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Different clinical presentation of vascular rings abnormalities: a clinical challenge for paediatricians

Parisi GF1, Sciacca P1, Papale M1, Franzonello C1, Cuppari C2, Manti S2, Salpietro C2, Leonardi S1
1Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy
2Pediatric Unit, University of Messina, Messina, Italy

Abstract
The term “vascular ring” is generally used to describe a variety of congenital vascular abnormalities of the aortic arch system that can cause tracheal or oesophageal compression. Its clinical presentation is extremely heterogeneous, representing a clinical challenge for paediatricians especially when atypical and/or aspecific clinical symptoms are occurring.
Here, we report three cases of children with three different clinical presentations of a double aortic arch, one of them showed both middle lobe syndrome and double aortic arch, firstly reported in literature.

Key words: children; double aortic arch; middle lobe syndrome; vascular ring.


Introduction
The term “vascular ring” is generally used to describe a variety of congenital vascular abnormalities of the aortic arch system that can cause tracheal or oesophageal compression. The multiple different varieties of vascular rings are due to a defective persistence, involution, or regression of embryonic aortic arches. The clinical manifestations vary primarily with the severity of encroachment by the abnormal vessel on the oesophagus, trachea or bronchus and therefore include stridor, wheezing, cough and dysphagia1.
The double  aortic arch is the most common vascular ring and comprise 1-2% of all cardiac abnormalities2-3. It results from the failure of the fourth embryonic branchial arch to regress, leading to an ascending aorta that divides into a left and right arch that fuse together to completely encircle the oesophagus and the trachea4. The three main types of double aortic arch are the dominant right arch with a small left arch (80%), dominant left arch (10%), and balanced aortic arches (10%)5.
Here, we report three cases of children with three different clinical presentations of a double aortic arch, one of them showed both middle lobe syndrome and double aortic arch, firstly reported in literature.

Case reports
Case 1
We report the case of a one-month-old baby who came to our attention because affected by episodes of asphyxia during the feeding. After delivery she was hospitalized elsewhere due to tremor and dyspnoea. She was discharged and diagnosed with convulsions and gastroesophageal reflux. Afterwards, she suffered from 3 chocking episodes during the feeding. Due to the persistence of the symptoms, she was admitted to our attention. At the entrance her general conditions were poor: mental dullness, stridor was found after chest auscultation, jugular-epigastric inspiratory dyspnoea and rhonchi and rales spread all over the lungs.  For this reason, nourishment was suspended; Infusion therapy and constant monitoring have been carried out. Blood test results were normal. Chest X-ray examination, electroencephalogram and electrocardiogram were negative. Laryngeal-fibroscopy highlighted a posterior hyperaemia of the larynx due to gastroesophageal reflux. Then a contrast-enhanced oesophagogram has been carried out. It showed an anterior tracheal deviation and an oesophageal notch (Fig.1). The tracheoscopy showed a narrowing due to extrinsic compression at about 1cm from the carina. For this reason, we advanced the hypothesis of vascular ring, then confirmed following a computed tomography (CT)-angiography. The newborn girl underwent surgery in order to get her vascular defect repaired. The long-term follow-up showed a clear improvement and resolution of symptoms.

 

Fig. 1. Esophagogram showing an anterior tracheal deviation and an esophageal notch.

Case 2
We report the case of a 8-month-old baby girl born at 35 weeks gestation by emergency caesarean section. The baby girl came to our attention due to persistent cough since the first months of her life. When she entered the ward her general conditions were satisfactory. The general examination showed hyperaemic pharynx; the chest auscultation revealed sour breath with inconstant rhonchi and breathing noises. Blood tests only showed leucocytosis. Then a chest x-ray examination and ultrasound have been carried out. Laryngoscopy revealed morphological aspects that were consistent with laryngomalacia and signs of gastro-oesophageal reflux disease. The echocardiography highlighted a dominant right double aortic arch with anterior trachea. Furthermore, the oesophagogram showed a minor anomaly of the oesophageal profile in the middle tract, as no dilation in the upper cervical-thoracic duct was revealed. Vascular ring was diagnosed and confirmed by the CT-angiography. For this reason, the baby girl has undergone repair surgery, which was followed by improvement of the symptoms.

Case 3
We report the case of a 3-year-old baby girl born at 41 weeks gestation with an history of acute respiratory distress after birth. She had persistent cough since the first months of her life. For this reason, the baby girl came to our attention due to persistent barking cough and recurrent pneumonia. When she entered the ward her general conditions were poor. Physical examination revealed a febrile, baby-girl with cyanotic skin and oxygen saturation of 88% on room air. She was grunting with severe jugular and epigastric retractions, and the chest auscultation revealed rhonchi and rales all over the lungs. Blood tests showed a neutrophil leucocytosis. Then a chest x-ray examination showed a right lower and middle lobe consolidation with a wedge-shaped area of increased density with apex at the hilum and the base towards the pleura (Fig. 2). The CT-angiography highlighted an apparently right-sided aortic arch (Fig. 3, 4) with an area of increased density of pervious bronchi on the middle lobe, which refers to as subtotal atelectasis as middle lobe syndrome (MLS). Vascular ring was so diagnosed and for this reason the patient has later undergone repair surgery, which was followed by improvement of the symptoms.

