Limits...
I-gel assisted fiberoptic intubation in a child with Morquio's syndrome.

Dhanger S, Adinarayanan S, Vinayagam S, Kumar MP - Saudi J Anaesth (2015 Apr-Jun)

Bottom Line: Morquio's syndrome, also known as mucopolysaccharidosis type IV is an autosomal recessive disorder, caused by deficiency of n-acetylgalactosamine-6-sulphate.Anesthetic management of this syndrome is a great challenge, especially in pediatric age group as "cannot ventilate, cannot intubate" scenario can be encountered by anesthesiologist due to the possibility of total airway collapse.Herewith, we are reporting a case of child with Morquio's syndrome where I-gel assisted fiber-optic intubation was used for safe endotracheal intubation.

View Article: PubMed Central - PubMed

Affiliation: Department of Anaesthesiology and Critical Care, Indira Gandhi Medical College and Research Institute, Puducherry, India.

ABSTRACT
Morquio's syndrome, also known as mucopolysaccharidosis type IV is an autosomal recessive disorder, caused by deficiency of n-acetylgalactosamine-6-sulphate. Anesthetic management of this syndrome is a great challenge, especially in pediatric age group as "cannot ventilate, cannot intubate" scenario can be encountered by anesthesiologist due to the possibility of total airway collapse. Herewith, we are reporting a case of child with Morquio's syndrome where I-gel assisted fiber-optic intubation was used for safe endotracheal intubation.

No MeSH data available.


Related in: MedlinePlus

Magnetic resonance imaging cervical spine sagittal section, showing cord compression at C1 vertebrae with loss of cerebrospinal fluid space
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4374234&req=5

Figure 1: Magnetic resonance imaging cervical spine sagittal section, showing cord compression at C1 vertebrae with loss of cerebrospinal fluid space

Mentions: Hemogram, urine and liver function test were within normal limits. Echocardiography showed mild aortic regurgitation with no wall motion abnormality. Chest X-ray revealed wide ribs, foreshortened clavicle and cevico-dorsal scoliosis. Spiral computed tomography cervical-spine revealed atlanto axial subluxation and rotation of C2 over C1. Magnetic resonance imaging spine showed cord compression at C1 with the loss of cerebrospinal fluid space and signal changes in cord below the compression [Figure 1]. Skeletal radiograph findings were as follows: X-ray skull revealed normal calvarium with j-shaped pituitary fossa; X-ray hands showed flexion deformity at interphalangeal joints and wrist joint; X-ray pelvis showed bilateral wide iliac blades with obliquely placed acetabular roof and coxa vera deformity of femur.


I-gel assisted fiberoptic intubation in a child with Morquio's syndrome.

Dhanger S, Adinarayanan S, Vinayagam S, Kumar MP - Saudi J Anaesth (2015 Apr-Jun)

Magnetic resonance imaging cervical spine sagittal section, showing cord compression at C1 vertebrae with loss of cerebrospinal fluid space
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4374234&req=5

Figure 1: Magnetic resonance imaging cervical spine sagittal section, showing cord compression at C1 vertebrae with loss of cerebrospinal fluid space
Mentions: Hemogram, urine and liver function test were within normal limits. Echocardiography showed mild aortic regurgitation with no wall motion abnormality. Chest X-ray revealed wide ribs, foreshortened clavicle and cevico-dorsal scoliosis. Spiral computed tomography cervical-spine revealed atlanto axial subluxation and rotation of C2 over C1. Magnetic resonance imaging spine showed cord compression at C1 with the loss of cerebrospinal fluid space and signal changes in cord below the compression [Figure 1]. Skeletal radiograph findings were as follows: X-ray skull revealed normal calvarium with j-shaped pituitary fossa; X-ray hands showed flexion deformity at interphalangeal joints and wrist joint; X-ray pelvis showed bilateral wide iliac blades with obliquely placed acetabular roof and coxa vera deformity of femur.

Bottom Line: Morquio's syndrome, also known as mucopolysaccharidosis type IV is an autosomal recessive disorder, caused by deficiency of n-acetylgalactosamine-6-sulphate.Anesthetic management of this syndrome is a great challenge, especially in pediatric age group as "cannot ventilate, cannot intubate" scenario can be encountered by anesthesiologist due to the possibility of total airway collapse.Herewith, we are reporting a case of child with Morquio's syndrome where I-gel assisted fiber-optic intubation was used for safe endotracheal intubation.

View Article: PubMed Central - PubMed

Affiliation: Department of Anaesthesiology and Critical Care, Indira Gandhi Medical College and Research Institute, Puducherry, India.

ABSTRACT
Morquio's syndrome, also known as mucopolysaccharidosis type IV is an autosomal recessive disorder, caused by deficiency of n-acetylgalactosamine-6-sulphate. Anesthetic management of this syndrome is a great challenge, especially in pediatric age group as "cannot ventilate, cannot intubate" scenario can be encountered by anesthesiologist due to the possibility of total airway collapse. Herewith, we are reporting a case of child with Morquio's syndrome where I-gel assisted fiber-optic intubation was used for safe endotracheal intubation.

No MeSH data available.


Related in: MedlinePlus