Anesthesia in a child with Seckel syndrome

Purpose To systematically review reported cases of Seckel syndrome (SS) and point out cases associated with central nervous system (CNS) vasculopathy and provide a summary of their clinical presentation, management, and outcomes including our illustrative case. Methods We conducted a search on the MEDLINE, PubMed, Google Scholar, and Cochrane databases using the keywords “Seckel + syndrome.” We identified 127 related articles reporting 252 cases of SS apart from our case. Moreover, we searched for SS cases with CNS vasculopathies from the same databases. We identified 7 related articles reporting 7 cases of CNS vasculopathies in SS patients. Results The overall rate of CNS vasculopathy in SS patients is 3.16% (n = 8/253), where moyamoya disease (MMD) accounted for 1.97%. The mean age is 13.5 years (6–19 years), with equal gender distribution (M:F, 1:1). The most common presenting symptoms were headache and seizure followed by weakness or coma. Aneurysms were mostly located in the basilar artery, middle cerebral artery, and internal carotid artery, respectively. Regardless of the management approach, 50% of the cases sustained mild-moderate neurological deficit, 37.5% have died, and 12.5% sustained no deficit. Conclusion A high index of suspicion should be maintained in (SS) patients, and MMD should be part of the differential diagnosis. Prevalence of CNS vasculopathy in SS is 3.16% with a much higher prevalence of MMD compared to the general population. Screening for cerebral vasculopathy in SS is justifiable especially in centers that have good resources. Further data are still needed to identify the most appropriate management plan in these cases.

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Central nervous system vasculopathy and Seckel syndrome: case illustration and systematic review

Khojah

Alamoudi

Aldawsari

et al. 2021

Childs Nerv Syst

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Nasotracheal Intubation of a Patient with Seckel Syndrome by C-Mac Videolaryngoscope

Özkan¹,

Akbaş²,

Yalın³

et al. 2017

Anadolu Kliniği Tıp Bilimleri Dergisi

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Anesthetic experience of a pediatric patient with Seckel syndrome associated with pneumonia - A case report -

Kim

Song

et al. 2009

Korean J Anesthesiol

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Seckel syndrome, also called bird-headed dwarfism, is an extremely rare, inherited, autosomal recessive disorder. The patients with Seckel syndrome are characterized by growth retardation, microcephaly with mental retardation, proportional dwarfism, bird like faces, and beak-like triangular nose. A literature review reveals that they have multiple anesthetic problems such as difficult airway management, difficult venous cannulation and concomitant medical diseases. We describe our experience in anesthetic management of a 21-month-old male patient with Seckel syndrome associated with pneumonia who underwent orchiopexy for bilateral cryptochidism.

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Anesthesia in a child with Seckel syndrome

Cited by 3 publications

References 5 publications

Central nervous system vasculopathy and Seckel syndrome: case illustration and systematic review

Central nervous system vasculopathy and Seckel syndrome: case illustration and systematic review

Nasotracheal Intubation of a Patient with Seckel Syndrome by C-Mac Videolaryngoscope

Anesthetic experience of a pediatric patient with Seckel syndrome associated with pneumonia - A case report -

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