DEPARTMENT
Case Study—Acute & Specialty Care
An Uncommon Cause for a Preschool Child’s Chronic Cough Beverly P. Giordano, MS, RN, CPNP, PMHS, Sanjeev Y. Tuli, MD, & Sonal S. Tuli, MD
KEY WORDS Chronic cough, double aortic arch
Section Editors Karin Reuter-Rice, PhD, CPNP-AC, FCCM Corresponding Editor Duke University Durham, North Carolina Terea Giannetta, DNP, RN, CPNP California State University Children’s Hospital Central California Fresno, California Maureen A. Madden, MSN, RN, CPNP-AC, CCRN, FCCM Rutgers Robert Wood Johnson Medical School New Brunswick, New Jersey Bristol Myers Squibb Children’s Hospital New Brunswick, New Jersey Beverly P. Giordano, Pediatric Nurse Practitioner, Department of Pediatrics, University of Florida, Gainesville, FL. Sanjeev Y. Tuli, Associate Professor, Department of Pediatrics, University of Florida, Gainesville, FL. Sonal S. Tuli, Associate Professor, Department of Ophthalmology, University of Florida, Gainesville, FL. Conflicts of interest: None to report. Correspondence: Beverly P. Giordano, MS, RN, CPNP, PMHS, Department of Pediatrics, University of Florida, PO Box 100383, Gainesville, FL 32610; e-mail: [email protected]. J Pediatr Health Care. (2014) 28, 267-271. 0891-5245/$36.00 Copyright Q 2014 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. Published online December 19, 2013. http://dx.doi.org/10.1016/j.pedhc.2013.10.003
www.jpedhc.org
CASE PRESENTATION A 2½-year-old white girl presented to the clinic with a 2-month history of coughing and parent-perceived ‘‘wheezing.’’ Her mother reported that the cough had developed after the child underwent a tonsillectomy and adenoidectomy (T & A) for obstructive sleep apnea (OSA) 2 months earlier. The cough was described as ‘‘wet and not croupy,’’ and it interfered with the child’s sleep. She also had activity-induced daytime coughing. The mother had tried ‘‘home remedies’’ (e.g., over-thecounter dextromethorphan preparations and the sibling’s nebulized albuterol) and reported that these interventions had little effect on the child’s cough. MEDICAL HISTORY The child’s neonatal course was unremarkable for feeding or respiratory problems. At 18 months of age, she underwent rigid esophagoscopy and diagnostic laryngoscopy for removal of a coin. A sleep study at 24 months of age demonstrated moderate OSA, which prompted the T & A at 29 months of age. Immunizations were up to date as per the recommendations of the Centers for Disease Control and Prevention. She had never had emergency department visits or clinic visits for wheezing. FAMILY/SOCIAL HISTORY The family history was positive for intermittent wheezing in the child’s sister but negative for congenital heart disease, arrhythmia, cardiomyopathy, sudden death, and tuberculosis. The child was in day care at the time she presented with the prolonged cough history. She lived with her mother, maternal grandmother, and older sister. There was no suspected exposure to tuberculosis at home or day care. REVIEW OF SYSTEMS The mother noted that the child had had a ‘‘raspy’’ voice since birth. The child’s appetite had been slightly May/June 2014
267
decreased for several weeks before this office visit, but her activity level was normal. She had occasional posttussive emesis. Fever had been documented only on the first day of this 2-month cough. The child was thought to have ‘‘wheezing’’ and noisy respirations, particularly at night. The T & A had little effect on the wheezing or noisy nocturnal respirations. The child had a negative history for significant respiratory distress, persistent dysphagia, malodorous stools, or growth failure. She had a positive history of allergies to chicken, corn products, mold, peanuts, soybeans, and amoxicillin. The child had a long history of snoring and very loud daytime respirations. A polysomnogram obtained before the T & A revealed moderate OSA. No postoperative polysomnogram had been performed. She had no symptoms to suggest other causes of chronic childhood cough (e.g., asthma, gastroesophageal reflux, cystic fibrosis, retained airway foreign body, ciliary dyskinesia syndrome, aspiration, pertussis, tracheoesophageal fistula, airway polyps, tumor, habit cough, or Tourette syndrome). PHYSICAL