Imaging of Horner syndrome in pediatrics: association with neuroblastoma
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REVIEW
Imaging of Horner syndrome in pediatrics: association with neuroblastoma Hedieh Khalatbari 1
&
Gisele E. Ishak 1
Received: 23 April 2020 / Revised: 23 June 2020 / Accepted: 4 August 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Neuroblastoma is the most common neoplasm associated with pediatric Horner syndrome. The laboratory and imaging evaluation of isolated pediatric Horner syndrome is controversial. We review the literature published in the last several decades and present the rationale for the imaging work-up in this patient cohort. Keywords Anisocoria . Children . Horner syndrome . Magnetic resonance imaging . Neuroblastoma . Oculosympathetic pathway . Ptosis
Introduction Horner syndrome presents with the classical triad of ipsilateral miosis, ptosis and anhidrosis. In patients presenting with unequal size of the pupils (anisocoria), the clinician needs to determine whether the anisocoria is physiological or pathological, and if pathological which pupil, the small miotic or the large mydriatic pupil, is abnormal. The two main etiologies of anisocoria that require imaging work-up are impaired pupillary constriction and impaired pupillary dilation due to lesions in the parasympathetic and sympathetic pathways, respectively [1, 2]. Clinical exam and pharmacological tests aid in the differentiation [3, 4]. Increased discrepancy in the size of the pupils in the dark signifies that the small miotic pupil is abnormal. Pharmacological testing, with apraclonidine or cocaine eye drops, can help differentiate Horner syndrome from physiological anisocoria. In this article, we will review the lesions in the oculosympathetic pathway that result in pediatric Horner syndrome. We will also review the literature regarding the imaging investigation of isolated Horner
Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00247-020-04796-w) contains supplementary material, which is available to authorized users. * Hedieh Khalatbari [email protected] 1
Department of Radiology, University of Washington School of Medicine, Seattle Children’s Hospital, 4800 Sand Point Way NE, Seattle, WA 98105, USA
syndrome and the less well-known association of intraabdominal neuroblastoma with Horner syndrome.
Clinical presentation and evaluation Pediatric Horner syndrome has an estimated incidence of 1.42 per 100,000 children [5]. Horner syndrome (oculosympathetic paresis) consists of the triad of ipsilateral miosis, ptosis and facial anhidrosis [6]. There may also be an upside-down ptosis of the lower eyelid that further narrows the palpebral fissure and results in apparent enophthalmos [7]. Symptoms are rarely intermittent [7, 8]. Iris heterochromia may be present in congenital cases, but rarely in long-standing Horner syndrome due to the lack of the normal sympathetic input necessary for melanocyte development [7, 9–13]. Horner syndrome may be alternating (i.e. from one side to the other) with cervical spinal cord lesions [7]. Lack of fac
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