Anesthetic management for a patient with oculocerebrorenal (Lowe's) syndrome

Komatsu, Hisao; Sakakibara, Masatomo; Yoshimura, Yutaka; Kinoshita, Hiroyuki; Yokono, Satoshi; Ogli, Kenji

doi:10.1007/bf02482771

Cited by 1 publication

(1 citation statement)

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“…In summary, we discuss the perioperative care of patient Lowe syndrome, an X-linked disorder often associated with renal involvement with Fanconi syndrome, ocular involvement including cataracts and glaucoma, as well as central nervous system with seizures and developmental delay. To date, there are a limited number of reports in the English-language literature outlining the perioperative care of these patients (Table 1) [6,7,18,19]. Specific perioperative concerns include the potential for difficulties with airway management, bone fragility with positioning issues, electrolyte disturbances, chronic metabolic acidosis, and hypotonia (Table 2).…”

Section: Discussionmentioning

confidence: 99%

Anesthetic Management of An Adolescent With Lowe Syndrome During Posterior Spinal Fusion

et al. 2017

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Lowe syndrome, also known as oculocerebrorenal syndrome, is a rare X-linked disorder that was first described in 1952. Associated endorgan involvement includes the eye, central nervous system (CNS), and the kidney with proximal renal tubular acidosis. Life expectancy is reduced to mid-adulthood as a result of renal disease, hypotonia, and an increased susceptibility to infections. Given the multi-system involvement, surgical intervention may be required to address the sequelae of the disease process. We present a 16-year-old boy with Lowe syndrome who required anesthetic care for posterior spinal fusion for the treatment of neuromuscular scoliosis. Previous reports of anesthetic care of these patients are reviewed, end-organ involvement is discussed, and options for anesthetic care are presented.

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Section: Discussionmentioning

confidence: 99%