EXAMINATION The child actively explored the examination room during the visit, but she did not cough. She weighed 15.3 kg (86th percentile), her height was 91 cm (40th percentile), and her body mass index was 18.54 kg/m2. Her vital signs were as follows: temperature, 36.7 C; heart rate, 101 beats per minute; respiratory rate, 22 breaths per minute; blood pressure, 77/60 mm Hg; and oxygen saturation, 95% on room air. Findings of the examination of her head, eyes, ears, and nose were unremarkable. Her oropharynx examination revealed faint circumoral cyanosis but pink mucosa, a pink tongue, and a pharynx without tonsils. Her trachea was midline, and her neck was supple without masses or lymphadenopathy. Her skin was warm and dry. Nail beds were pink and normally shaped. Her voice quality was raspy, and the pitch was deeper than expected for age. Her chest was symmetrical with equal rise upon breathing. She had slightly diminished breath sounds in the left lung, but no wheezing, stridor, rhonchi, crackles, or retractions were appreciated. She had prominent upper airway inspiratory noise, similar to that heard in persons with laryngomalacia. Findings of cardiovascular and abdominal examinations were unremarkable. She had scattered red macules and papules in the diaper area. Findings of her neurologic examination were normal for her age.
tissue density posterior to and to the right of the trachea. This soft tissue density was causing a mass effect that displaced the trachea laterally to the left. The density could be seen on the lateral projection at the approximate level of the fourth thoracic vertebrae along the posterior margin of the trachea. The lungs were clear without pneumothorax, effusion, or focal consolidation, and her heart size was normal. The radiologist commented that the soft tissue density could represent a right aortic arch or other vascular anomaly, a soft tissue mass, or lymphadenopathy and recommended computed tomography (CT) of the child’s chest. The following week, the child had a chest CT scan that demonstrated a double aortic arch (DAA) with a dominant right arch (Figures 3 and 4). The left subclavian and left common carotid arteries arose from the left arch, while the right subclavian and right common carotid arteries arose from the right arch. There were no additional vascular abnormalities. The radiologist stated that the DAA accounted for the paratracheal mass that had been seen on the chest radiograph. The child was referred to a pediatric cardiologist for evaluation and management. An echocardiogram confirmed the presence of a DAA. The right arch was larger than the left arch, but there was no coarctation of the
FIGURE 1. A chest radiograph (lateral view) illustrates abnormal soft tissue density posterior to and to the right of the trachea, visible at the level of the fourth thoracic vertebrae and causing a mass effect that displaces the trachea laterally to the left. The arrow identifies the second arch that is displacing the trachea and esophagus.
DIAGNOSTIC TESTING Chest radiographs were ordered because of the diminished breath sounds, prolonged cough history, and the child’s previous foreign object ingestion episode. The radiographs (Figures 1 and 2) were negative for a foreign object but demonstrated an abnormal soft 268
Volume 28 Number 3
Journal of Pediatric Health Care
FIGURE 2. A chest radiograph (posterioranterior view) demonstrates abnormal soft tissue density (dashed arrow) displacing the narrowed trachea (solid arrow).
FIGURE 4. A computed tomography scan with contrast demonstrates tracheal compression (arrow). The esophagus is not visible because of compression by the vascular ring.
right aortic arch, and corrective surgery was recommended to relieve the tracheal ring malacia. SURGERY AND POSTOPERATIVE COURSE One month after she presented to the pediatric clinic with the chronic cough history, the child underwent dissection of the communication between the right arch and the descending thoracic aorta and resection of the left aortic arch. At the completion of the surgical FIGURE 3. A computed tomography scan without contrast demonstrates a vascular ring surrounding and compressing the trachea (arrow).
