Split cord malformation with dermal sinus, one hemicord harboring dermoid cyst, thickened filum terminale in pediatric population: Report of first case in western literature

Split cord malformation (SCM) is a term used for all double spinal cords. It represents 3.8%–5% of spinal dysraphisms. Pang et al.’s embryological theory proposes the formation of an “accessory neurenteric canal” that communicates with the yolk sac and amnion. To the authors’ knowledge, only three cases of diastematobulbia (basicranial SCM) associated with a spur or dermoid have been reported in the literature.The case patient is a newborn girl with an occipitocervical meningocele and dermal sinus associated with an anomaly of notochordal development in the transition between the medulla oblongata and the spinal cord (diastematobulbia) without a bony septum or dermoid cyst. The patient also has agenesis of the atlas and an absence of corticospinal tract decussation. This patient underwent reconstruction of the occipital meningocele and dermal sinus excision.To the authors’ knowledge, this is the first described case of type II diastematobulbia (basicranial SCM), without a dermoid cyst. The authors analyzed the embryological errors present in the case patient and considered the option of further surgical treatment depending on the evolution of the patient’s condition. At the time of this report, the patient had shown normal psychomotor development. However, this fact may only be due to the patient’s young age. Considering that after initial untethering the patient remained clinically asymptomatic, conservative and close surveillance has been and continues to be the proposed treatment.

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Growth of Intramedullary Spinal Cord Dermoid Cyst from a Congenital Thoracic Dermal Sinus Tract after Negative Screening Ultrasound Imaging

Pediatric Neurosurgery ◽

10.1159/000512580 ◽

2021 ◽

pp. 1-6

Author(s):

Liam G. Coulthard ◽

Craig Robert Vonhoff ◽

Ahmad M. Badran ◽

Thomas E. Robertson ◽

Martin J. Wood

Keyword(s):

Spinal Cord ◽

Dermoid Cyst ◽

English Literature ◽

False Negative ◽

Dermal Sinus ◽

Thoracic Spinal Cord ◽

Intramedullary Spinal Cord ◽

First Case ◽

Thoracic Spinal ◽

Screening Ultrasound

Introduction: Intramedullary thoracic dermoid cysts are rare lesions that are associated with dermal sinus tracts (DSTs). Current recommendations advocate for imaging-based screening of suspected DSTs shortly after birth to exclude associated inclusion lesions. Case Presentation: A 6-year-old male child presented with a 2-week history of progressive ataxia, lower limb weakness, and hyperreflexia. He was suspected to have a thoracic DST at birth, though initial screening ultrasound was negative for an inclusion lesion or intradural tract. On representation, MRI demonstrated a 3.9-cm intramedullary thoracic dermoid cyst causing significant spinal cord compression. Intraoperatively, a DST extending intradurally was found. The associated dermoid cyst was removed via intracapsular resection. Conclusions: Whilst dermoid cysts are presumed to progressively develop from DSTs, to our knowledge, this is the first case in English literature documenting a thoracic spinal cord intramedullary dermoid cyst following a negative screening ultrasound for a suspected DST. We use this case to highlight the false-negative rates associated with postnatal screening and advocate for early neurosurgical referral of suspected DSTs, regardless of imaging findings.

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Tetrad of Split Cord Malformation I with Neuroenteric Cyst, Dermoid Cyst, and Thickened Filum Terminale in a 2-Year-Old Child: A Case Report

Pediatric Neurosurgery ◽

10.1159/000514534 ◽

2021 ◽

pp. 1-7

Author(s):

Ajit Mishra ◽

Mohammed Nadeem ◽

Andiperumal Raj Prabhuraj ◽

Paramita Paul ◽

Dhananjaya Bhat

Keyword(s):

Differential Diagnosis ◽

Dermoid Cyst ◽

Surgical Repair ◽

Complete Recovery ◽

Split Cord Malformation ◽

Congenital Defects ◽

Filum Terminale ◽

Case Presentation ◽

Neuroenteric Cyst

Introduction: Split cord malformations (SCMs) are developmental anomalies that are associated with a number of congenital defects. However, a combination of SCM I with a neuroenteric cyst (NEC) is extremely rare, and only 11 cases have been described in the literature. To the best of authors’ knowledge, the combination of the above two with dermoid cyst and thickened filum terminale has never been reported in the literature. Case Presentation: We present a case of the above combination in a 2-year-old child who underwent microsurgical excision of all 4 pathologies and complete recovery. Conclusion: NEC and dermoid should be considered in the differential diagnosis when imaging reveals cystic pathology along with SCM. Expeditious surgical repair resulted in an outstanding functional outcome at 1-year follow-up.

