Limits...
Anaesthetic management of a child with panthothenate kinase-associated neurodegeneration.

Sinha R, Biyani G, Bhattacharjee S - Indian J Anaesth (2015)

Bottom Line: Panthothenate kinase-associated neurodegeneration (PKAN) (Hallervorden-Spatz disease) is a rare autosomal recessive chromosomal disorder characterised by progressive neuroaxonal dystrophy.The characteristic features include involuntary movements, rigidity, mental retardation, seizures, emaciation.The anaesthetic concerns include difficult airway, aspiration pneumonia, dehydration, and post-operative respiratory, and renal insufficiency.

View Article: PubMed Central - PubMed

Affiliation: Department of Anaesthesiology, All India Institute of Medical Sciences, New Delhi, India.

ABSTRACT
Panthothenate kinase-associated neurodegeneration (PKAN) (Hallervorden-Spatz disease) is a rare autosomal recessive chromosomal disorder characterised by progressive neuroaxonal dystrophy. The characteristic features include involuntary movements, rigidity, mental retardation, seizures, emaciation. The anaesthetic concerns include difficult airway, aspiration pneumonia, dehydration, and post-operative respiratory, and renal insufficiency. We report successful anaesthetic management of a 9-year-old intellectually disabled male child with PKAN, scheduled for ophthalmic surgery under general anaesthesia.

No MeSH data available.


Related in: MedlinePlus

Child with panthothenate kinase associated neurodegeneration presenting with right eyelid oedema and maggots
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4322101&req=5

Figure 1: Child with panthothenate kinase associated neurodegeneration presenting with right eyelid oedema and maggots

Mentions: Physical examination revealed cognitive dysfunction, facial dysmorphism, rigidity and involuntary movements of upper limbs. Airway examination showed microstomia, micrognathia, retrognathia, Modified Mallampatti class II, with normal thyromental distance and neck movements [Figure 1]. Magnetic resonance imaging (MRI) brain revealed specific pattern of hyperintensity within hypointensity of anteromedial globus pallidus with “eye-of-the-tiger” sign. Rest of the investigations, including blood chemistry were within normal limits.


Anaesthetic management of a child with panthothenate kinase-associated neurodegeneration.

Sinha R, Biyani G, Bhattacharjee S - Indian J Anaesth (2015)

Child with panthothenate kinase associated neurodegeneration presenting with right eyelid oedema and maggots
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4322101&req=5

Figure 1: Child with panthothenate kinase associated neurodegeneration presenting with right eyelid oedema and maggots
Mentions: Physical examination revealed cognitive dysfunction, facial dysmorphism, rigidity and involuntary movements of upper limbs. Airway examination showed microstomia, micrognathia, retrognathia, Modified Mallampatti class II, with normal thyromental distance and neck movements [Figure 1]. Magnetic resonance imaging (MRI) brain revealed specific pattern of hyperintensity within hypointensity of anteromedial globus pallidus with “eye-of-the-tiger” sign. Rest of the investigations, including blood chemistry were within normal limits.

Bottom Line: Panthothenate kinase-associated neurodegeneration (PKAN) (Hallervorden-Spatz disease) is a rare autosomal recessive chromosomal disorder characterised by progressive neuroaxonal dystrophy.The characteristic features include involuntary movements, rigidity, mental retardation, seizures, emaciation.The anaesthetic concerns include difficult airway, aspiration pneumonia, dehydration, and post-operative respiratory, and renal insufficiency.

View Article: PubMed Central - PubMed

Affiliation: Department of Anaesthesiology, All India Institute of Medical Sciences, New Delhi, India.

ABSTRACT
Panthothenate kinase-associated neurodegeneration (PKAN) (Hallervorden-Spatz disease) is a rare autosomal recessive chromosomal disorder characterised by progressive neuroaxonal dystrophy. The characteristic features include involuntary movements, rigidity, mental retardation, seizures, emaciation. The anaesthetic concerns include difficult airway, aspiration pneumonia, dehydration, and post-operative respiratory, and renal insufficiency. We report successful anaesthetic management of a 9-year-old intellectually disabled male child with PKAN, scheduled for ophthalmic surgery under general anaesthesia.

No MeSH data available.


Related in: MedlinePlus