procedure, there was a 2-cm separation between the two ends of the divided arch, and the esophagus was completely mobilized. The immediate postoperative course was uneventful, and she was discharged on the fourth postoperative day. Five weeks after surgery, erythema, tenderness, and purulent discharge developed from the lower aspect of the incision on the left side of the back. Cellulitis of the thoracotomy incision and a chylous left pleural effusion were diagnosed. She was admitted for 1 week of antibiotic therapy, chest tube drainage, and respiratory status monitoring, and a low-fat diet was initiated because of the chyle leak into the chest. Two weeks later, the child presented to the pediatric nurse practitioner with left back and shoulder pain, which were the symptoms that prompted the previous admission for pleural effusion. A radiograph revealed reaccumulation of chyle in the left chest. The cardiologist subsequently managed the child as an outpatient with daily furosemide, and the low-fat diet was continued. A follow-up chest radiograph 2 weeks later demonstrated resolution of the pleural effusion. DISCUSSION Vascular rings that compress the trachea were first recognized in the 1930s (Berdon, 2000). The term ‘‘vascular ring’’ refers to any vascular anomaly that partially or completely encircles the trachea and esophagus, causing compression (Shanmugam, Macarthur, & Pollock, 2005). Isolated vascular rings are rare, with a prevalence of approximately 1 of 1000 pregnancies (Bronshtein, Lober,
www.jpedhc.org
May/June 2014
269
Auslander, & Zimmer, 2005). However, they represent about 1% to 2% of all congenital malformations of the heart and great vessels. DAA is the most common form of vascular ring and results from the persistence of the fourth pharyngeal arches during embryologic development. Six pairs of pharyngeal arch arteries develop in conjunction with the brachial pouches. The fourth pharyngeal arches form the right and left dorsal aortas. The right dorsal aorta usually involutes, and the left dorsal aorta gives rise to the aortic arch. However, if the right dorsal aorta also persists, the pair of arches meets behind the trachea and esophagus, resulting in a vascular ring that encircles these structures. DAA are grouped into three categories: right dominant, which is the case in more than 75% of patients with DAA; left dominant; or balanced (Backer & Mavroudis, 2000; Bernstein, 2011). Vascular rings are the most important vascular cause of tracheal obstruction (Berdon, 2000). The symptoms associated with vascular rings involve constriction of the trachea and/or esophagus by the Children with surrounding aortic vascular rings branches. Children with vascular rings caused by aortic caused by aortic arch arch anomalies anomalies generally generally present present with respiratory distress, dyspnea, with respiratory stridor, and swallowdistress, dyspnea, ing difficulties shortly stridor, and after birth. Respiration may be difficult if the swallowing trachea is compressed, difficulties shortly resulting in wheezing after birth. or other respiratory distress symptoms. With esophageal compression, the DAA causes the esophagus to bulge forward, which indents the trachea and exacerbates respiratory distress. Esophageal compression can also cause regurgitation and aspiration pneumonia. Solid food may not be swallowed easily, and vomiting or choking may occur. Tracheal compression can obstruct air flow and decreased mucociliary clearance of secretions, which can cause recurrent bronchopulmonary infections (Shanmugam et al., 2005). Some children, especially those with loose vascular rings, may be completely asymptomatic or have such mild symptoms that their vascular ring diagnosis is delayed until they are noted to have recurrent respiratory tract difficulties or dysphagia later in life (Bernstein, 2011; Shanmugam et al., 2005). DAA usually occurs without associated cardiovascular anomalies, but it may occur with ventricular septal defect and tetralogy of Fallot. DAA also is associated with various genetic syndromes (e.g., chromosome band 22q11 deletion in DiGeorge sequence, 270
Volume 28 Number 3