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Composite split cord malformation associated with a dermal sinus tract, dermoid cyst, and epidural abscess: A case report and review of literature

Surgical Neurology International ◽

10.4103/2152-7806.180764 ◽

2016 ◽

Vol 7 (1) ◽

pp. 43 ◽

Cited By ~ 1

Author(s):

MuhammadShahzad Shamim ◽

Saad Akhtar ◽

Abdul Azeem ◽

MuhammadZubair Tahir

Keyword(s):

Case Report ◽

Dermoid Cyst ◽

Epidural Abscess ◽

Sinus Tract ◽

Split Cord Malformation ◽

Dermal Sinus ◽

Review Of Literature ◽

Dermal Sinus Tract

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Prevotella melaninogenica Meningitis and Abscess: A Complication of the Removal of a Midline Dermoid Cyst

Journal of Pediatric Infectious Diseases ◽

10.1055/s-0038-1653982 ◽

2018 ◽

Vol 15 (03) ◽

pp. 152-154

Author(s):

Megan B. Garcia ◽

Anjali N. Kunz

Keyword(s):

Dermoid Cyst ◽

Bacterial Infections ◽

Surgical Excision ◽

Sinus Tract ◽

Neural Tube Closure ◽

Intracranial Extension ◽

Dermal Sinus ◽

Oral Flora ◽

Prevotella Melaninogenica ◽

Dermal Sinus Tract

Abstract Prevotella species are gram-negative anaerobic commensal bacteria of the oropharynx, which frequently cause periodontal disease but are otherwise rarely implicated in serious bacterial infections. Cranial dermoid cysts are benign neoplasms that grow along the planes of the embryonic neural tube closure. In infants, they most commonly present in frontal locations, including periorbital, nasal, and within the anterior fontanelle. Although dermoid cysts are slow growing, usually uncomplicated, and easily treated definitively with surgical excision, cranial cysts located on the midline are associated with a higher risk for persistent dermal sinus tract with intracranial extension of the tumor. We describe a case of a 10-month-old male patient with an occipital midline dermoid cyst with intracranial extension, infected with Prevotella melaninogenica, and complicated by intracranial abscess formation and meningitis.This case highlights two unusual disease entities: the uncommon occipital location of a dermoid cyst, and complications of that cyst caused by a serious bacterial infection with a normal oral flora. We discuss the recommendation for neuroimaging prior to surgical excision of a midline dermoid cyst, given the risk for dermal sinus tract with intracranial communication. We also discuss potential mechanisms for bacterial inoculation of this cyst with Prevotella melaninogenica. This pathogen has not previously been reported as a complication of dermoid cysts.

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Cervical Dermal Sinus Associated With Intradural Dermoid Cyst

Consultant ◽

10.25270/con.2020.07.00010 ◽

2020 ◽

Author(s):

Aviva Jaari Whelan ◽

James N. Crooks

Keyword(s):

Dermoid Cyst ◽

Dermal Sinus

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Fluorescent in situ hybridization (FISH): A useful diagnostic tool for childhood conjunctival melanoma

European Journal of Ophthalmology ◽

10.1177/11206721211030775 ◽

2021 ◽

pp. 112067212110307

Author(s):

Raquel María Moral ◽

Carlos Monteagudo ◽

Javier Muriel ◽

Lucía Moreno ◽

Ana María Peiró

Keyword(s):

In Situ Hybridization ◽

Fluorescent In Situ Hybridization ◽

Pediatric Population ◽

Diagnostic Technique ◽

Histopathological Diagnosis ◽

Fish Analysis ◽

Conjunctival Melanoma ◽

Adequate Treatment ◽

First Case

Introduction: Conjunctival melanoma is extremely rare in children and has low rates of resolution. Definitive histopathological diagnosis based exclusively on microscopic findings is sometimes difficult. Thus, early diagnosis and adequate treatment are essential to improve clinical outcomes. Clinical case: We present the first case in which the fluorescent in situ hybridization (FISH) diagnostic technique was applied to a 10-year-old boy initially suspected of having amelanotic nevi in his right eye. Based on the 65% of tumor cells with 11q13 (CCND1) copy number gain and 33% with 6p25 (RREB1) gain as measured by the FISH analysis, and on supporting histopathological findings, the diagnosis of conjunctival melanoma could be made. Following a larger re-excision, adjuvant therapy with Mitomycin C (MMC), cryotherapy and an amniotic membrane graft, the patient has remained disease-free during 9 years of long-term follow-up. Case discussion: Every ophthalmologist should remember to consider and not forget the possibility of using FISH analyses during the differential diagnosis of any suspicious conjunctival lesions. Genetic techniques, such as FISH, have led to great advances in the classification of ambiguous lesions. Evidence-based guidelines for diagnosing conjunctival melanoma in the pediatric population are needed to determine the most appropriate strategy for this age group.

