Title: Two Year Old White Female with a form of Dwarfism
Two year old while female with a form of dwarfism (Spondyloepiphyseal Dysplasia or SED) was scheduled to undergo posterior cervical fusion for C-Spine instability. Family history was negative for anesthetic complications; patient had no known allergy and was not on any medication. Anesthetic consisted of isoflurane, sufentanyl and vecuronium had been used for neuromuscular blocking. Only non-glucose containing fluids were used in the OR due to the procedure being close to the brain and for fear of hyperglycemia related compromise in the neurological recovery. Axillary temperature at the beginning of the case was 36.0C0 which progressively increased to 37.9C0 towards end of the surgery. There were no other unusual events noted during surgery. Four hours later temperature was found to be 105.0C0 while in PACU.
Laboratory investigations were unremarkable with normal CPK, electrolytes and acid base status except for blood sugar that was below normal. Sufficient response to cooling and acetaminophen eventually resulted in normothermia.
Questions: Choose all correct answers
- What is the likely diagnosis
a. Malignant Hyperthermia
b. Hyperthermia unrelated to MH
c. Hypoglycemia
d. Dehydration
- What is the most likely cause of hypoglycemia
a. Increased need for substrates due to hyperthermia temperature
b. Undiagnosed pancreatic tumor
c. Lack of glucose containing intravenous fluids during the surgery
d. Lab error
- Patients who are dwarfs are known to
a. Have bone, cartilage and collagen disorders
b. Often have a known genetic defect
c. Known to exhibit increased sweating and run elevated temperatures
d. Often have abnormalities of spine and vertebral column
e. All the above
Answers:
1. b
Many forms of dwarfism (skeletal dysplasia) are known to cause temperature elevation, usually of mild nature, in children. Literature provides information specifically regarding forms of dwarfisms such as Arthrogryposis and Osteogenesis Imperfecta. There are case reports of children with such skeletal dysplasia’s being treated with dantrolene for fear of ensuing MH, but often without supporting investigational evidence.
It is speculated that the dissipation of heat generated is impaired in these children, perhaps due to their smaller body surface area which contributes to the elevation in core temperature. Parents of such children will often provide a history of their children sweating profusely to the extent that the bed covers are often wet.
2. a and c
The patients undergoing a neurosurgical procedure are often not given glucose containing solution for fear of hyperglycemia and worse neurological outcome. Children who are otherwise NOT normal and may have an altered or increased need for substrates including glucose are better off being monitored for their intraoperative glucose levels.
Perioperative glucose studies have all been done in otherwise normal children and the results and conclusions are better applied to normal children.
3. e
This is a great site to learn about 'dwarfs'
