case report alte and feeding intolerance as a presentation...

Case ReportALTE and Feeding Intolerance as a Presentation ofDouble Aortic Arch

Rekha Krishnasarma, Liza Green Golan Mackintosh, and Francine Bynum

Department of Pediatrics at the Children’s Hospital of Los Angeles, Los Angeles, CA, USA

Correspondence should be addressed to Rekha Krishnasarma; [email protected]

Received 8 July 2016; Accepted 29 August 2016

Academic Editor: Carmelo Romeo

Copyright © 2016 Rekha Krishnasarma et al. This is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properlycited.

Many children who are admitted to pediatric hospitals with the chief complaint of apparent life-threatening event (ALTE) are,in fact, well appearing by the time the inpatient medical team evaluates the patient. This presents a diagnostic and therapeuticchallenge. We describe a case of a six-month-old full-term female presenting with an ALTE and found to have a double aortic arch,a congenital anomaly that usually presents with a more progressive onset of symptoms such as chronic cough, positional stridor,and feeding difficulties.This case highlights the importance of maintaining a broad differential in a patient presenting with findingsof tracheoesophageal pathology on clinical exam.

1. Introduction

We discuss the case of a 6-month-old full-term female withlong-standing feeding intolerance and chronic respiratorysymptoms presenting with an apparent life-threatening eventand found to have a double aortic arch.

2. Case Presentation

A 6-month-old full-term female with a history of respiratorysyncytial virus bronchiolitis at two weeks of age presentedto our hospital via emergency medical services with thechief complaint of ALTE. As per the mother, the patient wasnoted to have an episode of perioral cyanosis, coughing, andincreased work of breathing after being placed on her backimmediately following a bottle-feed. When the mother notedthese symptoms, she picked up the patient whowas describedas “pale” and “limp.”Abystanderwas able to forcefully pat thepatient’s back and formula was expressed from themouth. Bythe time medical personnel arrived, the patient was back toher baseline behavior.

Upon further questioning, it became clear that the patienthad a several month history of chronic cough, congestion,and feeding intolerance for which she had already undergonean extensive outpatient workup.Thisworkup included testing

for various immunodeficiencies, as well as a laryngoscopyby otolaryngology. Per report, these tests and the aboveprocedure did not reveal any pathology.Themother reportedthat the primary care physician had seen the patient earlierin the week of presentation for increased coughing andprescribed nebulized albuterol treatments and a short courseof steroids for presumed reactive airway disease. Neitherintervention alleviated her symptoms. While this was thepatient’s first episode of cyanosis associated with cough, themother reported a worsening of the patient’s cough andshortness of breath upon lying flat and after feeding.

After admission to the floor, the teamobserved the patientfeed which resulted in a choking-like episode and overtrespiratory distress, only alleviated by positional maneuvers.Other pertinent positives on her physical exam were baselineaudible breathing with occasional inspiratory stridor. Giventhe patient’s increased symptomatology with feeding and herconcerning baseline respiratory exam, shewas deemedunsafefor oral feeds until her evaluation was complete.

Our initial evaluation included a chest radiograph andgastric emptying scintigraphy. The chest radiograph demon-strated slightly hyperinflated lung fields bilaterally but wasotherwise unremarkable.The gastric emptying study revealeddelayed gastric emptying, without overt evidence of reflux.Additional workup for potential anatomic abnormalities was

Hindawi Publishing CorporationCase Reports in PediatricsVolume 2016, Article ID 8475917, 3 pageshttp://dx.doi.org/10.1155/2016/8475917

2 Case Reports in Pediatrics

Left archRight arch

TracheaEsophagus

(a)

Left archRight arch

(b)

Trachea

(c)

Figure 1: Contrast-enhanced magnetic resonance angiography demonstrating the double aortic arch with dominant right arch asdemonstrated on axial (a), coronal (b), and sagittal planes (c). The axial and sagittal images reveal that the trachea and esophagus aresurrounded and compressed by a vascular ring formed by the double aortic arch. The coronal image demonstrates that the right arch islarger and higher in position than left counterpart, which is hypoplastic.

