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Department of Anesthesia and Critical Care, atung{at}dacc.uchicago.edu(Tung, Anderson, Daves) Department of Pediatrics and Human Genetics(Waggoner) Department of Anesthesia and Critical Care and Pediatrics, University of Chicago, atung{at}dacc.uchicago.edu(Kahana)
To the Editor:
Holoprosencephaly (HPE) is a congenital brain malformation wherein the forebrain fails to normally bifurcate. The clinical presentation is variable but can include mild-to-severe mental retardation, developmental delay, hypothyroidism, adrenal insufficiency, and diabetes insipidus (1–3). Although the condition is rare, patients with HPE frequently require craniofacial surgery for cleft lip and midface hypoplasia. We recently anesthetized such a patient.
A 5-mo-old, 6.3-kg infant underwent cleft lip repair under general anesthesia. During preoperative evaluation, HPE was suggested by clinical examination and confirmed by magnetic resonance imaging/computed tomography. Endocrine examination revealed a normal cortisol level, fluid intake, and urine output and no clinical evidence of posterior pituitary dysfunction. The patient received 300 mL of lactated Ringer's solution during the uneventful 190-min surgery.
During the first 3 postoperative days, the patient received D5/0.9 normal saline at 28 mL/day with oral Pedialyte but appeared lethargic. Serum electrolytes on postoperative day #4 showed: Sodium = 192 meq/dL. Concurrent urine osmolarity was 405 Mosm/kg, and serum osmolarity = 381 Mosm/kg. Her hypernatremia gradually corrected with hypotonic fluids, and she was discharged on postoperative day 12. A subsequent serum sodium measurement in blood samples taken preoperatively and frozen was 157 meq/dL.
Although many patients with HPE also have diabetes insipidus (2), treatment is often unnecessary, as patients respond to antidiuretic hormone, fluid balance is normal, and sodium levels only mildly elevated. Physicians familiar with HPE, however, anecdotally describe dramatic worsening of diabetes insipidus during the physiologic stress of surgery or illnesses such as the flu (personal communication, Dr. Eric Levey, Carter Center for the Evaluation and Management of Patients with Holoprosencephaly, Baltimore, MD). Because pediatric patients are more commonly hyponatremic after surgery (4), this report highlights the importance of monitoring serum electrolytes perioperatively in patients with developmental delay or hypotelorism who present for craniofacial surgery. Such patients may have HPE and thus be at increased risk for postoperative hypernatremia.
References
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