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Early diagnosis and surgical treatment of children with congenital vascular rings and accompanying heart lesions
Early Diagnosis and Surgical Treatment of Children with Congenital Vascular Rings and Accompanying Heart Lesions By E. Zdebska, l
Methods
treatment scribed cular
in rings.
according Among children
of early
and
42
diagnosis,
postoperative
children
with
The
ring
to
Cooley’s
M. Markowa,
operative
were
and
W.
cause of severe
care are de: congenital
types
recognized
required
I. Sm&ka,
symptoms
vas-
grouped
of
Miezyirski
or noticeable esophagus
and
the postoperative
period
early
care is especially
important.
compressive trachea.
In
respiratory
classification. vascular
surgical
rings
treatment
34 be-
INDEX
WORD:
Vascular
rings.
C
ONGENITAL MALFORMATIONS of the aortic arch described as vascular ring are the most uncommon heart lesions in chi1dhood.‘-6 Although these anomalies involve mainly the circulatory system, the most significant symptoms concern the respiratory and digestive tract. Three types of compressing vascular rings are presented: 1) double aortic arch, 2) retroesophageal subclavian artery, 3) right aortic arch with left-posterior arterial duct (Cooley classification). CASE
STUDIES
from November 1971 to January 1976. 42 children with congenital vascular rings were observed. Thirty-four of them required operative release of the ring because of compressive symptoms. This is over 12 per cent of all cardiac operations performed without using the extracorporal circulation during this period. This group of patients is summarized in Table I. Clinical symptoms appeared in the first weeks of life. Recurrent respiratory distress, pneumonia, spastic bronchitis. stridor and dyspnea were most common in type a-double aortic arch. Dysphagia, vomiting, constant feeding distress, and difficulty in swallowing were mostly observed in type b-retroesophaegeal subclavian artery.‘-13 Ten of our patients had constant or In the period
repeated cardiac failure (Fig. 1). The accompanying malformations occurring with vascular rings in our series are summarized in Fig. 2. Figure 3 shows all additional heart malformations. The most common was patent ductus arteriosus (PDA), which occurred in 17 children. ventricular septal defect (VSD). in 7 children. and 2 children had severe pulmonary hypertension. Seven children in this series had tetralogy of Fallot (TOF).
(Fig. 3). The clinical findings DIAGNOSIS
were physical AND
deficiencies
and mental
retardation.
TREATMENT
The diagnoses were based on the significant clinical findings, x-ray examination of the esophagus with barium contrast, and subsequent angiocardiography. The ring was dissected in the region where the aortic arch was hypoplastic. In our series it was the anterior arch in 15 children, and posterior in one child. From the Children’s Surgical Clinic. Medical Academ?, Krakbw, Poland. Presented before the XXIII Annual International Congress of the British Association of Paediatric Surgeons, Sheffield. England, Ju1.v 7-9. 1976 and Warsaw, Poland, July I I-13. 1976. Address Jbr reprint requests: Eugenia Zdebska, M.D., Children’s Surgical Pediatrics, Medical Academy, ul. Wielicka 265, 30-663 Krakbtis. Poland. :L 1977 by Grune & Stratton, Inc. Journo/ofPsdiofricSvrgery,Vol.
12, No. 1 (February), 1977
Clinic, Insriture 01
121
ZDEBSKA
122
Table 1.
a.) Double b.) c.)
oortic
Retroesophageal Right
aortic
arch with
Al.
Data Summary of Children Series
arch subclavion
ET
artery
Number
Sex
Beginning
of cases
M:F
of SvmDtomr
16
9:7
2wk
1Omo
11
5%
1 wk
25 mo
Age Ooerotive
left ductus
orteriosus Total
7
4:3
34
l&18
1 mo
2.0 yr
2wk
2.2 yr
The essential detail was the place of dissection. If two left arteries arose from the minor-anterior arch the ring was divided between the left subclavian artery and the descending aorta. If one left subclavian artery arose from the anterior arch the ring was released leaving the artery near the descending aorta. The ductus arteriosus, patent or not, was divided in all children with double aortic arch and the posterior ductus arteriosus with the arch located on the right side. In only 8 children with retroesophageal subclavian artery was ligation of the ductus employed. After having sufficiently removed the esophageal and tracheal adhesions, there was no necessity to suture the arteries to the posterior wall of the sternum. Among the children operated on for vascular rings, 26 had associated heart lesions. In 5 children with tetralogy of Fallot, release of the vascular ring was combined with systemic pulmonary anastomosis using the divided right subclavian artery to the left pulmonary artery in 3 children. In two cases the anterior hypoplastic aortic arch with left subclavian artery was used for anastomosis with the left pulmonary branch. In two other children with ventricular septal defect and significant pulmonary hypertension, pulmonary arterybanding was performed. HISTOLOGICAL
FINDINGS
In view of a great age range of operated children with different ring forms and different degrees of compression on the esophagus and trachea, histological studies of lung specimens and resected vessels were made. Fragments were competently colored with hematoxylin and eosin and Masson’s, van Gierson’s, Gomory’s methods, and with orcein for elastic fibers. In the first group with double aortic arch, relative silver absorptive fiber changes were found. These alterations were expressed by intensification of different degrees in intervesical septa mostly around small blood vessels. The above alterations were strongly marked among older children, above 1 yr of age
a
b
@J
respiratory
Cl
digestive
q
cardiac Fig. 1.
