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Daniel S. Tsze, Yaffa M. Vitberg, Joel Berezow, Thomas J. Starc and Peter S. Dayan
Pediatrics 2014;134;e266; originally published online June 16, 2014;
DOI: 10.1542/peds.2013-3183
The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pediatrics.aappublications.org/content/134/1/e266.full.html
e266 TSZE et al
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CASE REPORT
Tetralogy of Fallot (TOF) is one of the most or intravenous (IV) route, all of which was 175 bpm, RR 44 breaths/minute,
common forms of cyanotic congenital can be painful and distressing to the BP 99/74 mm Hg, and the O2 sat had
heart disease, and consists of a ven- child, and may exacerbate the existing dropped to 37%. At this time, we ad-
tricular septal defect (VSD), pulmonary hypoxia. There is also the concern that ministered 10 mg (2 mg/kg) of IN fen-
valve stenosis, an overriding aorta, and morphine may exacerbate a hypoxic tanyl using a mucosal atomization device.
right ventricular hypertrophy.1 The lead- spell because of its potential to de- Ten minutes after IN fentanyl administra-
ing cause of morbidity and mortality in crease systemic vascular resistance.3,4 tion, the patient had calmed down, was no
patients who have uncorrected TOF are Intranasal (IN) fentanyl is an alterna- longer grunting, and remained awake
acute episodes of hypoxia and cyanosis tive strategy, combining a painless and and well perfused. The vital signs had
known as hypoxic, hypercyanotic, or “tet” effective route of administration with stabilized, with a HR of 163 bpm, RR 34
spells. These spells are characterized by the use of an opioid less likely to cause breaths/minute, BP of 92/48 mm Hg,
a paroxysm of hyperpnea, irritability or hemodynamic instability than mor- and an O2 sat of 78% on a 100% oxygen
agitation, and prolonged crying, leading phine.3–6 We describe the first case, to non-rebreather. At this point, an IV line
to worsening cyanosis. The underlying our knowledge, of administering IN was placed without complication, so
pathophysiology involves a shunting of fentanyl to successfully treat a child that IV access would be available dur-
deoxygenated blood from the right to left experiencing a TOF hypoxic spell. ing the hospital admission. A 20-mL/kg
ventricle through the VSD, which results bolus of normal saline was adminis-
from increased pulmonary outflow tract CASE REPORT tered. The patient remained in the ED
obstruction, decreased systemic vascular for ∼2 hours before admission to the
A 3-month-old girl with standard TOF
resistance, and obstruction of the right hospital, during which time her clinical
physiology, consisting of a large outlet
ventricular outflow tract. A cycle is condition and vital signs remained
VSD and infundibular and pulmonary
established as the subsequent de- stable with O2 sat .80%. She did not
valve stenosis, was initially being eval-
crease in the partial pressure of oxy- receive any additional medications and
uated in the cardiology clinic for a pre-
gen and increase in carbon dioxide in did not experience any more hypoxic
operative appointment. She began crying
episodes during her ED course.
the blood continue to stimulate and per- vigorously when an EKG was being
petuate hyperpnea. This results in in- obtained, and her oxygen saturation (O2
creased systemic venous return, and in sat) dropped from “the 80’s into the DISCUSSION
turn increases the shunting through the 50’s.” She did not improve with knee-to- We have presented the first report of the
VSD.2 If left untreated and the cycle per- chest positioning and was emergently successful treatment of a TOF hypoxic
sists, the patient will become pro- transported to the pediatric emergency spell using IN fentanyl. The clinical re-
gressively more hypoxic and acidotic, department (ED). sponse that we observed in our patient
which will lead to eventual cardiac arrest. On arrival to the pediatric ED, the patient is consistent with the known pharma-
The treatment of TOF hypoxic spells is was grunting and centrally cyanotic, but cologic and clinical CNS effects of IN
aimed at breaking this cycle by abol- awake, alert, and crying vigorously. Her fentanyl, and illustrates the successful
ishing the hyperpnea and/or increasing initial ED vital signs were a temperature application of a novel technique in re-
the systemic vascular resistance. The of 36.7° C, a heart rate (HR) of 176 beats solving a life-threatening cardiac event.
easiest and most readily available per minute (bpm), respiratory rate (RR) The potential benefits of IN fentanyl
maneuvers are to place the patient in of 63 breaths/minute, blood pressure rather than SC or IM morphine result
a knee-to-chest position, which will in- (BP) of 107/78 mm Hg, and an O2 sat of from being able to avoid needle sticks,
crease systemic vascular resistance, 56% in room air. Her extremities were the rapid CNS action achievable by IN
and to administer oxygen. If these warm and well perfused. A calming administration, and the specific prop-
maneuvers are unsuccessful, the most environment was immediately opti- erties of fentanyl.