velocardiofacial or Shprintzen-Goldberg syndrome, and conotruncal anomaly face syndrome; Cury, Zeidan, & Lobato, 2013; Jones & Smith, 2005). The patient in this case report did not have facial abnormalities or other cardiac defects. Management of DAA includes a thorough evaluation of the heart to rule out additional congenital anomalies. Either a CT scan or a barium upper gastrointestinal series is indicated to determine the effect on the trachea and esophagus. Surgical intervention is indicated in all cases with respiratory symptoms or in cases with cardiac or tracheal abnormalities detected on imaging. Surgical intervention involves access to the aorta, usually via a posterolateral approach, although an anterior approach is often needed if additional cardiac anomalies need to be repaired concomitantly (Ruzmetov, Vijay, Rodefeld, Turrentine, & Brown, 2009). The nondominant portion of the vascular ring is ligated and cut. In addition, any additional fibrous bands are divided to completely mobilize the trachea and esophagus. Care must be taken to ensure that the perfusion of the carotid arteries and all limbs are checked after clamping the vessels that are to be divided before they are ligated and cut. Surgical complications can include bleeding, pleural effusion, and chylothorax. One group of thoracic surgeons who described a 22-year experience of managing patients with vascular rings reported a 10.3% incidence of chylothorax among their patients (Adams & Cipolla, 2011). Thoracic duct leaks close spontaneously in 50% of patients. Management of chylothorax is based on treating the underlying cause, decreasing chyle production, draining and obliterating the pleural space, providing appropriate fluid and nutritional replacement, and providing respiratory care. Chyle production is reduced by either instituting total parental nutrition or having children follow a fatrestricted oral diet supplemented with medium-chain triglycerides. The child in this case study required chest tube drainage, dietary management, and diuretic therapy to resolve her postoperative episodes of chylothorax and pleural effusion. SUMMARY Cough is a common presenting complaint in primary care pediatrics. Following the maxim ‘‘think horses not zebras,’’ it would have been logical for the nurse practitioner to have attributed this mother’s concern about her child’s cough to common etiologies, such as allergic rhinitis. The visit could have focused on reminding the mother that OTC cough medications are no longer recommended for young children and that giving siblings each other’s medication is not advised. However, the child’s unequal breath sounds, her unusual voice quality, a negative review of systems for other causes of chronic cough, maternal concern, and the child’s previous episode of having ingested a foreign object Journal of Pediatric Health Care
prompted the nurse practitioner to order the chest radiograph at the initial clinic visit. An undiagnosed vascular ring was never considered in the differential diagnosis at the time of the initial office visit! The child’s husky voice quality has persisted, but the cough and circumoral cyanosis resolved after the DAA was corrected. REFERENCES Adams, S. D., & Cipolla, J. (2011). Chylothorax treatment and management. Retrieved from http://emedicine.medscape.com/ article/172527-treatment. Backer, C. L., & Mavroudis, C. (2000). Congenital Heart Surgery Nomenclature and Database Project. Vascular rings, tracheal stenosis, and pectus excavatum. Annals of Thoracic Surgery, 69(Suppl), S308-S318. Berdon, W. E. (2000). Rings, slings, and other things: Vascular compression of the infant trachea updated from the midcentury to the millennium—The legacy of Robert E. Gross, MD, and Edward B.D. Neuhauser, MD. Radiology, 216, 624-632. Bernstein, D. (2011). Other congenital heart and vascular malformations. In R. M. Kliegman, R. E. Behrman, H. B. Jenson &
www.jpedhc.org
B. F. Stanton (Eds.), Nelson textbook of pediatrics (19th ed.). Philadelphia, PA: Saunders Elsevier. Bronshtein, J. M., Lober, A., Auslander, R., & Zimmer, E. Z. (2005). Sonographic diagnosis of fetal vascular rings in early pregnancy. American Journal of Cardiology, 81, 101-103. Cury, M., Zeidan, F., & Lobato, A. C. (2013). Aortic disease in the young: Genetic aneurysm syndromes, connective tissue disorders, and familial aortic aneurysms and dissections. International Journal of Vascular Medicine, 2013. article 267215. Jones, L. J., & Smith, D. W. (2005). Facial-limb defects as major features, connective tissue disorders, and miscellaneous sequences pp. 298, 555, 714. Smith’s recognizable patterns of human malformation (6th ed.). Philadelphia, PA: Elsevier Health Sciences. Ruzmetov, M., Vijay, P., Rodefeld, M. D., Turrentine, M. W., & Brown, J. W. (2009). Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: A 38-year single institution experience. Journal of Pediatric Surgery, 44(7), 1328-1332. Shanmugam, G., Macarthur, K., & Pollock, J. (2005). Surgical repair of double aortic arch: 16-year experience. Asian Cardiovascular & Thoracic Annals, 13(1), 4-10.
