Anesthetic Management in Mucopolysaccharidoses

Table 2

Main associated clinical conditions and possible treatment.

Associated medical conditions	Surgical clinical conditions
Mental retardation; (MPSIH, MPSII, MPSIII) [32]	Communicating hydrocephalous → ventricular peritoneal shunt; (especially MPSI, MPSII, and MPSIIIA) [32]; AIEOP-SIMMESN/recommendations MPS I/25.10.2010
Seizures (MPSI, MPSII) [12]; AIEOP-SIMMESN/recommendations MPS I/25.10.2010	Spinal cord compression; →Decompression and rachis stabilization; (common to all MPS syndromes except MPSIII e MPSIX) [32–34]
Joint stiffness; (common to all MPS syndromes except MPS IV,; with ligamentous laxity prevalence) [32]	Skeletal deformities: kyphoscoliosis, valgus and varus limbs, and carpal tunnel; →Corrective orthopedic surgery; (especially in MPSII, MPSIV) [32, 35]
Glaucoma; (common to all MPS types) [32, 36]	Corneal opacity→corneal transplantation; (especially MPSI, MPSIV, MPSVI, and MPSVII) [32, 33, 37]; AIEOP-SIMMESN/recommendations MPS I/25.10.2010
Retina degeneration; (especially MPSI, MPSII, and MPSIII) [32, 36]
Sensor neural deafness; (common to all MPS types) [19, 32]	Chronic catarrhal otitis → transtympanic drainage; (common to all MPS types) [19, 32]; AIEOP-SIMMESN/recommendations MPS I/25.10.2010
Obstructive sleep apnea (OSA); (common to all MPS types) [32, 38]	OSA; →adenotonsillectomy, laser excision of tracheal lesions, and tracheotomy; (common to all MPS types) [6, 19, 32]; AIEOP-SIMMESN/recommendations MPS I/25.10.2010
Restrictive pneumopathy; (especially MPSI); AIEOP-SIMMESN/recommendations MPS I/25.10.2010
Systemic arterial blood hypertension; Heart failure for mitral aortic valvulopathy; Ischemic cardiomyopathy; Cardiac conduction abnormalities (BAV III°); (especially MPS I, MPSII, and MPSVI) [27]	mitral-aortic stenosis failure; →Valvular reparation/replacement; (especially MPS I, MPSII, and MPSIV) [27]
	Abdominal wall hernia(→)hernioplasty; (especially MPSI, MPSII) [39, 40]