pursued with fluoroscopy of the upper gastrointestinal tract,which showed a prominent posterior impression on theesophagus on the lateral projection at the level of the aorticknob. On the frontal view, the esophageal impression wasmore exaggerated on the left side compared to the right.These findings suggested an aberrant right subclavian artery,or double aortic arch, and the radiologist recommendedmagnetic resonance imaging (MRI) to better elucidate thesefindings (Figure 1). MRI of the chest revealed a double aorticarch with the right arch being dominant, significantly larger,andmore superior in position than its left counterpart, whichwas hypoplastic. The left carotid and left subclavian arosefrom the smaller left arch. There was marked narrowing ofthe trachea at the site of the vascular ring; the tracheal diam-eter measured approximately 2.5mm just above the carina.With the diagnosis of a double aortic arch compressing thetrachea and esophagus, our patient was transferred to thecardiothoracic team for surgical repair with video-assistedthoracoscopic surgery (VATS). She underwent division of leftarch, leaving the dominant right arch in place.

3. Discussion

An apparent life-threatening event (ALTE) is defined as anepisode that is frightening to the observer and is charac-terized by some combination of apnea, color change, andchoking or gagging [1]. The differential diagnosis for the eti-ology of ALTEs in infants includes a broad array of con-genital or acquired disorders and spans multiple organ sys-tems. ALTE is associated with infectious or inflammatorypulmonary conditions, gastroesophageal reflux, neurologicdisease, immune compromise, metabolic disorders, and con-genital anomalies [2]. While rare, congenital anomalies suchas vascular rings must be considered in an infant whopresents with an ALTE preceded by a history of chronicsymptomatology of respiratory and/or feeding difficulties, asdepicted by our case.

Vascular rings arise when specific portions of the pairedaortic arch system persist or regress abnormally. They canbe complete or incomplete depending on the compressionof the trachea and esophagus, with complete vascular rings

encircling and compressing the two structures. The doubleaortic arch, a complete vascular ring, is the most commontype, resulting from the failure of the fourth embryonic bran-chial arch to regress, leading to an ascending aorta thatdivides into a left and right arch that fuse together to com-pletely encircle the trachea and esophagus [3, 4].

Double aortic arches comprise 1-2% [5] of all cardiacanomalies and up to one-third of all vascular rings [6, 7].Symptoms usually appear in the first six months of life.Compression from complete anomalies leads to respiratoryand gastrointestinal symptoms. Airway symptoms such asinspiratory stridor and wheezing are found more commonlywhen the trachea is compressed in a double aortic arch,while gastrointestinal symptoms such as dysphagia, reflux,and choking episodes [6] are seen when the esophagus iscompressed posteriorly. Respiratory arrest has been reportedin 7% of reported cases [8]. Not surprisingly, complete anom-alies are more often associated with more severe symptomsthan incomplete forms, making a double aortic arch the mostcommon vascular ring that necessitates surgical repair.

Cross-sectional imaging with magnetic resonance angi-ography (MRA) or computed tomography angiography(CTA) of the chest [9] is crucial in the definitive diagnosisand surgical management of vascular rings. Generally, apatient has had abnormalities found on chest X-ray, echo-cardiography, bronchoscopy, gastric emptying studies, orfluoroscopic studies such as a modified barium swallow orupper gastrointestinal series. In our case, the upper gastro-intestinal series showed compression of the trachea andesophagus, thus prompting further evaluation with MRA.

Surgery to correct a double aortic arch is reportedlywell tolerated [10]. Two approaches are described, via a leftthoracotomy and/or VATS [11]. In repair of a double aorticarch, the division is made in the hypoplastic arch segment,which is usually the left. Complications and deaths during orafter this repair have been reported in patients who have hadother comorbid medical conditions. Operative mortality forvascular ring division is close to zero as demonstrated in thepediatric surgery literature [11].

In conclusion, our clinical case demonstrates the impor-tance of recognizing symptoms and physical findings of

Case Reports in Pediatrics 3

vascular rings. Although this patient presented with anacute event, she demonstrated repeated progressive episodesof postprandial choking and chronic respiratory distress,prompting additional workup. With advances in both radi-ologic diagnosis and surgical management, the expedientdiagnosis and treatment of the double aortic arch is possible.Needless to say, thorough history taking and physical examskills are of utmost importance as well as recognition of theneed for further diagnostic examinations.