May/June 2014
271
Case Study—Acute & Specialty Care
An Uncommon Cause for a Preschool Child’s Chronic Cough Beverly P. Giordano, MS, RN, CPNP, PMHS, Sanjeev Y. Tuli, MD, & Sonal S. Tuli, MD
KEY WORDS Chronic cough, double aortic arch
Section Editors Karin Reuter-Rice, PhD, CPNP-AC, FCCM Corresponding Editor Duke University Durham, North Carolina Terea Giannetta, DNP, RN, CPNP California State University Children’s Hospital Central California Fresno, California Maureen A. Madden, MSN, RN, CPNP-AC, CCRN, FCCM Rutgers Robert Wood Johnson Medical School New Brunswick, New Jersey Bristol Myers Squibb Children’s Hospital New Brunswick, New Jersey Beverly P. Giordano, Pediatric Nurse Practitioner, Department of Pediatrics, University of Florida, Gainesville, FL. Sanjeev Y. Tuli, Associate Professor, Department of Pediatrics, University of Florida, Gainesville, FL. Sonal S. Tuli, Associate Professor, Department of Ophthalmology, University of Florida, Gainesville, FL. Conflicts of interest: None to report. Correspondence: Beverly P. Giordano, MS, RN, CPNP, PMHS, Department of Pediatrics, University of Florida, PO Box 100383, Gainesville, FL 32610; e-mail: [email protected]. J Pediatr Health Care. (2014) 28, 267-271. 0891-5245/$36.00 Copyright Q 2014 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. Published online December 19, 2013. http://dx.doi.org/10.1016/j.pedhc.2013.10.003
www.jpedhc.org
CASE PRESENTATION A 2½-year-old white girl presented to the clinic with a 2-month history of coughing and parent-perceived ‘‘wheezing.’’ Her mother reported that the cough had developed after the child underwent a tonsillectomy and adenoidectomy (T & A) for obstructive sleep apnea (OSA) 2 months earlier. The cough was described as ‘‘wet and not croupy,’’ and it interfered with the child’s sleep. She also had activity-induced daytime coughing. The mother had tried ‘‘home remedies’’ (e.g., over-thecounter dextromethorphan preparations and the sibling’s nebulized albuterol) and reported that these interventions had little effect on the child’s cough. MEDICAL HISTORY The child’s neonatal course was unremarkable for feeding or respiratory problems. At 18 months of age, she underwent rigid esophagoscopy and diagnostic laryngoscopy for removal of a coin. A sleep study at 24 months of age demonstrated moderate OSA, which prompted the T & A at 29 months of age. Immunizations were up to date as per the recommendations of the Centers for Disease Control and Prevention. She had never had emergency department visits or clinic visits for wheezing. FAMILY/SOCIAL HISTORY The family history was positive for intermittent wheezing in the child’s sister but negative for congenital heart disease, arrhythmia, cardiomyopathy, sudden death, and tuberculosis. The child was in day care at the time she presented with the prolonged cough history. She lived with her mother, maternal grandmother, and older sister. There was no suspected exposure to tuberculosis at home or day care. REVIEW OF SYSTEMS The mother noted that the child had had a ‘‘raspy’’ voice since birth. The child’s appetite had been slightly May/June 2014
267
decreased for several weeks before this office visit, but her activity level was normal. She had occasional posttussive emesis. Fever had been documented only on the first day of this 2-month cough. The child was thought to have ‘‘wheezing’’ and noisy respirations, particularly at night. The T & A had little effect on the wheezing or noisy nocturnal respirations. The child had a negative history for significant respiratory distress, persistent dysphagia, malodorous stools, or growth failure. She had a positive history of allergies to chicken, corn products, mold, peanuts, soybeans, and amoxicillin. The child had a long history of snoring and very loud daytime respirations. A polysomnogram obtained before the T & A revealed moderate OSA. No postoperative polysomnogram had been performed. She had no symptoms to suggest other causes of chronic childhood cough (e.g., asthma, gastroesophageal reflux, cystic fibrosis, retained airway foreign body, ciliary dyskinesia syndrome, aspiration, pertussis, tracheoesophageal fistula, airway polyps, tumor, habit cough, or Tourette