Congenital vascular anomalies and persistent respiratory symptoms in children

doi:10.1016/S0165-5876(99)00252-9

International Journal of Pediatric Otorhinolaryngology

Volume 51, Issue 1, 15 November 1999, Pages 23-31

https://doi.org/10.1016/S0165-5876(99)00252-9 Get rights and content

Abstract

Congenital cardiovascular anomalies are a rare but treatable cause of stridor in the paediatric population. Despite this, we and the authors of other series have found a delay in referral for endoscopic diagnosis. The duration of symptoms prior to endoscopic diagnosis ranged from 6 weeks to 10 years. It has been estimated that up to 3% of the population have congenital anomalies of the aortic arch complex and we feel that many patients currently being diagnosed as having recurrent croup or atypical asthma must be excluded from this group. We had 16 cases of congenital vascular anomalies causing tracheobronchial compression. Fifteen were due to innominate artery compression and one was due to a double aortic arch. Presenting symptoms included stridor (100%), persistent cough (75%), chronic dyspnoea (75%), reflex apnoea (60%), recurring respiratory tract infections (56%) and dysphagia (25%). Direct laryngoscopy and bronchoscopy was diagnostic in all cases and is our investigation of choice. Eleven underwent aortopexy (69%), one had division of the non-dominant aortic arch (6%) and four were managed conservatively (25%). They were followed for up to 9 years post-operatively and 75% had complete resolution of their symptoms. We feel that only a high clinical index of suspicion will enable these potentially fatal anomalies to be diagnosed.

Introduction

Tracheobronchial compression due to congenital anomalies of the mediastinal great vessels has long been included in the differential diagnosis of persistent stridor in the paediatric population. The first reported case of a symptomatic vascular compression was in 1787 by Bayford [1]. He gave a graphic account of how a young woman starved to death as a result of oesophageal compression from a congenitally anomalous right subclavian artery. He coined the now classic phrase ‘Dysphagia Lusoria’ which attributed her cause of death to a ‘sport of nature’.

However despite this knowledge, It is our impression that both otolaryngologist and physician alike are still underestimating the incidence of this condition.

First arising from the mesoderm at 3 weeks of life, the majority of the six primitive aortic arches regress leaving segments of the III, IV, VI, ventral and dorsal roots to comprise the mature anatomy of the aortic arch, the aortic arch vessels, pulmonary artery and ductus arteriosus [2]. Abnormal development or inappropriate persistence of segments of the primitive arch anatomy will result in an abnormal configuration of the mediastinal great vessels. The incidence of abnormal aortic arch vessels has been estimated to be as high as 3%, but only one-third of these will result in significant symptoms [3]. Obviously the vast majority of these remain asymptomatic, but it is our contention that a number of paediatric patients currently being diagnosed as having ‘recurrent croup’ or ‘atypical asthma’ could possibly have an undiagnosed vascular compression of the tracheobronchial tree.

Holinger [4] looked at the aetiology of persistent stridor in a series of 219 paediatric patients presenting with stridor. He found that there was a congenital cause for the stridor in 87% of cases and that surprisingly many did not exhibit this symptom until after birth. Laryngomalacia was found to be the commonest congenital abnormality; but more worrisome was his finding that over 26% were referred with an erroneous presumptive diagnosis. He found that 7% had an underlying vascular anomaly and that of these 5% were due to innominate artery compression (IAC) of the trachea. Interestingly he excluded conditions such as croup from his study cohort and we believe if this group were included its possible the incidence of symptomatic vascular compression could be higher.

Section snippets

Materials and methods

Between 1986 and 1998 a total of 16 patients were diagnosed as having tracheal compression from an extrinsic cause. All of these patients were referred from paediatricians at our institution for investigation of persistent obstructive respiratory symptoms not responding to medical treatment. The hospital records on these patients were retrieved and note was made regarding presenting symptoms, age at presentation, pre-endoscopy diagnosis, duration of symptoms prior to diagnosis, investigations

Results

Of the 16 patients 11 were male (69%) and five were female (31%). A diagnosis of IAC was made in 15 cases (94%) and a double aortic arch (DAA) in one (6%). Both of these anomalies are illustrated in Fig. 1, Fig. 2. The median age at diagnosis was 13 months (range: 2 months to 10 years). The duration of symptoms prior to diagnosis ranged from 6 weeks to 10 years, the median duration was 4 months.

Presenting symptoms are shown in Table 1. Stridor occurred in all cases and was felt to be

Discussion

Jackson [6] stated as far back as 1942 that ‘stridor is of course a symptom and not a disease’ and that these cases ‘call urgently for accurate differential diagnosis by direct laryngoscopy’.

Despite this we and others [5], [4], [7] still see an inordinate delay in referring patients for endoscopic diagnosis. Holinger noted that his referrals had an erroneous diagnosis prior to endoscopy in 26.5% and were therefore being inappropriately managed. More recently McLaughlin et al. [8] found that

Conclusion

Congenital vascular anomalies causing significant upper aerodigestive tract obstruction are rare. Because of this their diagnosis is sometimes missed or delayed resulting in sometimes disastrous consequences for the child. Early diagnosis and prompt surgical repair for those at risk is vital. Especially where reflex apnoea exists there is not a potential but a real risk to these infants. In our series we had a 60% incidence of this symptom. It must, however, be highlighted also that as our

References (22)

E.G. King
Hypoxaemia during fibreoptic bronchoscopy [editorial]
Chest
(1974)
K. Altman et al.
Comparison of endoscopy and radiographic fluoroscopy in the evaluation of paediatric airway abnormalities
Int. J. Paediatr. Otorhinolaryngol.
(1998)
W.T. Mustard et al.
Tracheal compression by the innominate artery in children
Ann. Thorac. Surg.
(1969)
Bayford D. An account of a singular case of obstructed deglutition. Memoirs of the Medical Society of London printed by...
J. Langman
L.M. Marmon et al.
Vascular rings and slings: long term follow-up of pulmonary function
J. Paediatr. Surg.
(1984)
L.D. Holinger
Aetiology of stridor in the neonate, infant and child
Ann. Otol. Rhin. Laryng.
(1980)
B. Fearon et al.
Tracheobronchial compression by congenital cardiovascular anomalies in childhood: syndrome of apnoea
Ann. Otol. Rhinol. Laryngol.
(1963)
C. Jackson et al.
P. Horvath et al.
Intermediate to late results of surgical relief of vascular tracheobronchial compressions. Eur. J
Cardio.-Thorac. Surg.
(1992)

R.B. Mc Laughlin et al.

Vascular anomalies causing symptomatic tracheobronchial compression

Laryngoscope

(1998)

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¹: Present address: Royal Victorian Eye and Ear Hospital, 32 Gisborne Street, East Melbourne 3002, Vic., Australia. Tel.: +61-3-99298666; fax: +61-3-96637203.

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Congenital vascular anomalies and persistent respiratory symptoms in children

Abstract

Introduction

Section snippets

Materials and methods

Results

Discussion

Conclusion

Chest

Int. J. Paediatr. Otorhinolaryngol.

Ann. Thorac. Surg.

Vascular rings and slings: long term follow-up of pulmonary function

J. Paediatr. Surg.

Aetiology of stridor in the neonate, infant and child

Ann. Otol. Rhin. Laryng.

Tracheobronchial compression by congenital cardiovascular anomalies in childhood: syndrome of apnoea

Ann. Otol. Rhinol. Laryngol.

Intermediate to late results of surgical relief of vascular tracheobronchial compressions. Eur. J

Cardio.-Thorac. Surg.

Vascular anomalies causing symptomatic tracheobronchial compression

Laryngoscope