common subsequent treatment is to mized, including parental presence and Both the IM and SC routes have rela-
administer morphine, which calms the involvement of a child-life specialist; tively slow onset of action, and require
patient, resolves the hyperpnea, nor- knee-to-chest positioning was per- a needle stick that is painful and can
malizes the systemic venous return, formed; and 100% oxygen was admin- aggravate the distress and hyperpnea
and increases the partial pressure of istered by using a non-rebreather mask. that perpetuate the cycle of hypoxia and
oxygen in the blood.2 Eight minutes later, the patient remained worsening cyanosis. Obtaining IV ac-
Morphine may be administered by the awake, alert, and well perfused, but cess to administer morphine can be
intramuscular (IM), subcutaneous (SC), was still crying and cyanotic. Her HR similarly distressing, is dependent on
REFERENCES
1. Apitz C, Webb GD, Redington AN. Tetralogy sufentanil anesthesia for cardiac surgery: in- cardiovascular profiles. J Cardiothorac
of Fallot. Lancet. 2009;374(9699):1462– duction, emergence, and extubation. Anesth Anesth. 1988;2(6):749–757
1471 Analg. 1986;65(3):259–266 5. Chien YW, Su K, Chang SF. Nasal Systemic
2. Park MK. Pediatric Cardiology for Practi- 4. Benthuysen JL, Foltz BD, Smith NT, Sanford Drug Delivery. New York, NY: Marcel Dekker
tioners. 5th ed. Philadelphia, PA: Elsevier; TJ Jr, Dec-Silver H, Westover CJ. Prebypass Inc; 1989
2007:235–248 hemodynamic stability of sufentanil-O2, 6. Wolfe TR, Braude DA. Intranasal medication
3. Sanford TJ Jr, Smith NT, Dec-Silver H, Harrison fentanyl-O2, and morphine-O2 anesthesia delivery for children: a brief review and
WK. A comparison of morphine, fentanyl, and during cardiac surgery: a comparison of update. Pediatrics. 2010;126(3):532–537
e268 TSZE et al
Downloaded from pediatrics.aappublications.org at Bibliotheque de l'Universite Laval on July 4, 2014
CASE REPORT
7. Lahat E, Goldman M, Barr J, Bistritzer T, ministration: focus on opioids. Pharmacol laryngospasm in preterm and term infants.
Berkovitch M. Comparison of intranasal Ther. 2012;134(3):366–379 Crit Care Med. 2000;28(3):836–839
midazolam with intravenous diazepam for 15. Christrup LL, Foster D, Popper LD, Troen T, 21. Dewhirst E, Naguib A, Tobias JD. Chest wall
treating febrile seizures in children: prospective Upton R. Pharmacokinetics, efficacy, and tol- rigidity in two infants after low-dose fen-
randomised study. BMJ. 2000;321(7253):83–86 erability of fentanyl following intranasal ver- tanyl administration. Pediatr Emerg Care.
8. Borland M, Jacobs I, King B, O’Brien D. A sus intravenous administration in adults 2012;28(5):465–468
randomized controlled trial comparing in- undergoing third-molar extraction: a ran- 22. Coruh B, Tonelli MR, Park DR. Fentanyl-
tranasal fentanyl to intravenous morphine domized, double-blind, double-dummy, two- induced chest wall rigidity. Chest. 2013;
for managing acute pain in children in the way, crossover study. Clin Ther. 2008;30(3):
emergency department. Ann Emerg Med. 143(4):1145–1146
469–481
2007;49(3):335–340 23. Davis MP. Fentanyl for breakthrough pain:
16. Mercadante S, Radbruch L, Davies A, et al. a systematic review. Expert Rev Neurother.
9. Moksnes K, Fredheim OM, Klepstad P, et al.
A comparison of intranasal fentanyl spray
Early pharmacokinetics of nasal fentanyl: is 2011;11(8):1197–1216
with oral transmucosal fentanyl citrate
there a significant arterio-venous difference? 24. Mudd S. Intranasal fentanyl for pain man-
for the treatment of breakthrough cancer
Eur J Clin Pharmacol. 2008;64(5):497–502 agement in children: a systematic review of
pain: an open-label, randomised, cross-
10. Born J, Lange T, Kern W, McGregor GP, the literature. J Pediatr Health Care. 2011;
over trial. Curr Med Res Opin. 2009;25(11):
Bickel U, Fehm HL. Sniffing neuropeptides: 25(5):316–322
2805–2815
a transnasal approach to the human brain. 25. Smith H. A comprehensive review of rapid-
Nat Neurosci. 2002;5(6):514–516 17. Ellerton JA, Greene M, Paal P. The use of
analgesia in mountain rescue casualties onset opioids for breakthrough pain. CNS
11. Illum L. Is nose-to-brain transport of drugs Drugs. 2012;26(6):509–535
with moderate or severe pain. Emerg Med
in man a reality? J Pharm Pharmacol. 2004; 26. Hansen MS, Mathiesen O, Trautner S, Dahl
56(1):3–17 J. 2013;30(6):501–505
18. Zeppetella G, Davies A, Eijgelshoven I, Jansen JB. Intranasal fentanyl in the treatment of
12. Lötsch J, Walter C, Parnham MJ, Oertel BG,
JP. A network meta-analysis of the efficacy of acute pain—a systematic review. Acta
Geisslinger G. Pharmacokinetics of non-
opioid analgesics for the management of Anaesthesiol Scand. 2012;56(4):407–419
intravenous formulations of fentanyl. Clin
Pharmacokinet. 2013;52(1):23–36 breakthrough cancer pain episodes. J Pain 27. Hansen MS, Dahl JB. Limited evidence for
Symptom Manage. 2013;pii: S0885-3924(13) intranasal fentanyl in the emergency de-
13. Dietrich E, Gums JG. Intranasal fentanyl
spray: a novel dosage form for the treat- 00365-5 partment and the prehospital setting—
ment of breakthrough cancer pain. Ann 19. Vaughn RL, Bennett CR. Fentanyl chest wall a systematic review. Dan Med J. 2013;60(1):
Pharmacother. 2012;46(10):1382–1391 rigidity syndrome—a case report. Anesth A4563
14. Grassin-Delyle S, Buenestado A, Naline E, Prog. 1981;28(2):50–51 28. Rao PS. Diagnosis and management of cy-
et al. Intranasal drug delivery: an efficient 20. Fahnenstich H, Steffan J, Kau N, Bartmann anotic congenital heart disease: part I. In-
and non-invasive route for systemic ad- P. Fentanyl-induced chest wall rigidity and dian J Pediatr. 2009;76(1):57–70