Competing Interests

The authors declare that there are no competing interestsregarding the publication of this paper.

References

[1] J. S. Tieder, J. L. Bonkowsky, R. A. Etzel et al., “Brief resolvedunexplained events (formerly apparent life-threatening events)and evaluation of lower-risk infants,” Pediatrics, vol. 137, no. 5,Article ID e20160590, 2016.

[2] L. Y. Fu and R. Y. Moon, “Apparent life-threatening events: anupdate,” Pediatrics in Review, vol. 33, no. 8, pp. 361–369, 2012.

[3] N. Becit, B. Erkut, and Y. Karaca, “Vascular ring: tracheoeso-phageal compression associatedwith symmetrical double aorticarch,” Texas Heart Institute Journal, vol. 35, no. 2, pp. 209–210,2008.

[4] R. K. Shah, B. N. Mora, E. Bacha et al., “The presentation andmanagement of vascular rings: an otolaryngology perspective,”International Journal of Pediatric Otorhinolaryngology, vol. 71,no. 1, pp. 57–62, 2007.

[5] H. K. Seo, H. G. Je, I.-S. Kang, and K. A. Lim, “Prenatal doubleaortic arch presentingwith a right aortic arch and an anomalousartery arising from the ascending aorta,” International Journal ofCardiovascular Imaging, vol. 26, no. 1, pp. 165–168, 2010.

[6] A. Licari, E. Manca, G. A. Rispoli, S. Mannarino, G. Pelizzo, andG. L. Marseglia, “Congenital vascular rings: a clinical challengefor the pediatrician,” Pediatric Pulmonology, vol. 50, no. 5, pp.511–524, 2015.

[7] A. Turner, G. Gavel, and J. Coutts, “Vascular rings: presentation,investigation and outcome,” European Journal of Pediatrics, vol.164, no. 5, pp. 266–270, 2005.

[8] K. Alsenaidi, R. Gurofskya, T. Karamlou, W. G. Williams, andB. W. McCrindle, “Management and outcomes of double aorticarch in 81 patients,” Pediatrics, vol. 118, no. 5, pp. e1336–e1341,2006.

[9] M. Etesami, R. Ashwath, J. Kanne, R. C. Gilkeson, and P. Rajiah,“Computed tomography in the evaluation of vascular rings andslings,” Insights into Imaging, vol. 5, no. 4, pp. 507–521, 2014.

[10] A. Bonnard, F. Auber, L. Fourcade, V. Marchac, S. Emond, andY. Revillon, “Vascular ring abnormalities: a retrospective studyof 62 cases,” Journal of Pediatric Surgery, vol. 38, no. 4, pp. 539–543, 2003.

[11] M. Ruzmetov, P. Vijay, M. D. Rodefeld, M. W. Turrentine, andJ. W. Brown, “Follow-up of surgical correction of aortic archanomalies causing tracheoesophageal compression: a 38-yearsingle institution experience,” Journal of Pediatric Surgery, vol.44, no. 7, pp. 1328–1332, 2009.

Submit your manuscripts athttp://www.hindawi.com

Stem CellsInternational

Hindawi Publishing Corporationhttp://www.hindawi.com Volume 2014


MEDIATORSINFLAMMATION

of


Behavioural Neurology

EndocrinologyInternational Journal of



Disease Markers


BioMed Research International

OncologyJournal of



Oxidative Medicine and Cellular Longevity


PPAR Research

The Scientific World JournalHindawi Publishing Corporation http://www.hindawi.com Volume 2014

Immunology ResearchHindawi Publishing Corporationhttp://www.hindawi.com Volume 2014

Journal of

ObesityJournal of



Computational and Mathematical Methods in Medicine

OphthalmologyJournal of


Diabetes ResearchJournal of



Research and TreatmentAIDS


Gastroenterology Research and Practice


Parkinson’s Disease

Evidence-Based Complementary and Alternative Medicine

Volume 2014Hindawi Publishing Corporationhttp://www.hindawi.com

case report alte and feeding intolerance as a presentation...

Documents