 

Fig. 2. Antero-posterior chest radiograph showing a right lower and middle lobe consolidation.

Fig. 3. Sagittal view of CT showing the vascular ring.

 


Fig. 4. Coronal view of CT showing the double aortic arch.

 

Discussion
We reported three clinical cases of double aortic arch with three different dominant clinical manifestations: dysphagia with reflux and choking episodes in the first one; stridor, cough and episodes of regurgitation in the second one; recurrent respiratory infections and middle lobe syndrome in the third one; confirming the heterogeneity of clinical manifestations of double aortic arch.
In general, patients with a double aortic arch tend to have symptoms at an earlier age than do patients with other types of vascular ring. The compression of the trachea and/or the oesophagus causes the onset of respiratory and/or gastrointestinal symptoms respectively 6-7.
Our third clinical case is important because we describe for the first time in literature the simultaneous presence of the MLS and double aortic arch.
The MLS generally refers to atelectasis in the right middle lobe of the lung. Pathophysiologically, there are two forms of MLS, namely obstructive and non-obstructive. Obstructive MLS can be caused by extrinsic compression of the middle lobe bronchus or by endobronchial lesions. Aspirated foreign bodies and recurrent aspiration represent the main causes of intrabronchial obstruction in childhood8.
The middle lobe syndrome and double aortic arch are rare and usually isolated congenital malformations. However, the third described patient showed a MLS associated to the presence of double aortic arch. To date, the coexistence of these malformations it has been ever described, providing to paediatricians the proof that these entities can also coexist in the same patient. However, actually, we can not clarify whether this association is “casual” or “causal”.
In our case, we hypothesized that the MLS was originally caused by obstruction from the vascular ring but it has been perpetuated over time due to recurrent respiratory infections and aspiration. A long-term follow-up, during which the child will pursue a routine airway clearance regimen with aerosol therapy and chest physiotherapy, will give us the confirmation of this association.
The diagnosis of a child with vascular ring should proceed in a stepwise fashion without obtaining excessive studies. Since most patients with recurrent or persistent respiratory problems have already had chest radiographs, a careful review of these films may be helpful. A barium esophagram historically is the study used to diagnose vascular ring and still today a double aortic arch may be suggested by bilateral incidentations, usually at slightly different levels, in addition to a large posterior incidentation6. Cross-sectional imaging with CT-angiography or magnetic resonance angiography are then crucial for the definitive diagnosis and surgical management9. Echocardiography sometimes provides accurate diagnostic imaging of anomalies such as double aortic arch by visualization of both aortic arches but it is not the gold standard of the diagnosis of vascular ring. Finally, bronchoscopic examination would show extrinsic pulsatile compression of the trachea 3.
Surgery to correct a double aortic arch is the only treatment in symptomatic patients and is reportedly well tolerated 10.
The presentation of our cases confirms the clinical heterogeneity of this vascular malformation and, to date, in the best of our knowledge, it has been ever described the coexistence of middle lobe syndrome and double aortic arch in the same patient. In conclusion, although it remains to still elucidate whether this association is “casual” or “causal”, we suggest to also investigate this vascular malformation in patients with MLS.



References
1.    Backer CL, Mongé MC, Popescu AR, et al. Vascular rings. Seminars in Pediatric Surgery. 2016; 25:165-75
2.    Seo HK, JE HG, Kang IS, et al.  Prenatal double aortic arch presenting with a right aortich arch and an anomalous artery arising from the ascending aorta. International Journal of Cardiovascular Imaging. 2010;26:165-8
3.    Turner A, Gavel G, Coutts J. Vascular rings: presentation, investigation and outcome. European Journal of Pediatrics. 2005;164:266-70
4.    Becit N, Erkut B, Karaca Y. Vascular ring: tracheoesophageal compression associated with symmetrical double aortic arch. Texas Heart Institute Journal. 2008;35:209-10
5.    Backer CL, Mavroudis C, Rigsby CK, et al. Trends in vascular ring surgery. Journal of Thoracic Cardiovascular Surgery. 2005;129:1339-47
6.    Licari A, Manca E, Rispoli A, et al. Congenital vascular rings: a clinical challenge for the pediatrician. Pediatr Pulmonology. 2015;5:511-24
7.    Krishnasarma R, Golan Mackintosh LG, Bynum F. ALTE and feeding intolerance as a presentation of double aortic arch. Case reports in Pediatrics. 2016;2016:8475917
8.    Romagnoli V, Priftis KN, De Benedictis FM. Middle lobe syndrome in children today. Pediatric Respiratory Reviews. 2014;14:188-93
9.    Etesami M, Ashwath R, Kanne J, et al. Computed tomography in the evaluation of vascular rings and slings. Insights into Imaging. 2014;5:507-21
10.    Bonnard A, Auber F, Fourcade L. Vascular ring abnormalities: a retrospective study of 62 cases. Journal od Pediatric Surgery. 2003;38:539-43
 

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www.thechild.it Four-monthly Journal of Pediatrics edited by Genetics and Pediatric Association (APIG)
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