Anesthesia considerations for patients with de Barsy syndrome

Aponte, Elisabeth P.; Smith, Hugh M.; Crna, Bryan J. Wanek; Rettke, Steven R.

doi:10.1016/j.jclinane.2010.01.004

Cited by 3 publications

(3 citation statements)

References 14 publications

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“…A single investigation relating to anesthetic management of de Barsy syndrome patients was published in 2010 by authors of the present study . This article reported on 2 patients and 35 anesthetics associated with surgical and diagnostic procedures between 1968 and 2007.…”

Section: Introductionmentioning

confidence: 86%

“…De Barsy syndrome is a rare genetic syndrome characterized by cutis laxa III (ARCL3 OMIM# 219150, 614438), progeroid appearance, ophthalmic opacification, skeletal malformations, growth delays, and intellectual disability . Given the diverse array of ophthalmic and skeletal malformations, de Barsy patients require frequent surgical interventions .…”

Section: Introductionmentioning

confidence: 99%

“…The constellation of clinical manifestations of de Barsy syndrome include intrauterine growth retardation, postnatal growth delay, motor delay, cognitive impairment, hypotonia, athetoid movements, microcephaly, wormian bones, large fontanelles, facial dysmorphism, cataracts, corneal clouding, thin/wrinkled skin, easy bruising, sparse hair, joint laxity, osteopenia, and inguinal hernias . A number of de Barsy syndrome manifestations are directly relevant to perioperative patient management . Craniofacial abnormalities, cognitive delays, and skin fragility (ie, “paper thin”) are all issues requiring specific perioperative patient care consideration.…”

Section: Introductionmentioning

confidence: 99%

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Clinical implications of de Barsy syndrome

Warner

Olsen

Smith

2017

Pediatric Anesthesia

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Summary Background De Barsy syndrome is a rare, autosomal recessive syndrome characterized by cutis laxa, progeroid appearance, ophthalmic opacification, skeletal malformations, growth delays, and intellectual disability. Aims The aim of this case series is to identify the anesthetic considerations in the clinical management of patients with de Barsy syndrome. Methods A retrospective case review from 1968 to 2016 was performed at a single tertiary medical center to identify patients with de Barsy syndrome who underwent anesthesia for diagnostic and surgical procedures. We collected and analyzed the perioperative records and following data: age, sex, American Society of Anesthesiologists physical status, relevant comorbidities, surgical procedures, anesthesia management, and observed complications. Results Three patients underwent 64 unique anesthetics for a diverse collection of diagnostic and surgical procedures. An array of anesthetics and techniques were successfully used. Observations of the perioperative period found 7 episodes of intraoperative hyperthermia (>38.3°), a single difficult airway requiring fiberoptic bronchoscopic‐guided intubation, and repeatedly difficult intravenous access. Conclusion This expanded case series suggests that providers caring for patients with de Barsy syndrome should be aware of potential challenges with airway management, vascular access, and temperature monitoring.

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Section: Introductionmentioning

confidence: 86%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Clinical implications of de Barsy syndrome

Warner

Olsen

Smith

2017

Pediatric Anesthesia

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De Barsy Syndrome: Orthopedic Case report and Literature Review

Guerra-Jasso¹

2017

MOJOR

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MOJ Orthop Rheumatol 2017, 7(5): 00284OGJA, male patient of 5 years and 6 months sent for firsttime orthopedic evaluation from a telethon child rehabilitation center in the city of Irapuato, Mexico due to the antecedent of psychomotor retardation, asymmetry of the pelvic extremities and inability to stand and walk. It does not have a hereditary family history of importance, mother at the time of pregnancy 30 years of age, second pregnancy, no drug addiction during pregnancy, history of intake of folic acid, iron and vitamins, pregnancy at term 40 weeks gestation, Natural birth, weight 3000 gr, size 51 cm, do not report hypoxia at birth (does not remember APGAR scale), both graduates of the hospital at 24 hours. During development and birth it negates hyperbilirubinemia; Cephalic support at 1 year 5 months, sedestation 1 year and 8 months, currently does not achieve standing, ambulation not achieved, sporadic babbling, history of previous hospitalizations for bilateral congenital cataract surgery operated at 4 months of age, history of pneumonia Repetition, has been diagnosed with Barsy syndrome since the beginning of 2015. Physical ExaminationAthetoid movements, Macrocephaly, cephalic support, adequate hair implantation, asymmetric eyes (hypertelorism), eruptive asymmetry of the upper and lower canines, cylindrical mobile neck, short, chest thorax, with limitation to the amplexation of the Respiratory movements, unstructured kyphosis, normal cervical lordosis, no spinal dysrhaphy, soft abdomen, hepatic dullness 5 cm below the costal border, 2 cm umbilical hernia not protruded. Without ambulation, Thoracic integral extremities, normal glenohumeral joint, elbow with hyperextension, hand with integral fingers, thumb in proper position, can perform fine and thick clamp. Asymmetric pelvic extremities with bilateral proximal femoral angular deformity, barlow and ortolani negative bilateral positive piston, bilateral positive yawning sign, positive drainage test for anterior and posterior of both knees, and ankle without alterations, preserved distal mobility. Brighton criteria of 8 points (touching the ground with the palms was not possible) . In all radiographic projections, metaphyseal rarefaction, proximal femur, distal, proximal tibia, as well as proximal and distal humerus, radius and cubit are observed. Radiographic FindingsPulmonary perfusion scan: low uptake of right lower lobe and bilateral apices.

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Anaesthetic Management in a Child with Cutis Laxa for Bilateral Ureteric Reimplantation

Ramachandran¹,

Bhagya²

2021

AACC

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Cutis laxa is a rare congenital multisystem connective tissue disorder. Patients with cutis laxa can present with distinctive facial features, pulmonary emphysema and right-sided heart failure. Anaesthetic management is important because of difficult airway and respiratory/cardiac abnormalities. We have reported the preoperative evaluation and anaesthetic management of a child with Cutis laxa with right cleft lip and palate.

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Anesthesia considerations for patients with de Barsy syndrome

Cited by 3 publications

References 14 publications

Clinical implications of de Barsy syndrome

Clinical implications of de Barsy syndrome

De Barsy Syndrome: Orthopedic Case report and Literature Review

Anaesthetic Management in a Child with Cutis Laxa for Bilateral Ureteric Reimplantation

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