syndrome). PHYSICAL EXAMINATION The child actively explored the examination room during the visit, but she did not cough. She weighed 15.3 kg (86th percentile), her height was 91 cm (40th percentile), and her body mass index was 18.54 kg/m2. Her vital signs were as follows: temperature, 36.7 C; heart rate, 101 beats per minute; respiratory rate, 22 breaths per minute; blood pressure, 77/60 mm Hg; and oxygen saturation, 95% on room air. Findings of the examination of her head, eyes, ears, and nose were unremarkable. Her oropharynx examination revealed faint circumoral cyanosis but pink mucosa, a pink tongue, and a pharynx without tonsils. Her trachea was midline, and her neck was supple without masses or lymphadenopathy. Her skin was warm and dry. Nail beds were pink and normally shaped. Her voice quality was raspy, and the pitch was deeper than expected for age. Her chest was symmetrical with equal rise upon breathing. She had slightly diminished breath sounds in the left lung, but no wheezing, stridor, rhonchi, crackles, or retractions were appreciated. She had prominent upper airway inspiratory noise, similar to that heard in persons with laryngomalacia. Findings of cardiovascular and abdominal examinations were unremarkable. She had scattered red macules and papules in the diaper area. Findings of her neurologic examination were normal for her age.
tissue density posterior to and to the right of the trachea. This soft tissue density was causing a mass effect that displaced the trachea laterally to the left. The density could be seen on the lateral projection at the approximate level of the fourth thoracic vertebrae along the posterior margin of the trachea. The lungs were clear without pneumothorax, effusion, or focal consolidation, and her heart size was normal. The radiologist commented that the soft tissue density could represent a right aortic arch or other vascular anomaly, a soft tissue mass, or lymphadenopathy and recommended computed tomography (CT) of the child’s chest. The following week, the child had a chest CT scan that demonstrated a double aortic arch (DAA) with a dominant right arch (Figures 3 and 4). The left subclavian and left common carotid arteries arose from the left arch, while the right subclavian and right common carotid arteries arose from the right arch. There were no additional vascular abnormalities. The radiologist stated that the DAA accounted for the paratracheal mass that had been seen on the chest radiograph. The child was referred to a pediatric cardiologist for evaluation and management. An echocardiogram confirmed the presence of a DAA. The right arch was larger than the left arch, but there was no coarctation of the
FIGURE 1. A chest radiograph (lateral view) illustrates abnormal soft tissue density posterior to and to the right of the trachea, visible at the level of the fourth thoracic vertebrae and causing a mass effect that displaces the trachea laterally to the left. The arrow identifies the second arch that is displacing the trachea and esophagus.
DIAGNOSTIC TESTING Chest radiographs were ordered because of the diminished breath sounds, prolonged cough history, and the child’s previous foreign object ingestion episode. The radiographs (Figures 1 and 2) were negative for a foreign object but demonstrated an abnormal soft 268
Volume 28 Number 3
Journal of Pediatric Health Care
FIGURE 2. A chest radiograph (posterioranterior view) demonstrates abnormal soft tissue density (dashed arrow) displacing the narrowed trachea (solid arrow).
FIGURE 4. A computed tomography scan with contrast demonstrates tracheal compression (arrow). The esophagus is not visible because of compression by the vascular ring.
right aortic arch, and corrective surgery was recommended to relieve the tracheal ring malacia. SURGERY AND POSTOPERATIVE COURSE One month after she presented to the pediatric clinic with the chronic cough history, the child underwent dissection of the communication between the right arch and the descending thoracic aorta and resection of the left aortic arch. At the completion of the surgical FIGURE 3. A computed tomography scan without contrast demonstrates a vascular ring surrounding and compressing the trachea (arrow).
