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Case Report
A R T I C L E I N F O
A B S T R A C T
Article history:
Received 24 December 2015
Accepted 6 February 2016
Available online 12 February 2016
Cervicofacial segmental infantile hemangiomas (IH) may result in airway obstruction requiring use of
propranolol to induce hemangioma regression and reestablish the airway. We present the rst case using
intravenous (IV) propranolol for control of airway obstruction and rapid expansion of cervicofacial IH in
the setting of necrotizing enterocolitis (NEC) impaired gastrointestinal function. Intravenous dosing of
propranolol was tolerated well in a critically ill neonate with multisystem complications of prematurity.
2016 Elsevier Ireland Ltd. All rights reserved.
Keywords:
Segmental infantile hemangioma
Propranolol
Vascular anomaly
Necrotizing enterocolitis
Airway
1. Introduction
Segmental hemangiomas are a subtype of infantile hemangioma (IH). The segmental forms of infantile hemangiomas are often
extensive, and can present with complications of IH, including
airway compromise.
We present a case report of our experience using intravenous
propranolol in an attempt to expediently alleviate airway
obstruction secondary to a segmental hemangioma involving
the upper aerodigestive tract including the oor of mouth in an
infant with active NEC.
2. Case description
The baby was born at 27 and 6/7 weeks, weighing 0.645 kg with
respiratory insufciency secondary to prematurity. On physical
exam the infant was noted to be morphologically normal. There
PHACE (S) highly unlikely. Airway evaluation with rigid laryngoscopy and bronchoscopy was negative at that time for airway
involvement. Shortly thereafter, the patient had a bloody stool
with an abdominal X-ray demonstrating pneumatosis consistent
with NEC.
Given the concern for developing NEC, the patient was not
treated with propranolol. Following resolution of the NEC on DOL
41, the patient was started on oral propranolol at 0.2 mg/kg/day
divided TID. However, the IH had grown signicantly in the
interval with components involving the neck, oor of mouth, facial
and periorbital areas which continued to enlarge (Figs. 1 and 2).
Shortly following starting oral propranolol, the patient gradually
developed upper airway obstruction and required intubation
following episodes of respiratory distress. Over the following days,
the dosage was gradually increased to 0.4 mg/kg/day divided TID.
However the intraoral components continued to enlarge and
painful ulceration of the lower lip and tongue developed. The
hemangioma continued to rapidly enlarge with the IH involving
the FOM resulting in posteriorsuperior tongue displacement and
impingement on the airway.
Despite gradual increases in the dosage of the PO propranolol
(0.2 mg/kg/day to 1.6 mg/kg/day divided TID) the IH continued to
enlarge. It was then hypothesized that due to the NEC, the enteral
propranolol was not being made bioavailable via the patients
gastrointestinal tract given the lack of response to medication. At 8
weeks of age, she was switched to IV propranolol for 3 weeks. IV
propranolol was initially dosed at 0.3 mg/kg/day divided TID and
titrated up over the following period to 0.5 mg/kg/day which was
determined by the Pharmacology service to be the IV equivalent of
2 mg/kg/day given enterally.
Within 4 days of starting IV propranolol, there was signicant
decrease in the tongue and oor of mouth swelling and the
softening of the hemangioma over the mandible (Fig. 3). Subsequent direct laryngoscopy conrmed the absence of airway
obstruction secondary to hemangioma and noted continued
improvement of tongue and oor of mouth swelling. The patient
was successfully extubated and transitioned back to oral
propranolol. She has continued to improve on outpatient
propranolol at age 7.5 months continuing on a dosage of 2 mg/
kg/day divided TID (Fig. 4).
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3. Discussion
The use of propranolol for inducing regression of infantile
hemangiomas has been well established in the literature since the
initial discovery of the utility of the non-selective beta blocker in
promoting regression [1]. Following this discovery, propranolol
has become a primary treatment for infantile hemangiomas and is
considered to be standard of care [2,3]. The mechanism of action of
propranolol in causing regression of infantile hemangiomas
remains unclear. Hypotheses around its mechanism of action
include down-regulation of proangiogenic factors and apoptosis of
capillary cells [4]. The drug is typically completely absorbed from
the gastro-intestinal tract and is highly protein bound (8095%)
systemically [5]. In addition, the drug experiences a high rate of
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4. Conclusion
The use of IV propranolol, in the setting of a premature infant
with severe NEC, controlled a rapidly expanding IH with airway
impingement quickly within a matter of several days of treatment.
The dosing was tolerated well in a critically ill neonate with
multisystem complications of prematurity.
References