Acute Hydrocephalus in Hurler's Syndrome

Shinnar, Shlomo; Singer, Harvey S.; Valle, David

doi:10.1001/archpedi.1982.03970420080021

Cited by 8 publications

(9 citation statements)

References 5 publications

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“…Elevated ICP is a common finding across all types of MPS and is typically associated with communicating hydrocephalus (Shinnar et al, 1982; Roussey et al, 1983; Muenzer et al, 2009; Manara et al, 2011; Gupta et al, 2012). In the largest published series on Hunter syndrome (MPS-II patients: n = 36), Marana et al (2011) reported enlargement of the third ventricle in 100% of patients, subarachnoid enlargement in over 80% of patients, and craniosynostosis in 19% of patients.…”

Section: Discussionmentioning

confidence: 99%

“…In children without MPS, elevated intracranial pressure (ICP) prompts investigation for multiple etiologies, including hydrocephalus and craniosynostosis (Jane et al, 2007; Persing, 2008; Greig and Dunaway, 2015). In contrast, within the MPS spectrum, increased ICP is typically attributed to hydrocephalus or pseudotumor cerebri, despite a significant association between MPS, osteoclast dysfunction, and craniosynostosis (Goldberg et al, 1970 Fowler et al, 1975; Yatziv and Epstein, 1977; Shinnar et al, 1982; Van Aerde and Campbell, 1983; Sheridan and Johnston, 1994; Wilson et al, 2009; Greig and Dunaway, 2015). Accordingly, in MPS, elevated ICP is consistently treated by ventriculoperitoneal shunting, without investigating craniosynostosis (Shinnar et al, 1982; Roussey et al, 1983; Muenzer et al, 2009; Manara et al, 2011; Gupta et al, 2012).…”

Section: Introductionmentioning

confidence: 99%

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Markedly Elevated Intracranial Pressure Treated With Cranial Vault Expansion, Instead of CSF Shunting, in a Child With Hurler-Scheie Syndrome and Multiple Suture Craniosynostosis

Ching

Troy

Ruas

et al. 2018

The Cleft Palate Craniofacial Journal

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Despite a known association of mucopolysaccharidoses (MPS) and craniosynostosis, treatment of elevated intracranial pressure (ICP) in these patients is primarily cerebrospinal fluid (CSF) shunting. We present a unique case of Hurler-Scheie syndrome with multisuture craniosynostosis and elevated ICP, without ventriculomegaly, where elevated ICP was successfully treated with extensive cranial vault expansion and shunt placement was avoided. Patients with MPS should be evaluated for craniosynostosis, and calvarial vault expansion may be considered as a viable treatment alternative to CSF shunting for elevated ICP in select patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Markedly Elevated Intracranial Pressure Treated With Cranial Vault Expansion, Instead of CSF Shunting, in a Child With Hurler-Scheie Syndrome and Multiple Suture Craniosynostosis

Ching

Troy

Ruas

et al. 2018

The Cleft Palate Craniofacial Journal

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“…[5] Theories behind development of hydrocephalus include engorgement of the arachnoid granulations by stored material and obliteration of the subarachnoid space by infiltration of the accumulated material in the leptomeninges, both of which impede cerebrospinal fluid resorption. [4–6] Venous hypertension has been proposed as one of the possible causes responsible for ventricular and sulci enlargement in these patients. [5]…”

Section: Discussionmentioning

confidence: 99%

“…[3] Hydrocephalus with increased intracranial pressure (ICP) is a frequent complication seen in patients with Hurler's phenotype. [24–7] These patients may require emergency ventriculoperitoneal (VP) shunt surgery to reduce an increased ICP. Here, we describe successful anesthetic management in 2 such children who underwent insertion of VP shunt for acute hydrocephalus and the literature in this context has been reviewed.…”

Section: Introductionmentioning

confidence: 99%

Anesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgery

et al. 2012

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Mucopolysaccharidoses (MPSs) are a group of lysosomal storage disorder characterized by progressive multiorgan accumulation of glycosaminoglycans. Patients with MPS I (Hurler's syndrome) present as one of the most difficult airway problems to be managed by anesthesiologists. Hydrocephalus with increased intracranial pressure is a frequent complication seen in these patients. The rarity of the syndrome accounts for the small number of case reports and anecdotal information on the intracranial manifestations of this syndrome. We describe the successful anesthetic management of 2 children with Hurler's syndrome who underwent ventriculoperitoneal shunt surgery for acute hydrocephalus.

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“…3 We hypothesize that cerebrospinal fluid obstruction is related to macrophages, filled with periodic acid-Schiff-positive material, in the arachnoid villi. Only 1 patient with mucopolysaccharidosis type II syndrome has been shown to have pseudotumor cerebri.…”

Section: Mohammad Reza Mohebbi MDmentioning

confidence: 99%

Pseudotumor Cerebri in Morquio Type IV Syndrome

Chan

2009

J Child Neurol

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In recent years, the Child Neurology Society and the American Board of Psychiatry and Neurology have taken some steps forward to ease the process of becoming a child neurologist. This includes decreasing the length of the residency by combining pediatrics and child neurology programs into a 5-year package. Applicants can also join the programs after 1 year of neuroscience research and 1 year of internship in pediatrics. Despite all the attempts to encourage medical students to join child neurology programs, it seems that there is not enough understanding and cooperation between the pediatrics and child neurology programs during the recruitment process, and applicants still face a lot of problems during the application season. Most programs offer a 3-year child neurology residency and leave the first 2 years of preliminary pediatric residency to the applicant to fight for it! Application via 2 different matching systems (San Francisco Match and Electronic Residency Application Service [ERAS]) makes the process more complicated. One can match in a child neurology program and fail to secure a 2-year pediatric residency simply because the pediatric residency program is not willing to have a resident who will leave after 2 years, or because pediatrics and pediatric neurology residency programs in the same institution have different requirements for applicants. Therefore, in some cases even if the program offers a 5-year spot, the applicant could meet the requirements of 1 program but not some of the other requirements of the program; this includes the visa requirements for foreign graduates.Unlike the 1-year preliminary or transitional residencies in internal medicine and surgery, preliminary pediatric residency has no place in pediatric residency programs. Unfortunately, this leaves some candidates with no other option than breaching their ethical obligation with pediatric programs and exiting the program after 2 years. It seems logical at this time to think of a solution for the roller coaster of child neurology residency application in the United States. I trust that the Child Neurology Society and the Professors of Child Neurology can come up with a better strategy for the application process.

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Acute Hydrocephalus in Hurler's Syndrome

Cited by 8 publications

References 5 publications

Markedly Elevated Intracranial Pressure Treated With Cranial Vault Expansion, Instead of CSF Shunting, in a Child With Hurler-Scheie Syndrome and Multiple Suture Craniosynostosis

Markedly Elevated Intracranial Pressure Treated With Cranial Vault Expansion, Instead of CSF Shunting, in a Child With Hurler-Scheie Syndrome and Multiple Suture Craniosynostosis

Anesthetic management in children with Hurler′s syndrome undergoing emergency ventriculoperitoneal shunt surgery

Pseudotumor Cerebri in Morquio Type IV Syndrome

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