procedure, there was a 2-cm separation between the two ends of the divided arch, and the esophagus was completely mobilized. The immediate postoperative course was uneventful, and she was discharged on the fourth postoperative day. Five weeks after surgery, erythema, tenderness, and purulent discharge developed from the lower aspect of the incision on the left side of the back. Cellulitis of the thoracotomy incision and a chylous left pleural effusion were diagnosed. She was admitted for 1 week of antibiotic therapy, chest tube drainage, and respiratory status monitoring, and a low-fat diet was initiated because of the chyle leak into the chest. Two weeks later, the child presented to the pediatric nurse practitioner with left back and shoulder pain, which were the symptoms that prompted the previous admission for pleural effusion. A radiograph revealed reaccumulation of chyle in the left chest. The cardiologist subsequently managed the child as an outpatient with daily furosemide, and the low-fat diet was continued. A follow-up chest radiograph 2 weeks later demonstrated resolution of the pleural effusion. DISCUSSION Vascular rings that compress the trachea were first recognized in the 1930s (Berdon, 2000). The term ‘‘vascular ring’’ refers to any vascular anomaly that partially or completely encircles the trachea and esophagus, causing compression (Shanmugam, Macarthur, & Pollock, 2005). Isolated vascular rings are rare, with a prevalence of approximately 1 of 1000 pregnancies (Bronshtein, Lober,
www.jpedhc.org
May/June 2014
269
Auslander, & Zimmer, 2005). However, they represent about 1% to 2% of all congenital malformations of the heart and great vessels. DAA is the most common form of vascular ring and results from the persistence of the fourth pharyngeal arches during embryologic development. Six pairs of pharyngeal arch arteries develop in conjunction with the brachial pouches. The fourth pharyngeal arches form the right and left dorsal aortas. The right dorsal aorta usually involutes, and the left dorsal aorta gives rise to the aortic arch. However, if the right dorsal aorta also persists, the pair of arches meets behind the trachea and esophagus, resulting in a vascular ring that encircles these structures. DAA are grouped into three categories: right dominant, which is the case in more than 75% of patients with DAA; left dominant; or balanced (Backer & Mavroudis, 2000; Bernstein, 2011). Vascular rings are the most important vascular cause of tracheal obstruction (Berdon, 2000). The symptoms associated with vascular rings involve constriction of the trachea and/or esophagus by the Children with surrounding aortic vascular rings branches. Children with vascular rings caused by aortic caused by aortic arch arch anomalies anomalies generally generally present present with respiratory distress, dyspnea, with respiratory stridor, and swallowdistress, dyspnea, ing difficulties shortly stridor, and after birth. Respiration may be difficult if the swallowing trachea is compressed, difficulties shortly resulting in wheezing after birth. or other respiratory distress symptoms. With esophageal compression, the DAA causes the esophagus to bulge forward, which indents the trachea and exacerbates respiratory distress. Esophageal compression can also cause regurgitation and aspiration pneumonia. Solid food may not be swallowed easily, and vomiting or choking may occur. Tracheal compression can obstruct air flow and decreased mucociliary clearance of secretions, which can cause recurrent bronchopulmonary infections (Shanmugam et al., 2005). Some children, especially those with loose vascular rings, may be completely asymptomatic or have such mild symptoms that their vascular ring diagnosis is delayed until they are noted to have recurrent respiratory tract difficulties or dysphagia later in life (Bernstein, 2011; Shanmugam et al., 2005). DAA usually occurs without associated cardiovascular anomalies, but it may occur with ventricular septal defect and tetralogy of Fallot. DAA also is associated with various genetic syndromes (e.g., chromosome band 22q11 deletion in DiGeorge sequence, 270
Volume 28 Number 3
