally due to inadequate/delay in treatment. In the present case, despite the flaccid paraplegia, factors such as early surgery (within 36h of onset of presentation), safe maximal decompression leaving behind the component adherent to the conus (thus providing higher chances of neural recovery), and young age of the patient contributed in achieving good and satisfactory recovery.
| Conclusions | | |
IMSCA due to a congenital dermal sinus is a rare occurrence. Acute presentation including fever and neurological deficits is the most common clinical presentation. Early surgical drainage with prolonged antibiotic treatment is recommended. If adequately treated, outcome is good in around two-thirds of cases. Acute flaccid weakness in any child with a dermal sinus should prompt investigating with MRI in order to provide timely treatment and improving outcomes.
Acknowledgements
None.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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