Prominent clinical symptoms.
CONGENITAL
VASCULAR
123
RINGS
heart
26
a
b
C
renal nervous
13
a
b
c
others Fig. 2.
Total 47
Associated anomalies.
7
bc
llsD
7
P
rota137
Fig. 3.
Associated heart lesions.
and among children with coexisting congenital heart lesions as PDA and VSD. In 16 children no histological alteration was found. In the second group with postesophageal subclavian artery, right sided aortic arch, and posterior arterial duct, inflammatory alterations of interstitial nature were found with thickening and swelling of intervesical septa, endothelial vessel proliferation, and inflammatory groupings consisting of lymphocytic-type uninuclear cells (Fig. 4) and distinct increasing silver absorptive fibers.
Fig. 4. Thickened walls of pulmonary endothelial cells, X,450,H and E.
alveolus
infiltrated
by lymphocytes
and
proliferated
ZDEBSKA
124
ET AL.
RESULTS
Out of 34 children in whom the ring was divided, 31 survived. Pulmonary infections as well as dysphagia and circulatory problems subsided. Control x-rays done from 3 yr to 6 mo after the operation revealed slightly indented esophagus in only 10 children. One child with double aortic arch and Down’s Syndrome died 3 wk after release of the ring with a severe pneumonia. Two other children with multiple associated anomalies such as severe tetralogy of Fallot, common superior vena cava, situs inversus, and transposition of atria died with signs of brain edema. One child with double aortic arch and tetralogy of Fallot returned two yr after the division of the ring and systemic-pulmonary anastomosis because of insufficient shunt. A second anastomosis was performed with good results. CONCLUSIONS
The increasing number of children with vascular rings operated during last 3 yr result from the fact that more attention is paid to recurrent signs of respiratory and esophageal symptoms and the use of contrast examinations of esophagus and angiocardiography. If in these children an accompanying malformation of the circulatory system is encountered, it should be simultaneously corrected or other palliative surgery should be performed, depending on the age of child and the type of accompanying heart anomaly. REFERENCES I. Cole WH: Double aortic arch. New York. Appieton-Century-Crofts. 1956, p 133 2. Cooley DA. Hallman CL: Surgical treatment of congenital heart disease. Philadelphia. Foboger 1966, p 13 3. Giiycka et al: Wady rozwojowe &.tku aorty. Pediatria Polska 1:47, 1972 4. Gross RE: Present status of surgery for treatment of congenital cardiovascular malformations. Bull, NY Acad Med 33:305, 1957 5. Gross RE: Arterial malformation which cause compression of the trachea or esophagus. Circulation I I : 124, I955 6. Mustard WT et al.: Cardiovascular surgery in first year of life. J Thorac Cardiovasc Surg 9:764, 1970 7. Gerbode F et al: Surgery lesions of the heart and great 1970
of congenital vessels, 1:14,
8. Downes
JJ et al: Acute
respiratory
in infants foliowing cardiovascular Thorac Cardiovasc Surg 1:59, 1970 9. Hallman
GL,
Coley
DA:
aortic vascular ring. Surgical Arch Surg 88:666, I964 10. Izukowa T, Scott fifth aortic arch in men. Il90-1195, 1973 1 I. Mahoney Surgery
failure
surgery.
J
Congenital
consideration.
ME: Persistens left Brit Heart J 35:ll.
E, Manning
JA: Vascular
ring.
55:15, 1964
12. Mustard
WT: Vascular
ring. Ped Surg
427, 1962. 13. Gross RE: Thoracic surgery J Thor Cardiovasc 2: 152, 1964 14. Van Praagh fifth arterial arch 241279, 1969
for infants.
R, Van Praagh S: Persistens in men. Amer Jour Cardio!