velocardiofacial or Shprintzen-Goldberg syndrome, and conotruncal anomaly face syndrome; Cury, Zeidan, & Lobato, 2013; Jones & Smith, 2005). The patient in this case report did not have facial abnormalities or other cardiac defects. Management of DAA includes a thorough evaluation of the heart to rule out additional congenital anomalies. Either a CT scan or a barium upper gastrointestinal series is indicated to determine the effect on the trachea and esophagus. Surgical intervention is indicated in all cases with respiratory symptoms or in cases with cardiac or tracheal abnormalities detected on imaging. Surgical intervention involves access to the aorta, usually via a posterolateral approach, although an anterior approach is often needed if additional cardiac anomalies need to be repaired concomitantly (Ruzmetov, Vijay, Rodefeld, Turrentine, & Brown, 2009). The nondominant portion of the vascular ring is ligated and cut. In addition, any additional fibrous bands are divided to completely mobilize the trachea and esophagus. Care must be taken to ensure that the perfusion of the carotid arteries and all limbs are checked after clamping the vessels that are to be divided before they are ligated and cut. Surgical complications can include bleeding, pleural effusion, and chylothorax. One group of thoracic surgeons who described a 22-year experience of managing patients with vascular rings reported a 10.3% incidence of chylothorax among their patients (Adams & Cipolla, 2011). Thoracic duct leaks close spontaneously in 50% of patients. Management of chylothorax is based on treating the underlying cause, decreasing chyle production, draining and obliterating the pleural space, providing appropriate fluid and nutritional replacement, and providing respiratory care. Chyle production is reduced by either instituting total parental nutrition or having children follow a fatrestricted oral diet supplemented with medium-chain triglycerides. The child in this case study required chest tube drainage, dietary management, and diuretic therapy to resolve her postoperative episodes of chylothorax and pleural effusion. SUMMARY Cough is a common presenting complaint in primary care pediatrics. Following the maxim ‘‘think horses not zebras,’’ it would have been logical for the nurse practitioner to have attributed this mother’s concern about her child’s cough to common etiologies, such as allergic rhinitis. The visit could have focused on reminding the mother that OTC cough medications are no longer recommended for young children and that giving siblings each other’s medication is not advised. However, the child’s unequal breath sounds, her unusual voice quality, a negative review of systems for other causes of chronic cough, maternal concern, and the child’s previous episode of having ingested a foreign object Journal of Pediatric Health Care
prompted the nurse practitioner to order the chest radiograph at the initial clinic visit. An undiagnosed vascular ring was never considered in the differential diagnosis at the time of the initial office visit! The child’s husky voice quality has persisted, but the cough and circumoral cyanosis resolved after the DAA was corrected. REFERENCES Adams, S. D., & Cipolla, J. (2011). Chylothorax treatment and management. Retrieved from http://emedicine.medscape.com/ article/172527-treatment. Backer, C. L., & Mavroudis, C. (2000). Congenital Heart Surgery Nomenclature and Database Project. Vascular rings, tracheal stenosis, and pectus excavatum. Annals of Thoracic Surgery, 69(Suppl), S308-S318. Berdon, W. E. (2000). Rings, slings, and other things: Vascular compression of the infant trachea updated from the midcentury to the millennium—The legacy of Robert E. Gross, MD, and Edward B.D. Neuhauser, MD. Radiology, 216, 624-632. Bernstein, D. (2011). Other congenital heart and vascular malformations. In R. M. Kliegman, R. E. Behrman, H. B. Jenson &
www.jpedhc.org
B. F. Stanton (Eds.), Nelson textbook of pediatrics (19th ed.). Philadelphia, PA: Saunders Elsevier. Bronshtein, J. M., Lober, A., Auslander, R., & Zimmer, E. Z. (2005). Sonographic diagnosis of fetal vascular rings in early pregnancy. American Journal of Cardiology, 81, 101-103. Cury, M., Zeidan, F., & Lobato, A. C. (2013). Aortic disease in the young: Genetic aneurysm syndromes, connective tissue disorders, and familial aortic aneurysms and dissections. International Journal of Vascular Medicine, 2013. article 267215. Jones, L. J., & Smith, D. W. (2005). Facial-limb defects as major features, connective tissue disorders, and miscellaneous sequences pp. 298, 555, 714. Smith’s recognizable patterns of human malformation (6th ed.). Philadelphia, PA: Elsevier Health Sciences. Ruzmetov, M., Vijay, P., Rodefeld, M. D., Turrentine, M. W., & Brown, J. W. (2009). Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: A 38-year single institution experience. Journal of Pediatric Surgery, 44(7), 1328-1332. Shanmugam, G., Macarthur, K., & Pollock, J. (2005). Surgical repair of double aortic arch: 16-year experience. Asian Cardiovascular & Thoracic Annals, 13(1), 4-10.
May/June 2014
271