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Congenital Dermal Sinus Elements in Each Tethering Stalk of Coexisting Thoracic Limited Dorsal Myeloschisis and Retained Medullary Cord

Pediatric Neurosurgery ◽

10.1159/000511876 ◽

2020 ◽

pp. 1-8

Author(s):

Takato Morioka ◽

Nobuya Murakami ◽

Masako Ichiyama ◽

Takeshi Kusuda ◽

Satoshi O. Suzuki

Keyword(s):

Present Article ◽

Dermoid Cyst ◽

Spinal Dysraphism ◽

Conus Medullaris ◽

Dermal Sinus ◽

Case Presentation ◽

Upper Tract ◽

Secondary Neurulation ◽

Extradural Space ◽

Congenital Dermal Sinus

Introduction: The embryogenesis of limited dorsal myeloschisis (LDM) likely involves impaired disjunction between the cutaneous and neural ectoderms during primary neurulation. Because LDM and congenital dermal sinus (CDS) have a shared origin in this regard, CDS elements can be found in the LDM stalk. Retained medullary cord (RMC) is a closed spinal dysraphism involving a robust, elongated, cord-like structure extending from the conus medullaris to the dural cul-de-sac. Because the RMC is assumed to be caused by impaired secondary neurulation, concurrent RMC and CDS cannot be explained embryologically. In the present article, we report a case in which CDS elements were noted in each tethering stalk of a coexisting LDM and RMC. Case Presentation: A 2.5-month-old boy with left clubfoot and frequent urinary and fecal leakage had 2 tethering tracts. The upper tract, which ran from the thoracic tail-like cutaneous appendage, had CDS elements in the extradural stalk and a tiny dermoid cyst in the intradural stalk immediately after the dural entry. In the lower tract, which ran from the lumbosacral dimple, the CDS as an extradural stalk continued to the RMC at the dural cul-de-sac. Both stalks were entirely resected through skip laminotomy/laminectomy at 1 stage to untether the cord and resect the CDS elements. Conclusion: Surgeons should be aware that CDS elements, in addition to LDM, may coexist with RMC that extends out to the extradural space.

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A Case of COVID-19-Related Thrombocytopenia and Leukopenia in an Adolescent with Mild Symptoms

Children ◽

10.3390/children8060509 ◽

2021 ◽

Vol 8 (6) ◽

pp. 509

Author(s):

Lydia Kossiva ◽

Athanasios Thirios ◽

Eleni Panagouli ◽

Alexandros Panos ◽

Stavroula Lampidi ◽

...

Keyword(s):

Bone Marrow ◽

Viral Infection ◽

Nasal Congestion ◽

Pediatric Population ◽

Clinical Manifestations ◽

Bone Marrow Aspiration ◽

White Blood Count ◽

Chronic Obstructive ◽

First Case ◽

The Impact

Since the beginning of the COVID-19 pandemic, there have been numerous reports and reviews on the complications caused by the disease, analyzing the acute and chronic consequences. The main symptoms of SARS-CoV-2 are dry cough, fever, and fatigue. COVID-19 appears to affect all systems, including renal, cardiovascular, circulatory, and respiratory systems, causing chronic obstructive pulmonary disease. We report on a 14-year-old male adolescent, who presented with thrombocytopenia (platelet count 92 × 109 /L) and leukopenia (white blood count 4.2 × 103 /μL) that was observed two months ago. Ten days before the first blood test, a viral infection with nasal congestion and runny nose was reported, without other accompanying symptoms. Viral antibodies screening revealed positivity for all the three specific COVID-19 antibodies. Further haematological evaluation with bone marrow aspiration revealed non-specific dysplastic features of the red cell and megakaryocyte progenitors. Although haematological alterations due to COVID-19 infection are available from adult patients’ reports, the effect of COVID-19 infection in the pediatric population is underestimated and this is the first case with such haematological involvement. Noteworthy, in the current case, the impact of the COVID-19 infection was not related to the severity of the disease, as the symptoms were mild. In similar cases, bone marrow aspiration would not be performed as a part of routine work-up. Thus, it is important when evaluating pediatric patients with COVID-19 infection to search and report those alterations in order to better understand the impact and the spectrum of clinical manifestations of the specific viral infection in children and adolescents.

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