1:
Methods
treatment scribed cular
in rings.
according Among children
of early
and
42
diagnosis,
postoperative
children
with
The
ring
to
Cooley’s
M. Markowa,
operative
were
and
W.
cause of severe
care are de: congenital
types
recognized
required
I. Sm&ka,
symptoms
vas-
grouped
of
Miezyirski
or noticeable esophagus
and
the postoperative
period
early
care is especially
important.
compressive trachea.
In
respiratory
classification. vascular
surgical
rings
treatment
34 be-
INDEX
WORD:
Vascular
rings.
C
ONGENITAL MALFORMATIONS of the aortic arch described as vascular ring are the most uncommon heart lesions in chi1dhood.‘-6 Although these anomalies involve mainly the circulatory system, the most significant symptoms concern the respiratory and digestive tract. Three types of compressing vascular rings are presented: 1) double aortic arch, 2) retroesophageal subclavian artery, 3) right aortic arch with left-posterior arterial duct (Cooley classification). CASE
STUDIES
from November 1971 to January 1976. 42 children with congenital vascular rings were observed. Thirty-four of them required operative release of the ring because of compressive symptoms. This is over 12 per cent of all cardiac operations performed without using the extracorporal circulation during this period. This group of patients is summarized in Table I. Clinical symptoms appeared in the first weeks of life. Recurrent respiratory distress, pneumonia, spastic bronchitis. stridor and dyspnea were most common in type a-double aortic arch. Dysphagia, vomiting, constant feeding distress, and difficulty in swallowing were mostly observed in type b-retroesophaegeal subclavian artery.‘-13 Ten of our patients had constant or In the period
repeated cardiac failure (Fig. 1). The accompanying malformations occurring with vascular rings in our series are summarized in Fig. 2. Figure 3 shows all additional heart malformations. The most common was patent ductus arteriosus (PDA), which occurred in 17 children. ventricular septal defect (VSD). in 7 children. and 2 children had severe pulmonary hypertension. Seven children in this series had tetralogy of Fallot (TOF).
(Fig. 3). The clinical findings DIAGNOSIS
were physical AND
deficiencies
and mental
retardation.
TREATMENT
The diagnoses were based on the significant clinical findings, x-ray examination of the esophagus with barium contrast, and subsequent angiocardiography. The ring was dissected in the region where the aortic arch was hypoplastic. In our series it was the anterior arch in 15 children, and posterior in one child. From the Children’s Surgical Clinic. Medical Academ?, Krakbw, Poland. Presented before the XXIII Annual International Congress of the British Association of Paediatric Surgeons, Sheffield. England, Ju1.v 7-9. 1976 and Warsaw, Poland, July I I-13. 1976. Address Jbr reprint requests: Eugenia Zdebska, M.D., Children’s Surgical Pediatrics, Medical Academy, ul. Wielicka 265, 30-663 Krakbtis. Poland. :L 1977 by Grune & Stratton, Inc. Journo/ofPsdiofricSvrgery,Vol.
12, No. 1 (February), 1977
Clinic, Insriture 01
121
ZDEBSKA
122
Table 1.
a.) Double b.) c.)
oortic
Retroesophageal Right
aortic
arch with
Al.
Data Summary of Children Series
arch subclavion
ET
artery
Number
Sex
Beginning
of cases
M:F
of SvmDtomr
16
9:7
2wk
1Omo
11
5%
1 wk
25 mo
Age Ooerotive
left ductus
orteriosus Total
7
4:3
34
l&18
1 mo
2.0 yr
2wk
2.2 yr
The essential detail was the place of dissection. If two left arteries arose from the minor-anterior arch the ring was divided between the left subclavian artery and the descending aorta. If one left subclavian artery arose from the anterior arch the ring was released leaving the artery near the descending aorta. The ductus arteriosus, patent or not, was divided in all children with double aortic arch and the posterior ductus arteriosus with the arch located on the right side. In only 8 children with retroesophageal subclavian artery was ligation of the ductus employed. After having sufficiently removed the esophageal and tracheal adhesions, there was no necessity to suture the arteries to the posterior wall of the sternum. Among the children operated on for vascular rings, 26 had associated heart lesions. In 5 children with tetralogy of Fallot, release of the vascular ring was combined with systemic pulmonary anastomosis using the divided right subclavian artery to the left pulmonary artery in 3 children. In two cases the anterior hypoplastic aortic arch with left subclavian artery was used for anastomosis with the left pulmonary branch. In two other children with ventricular septal defect and significant pulmonary hypertension, pulmonary arterybanding was performed. HISTOLOGICAL
FINDINGS
In view of a great age range of operated children with different ring forms and different degrees of compression on the esophagus and trachea, histological studies of lung specimens and resected vessels were made. Fragments were competently colored with hematoxylin and eosin and Masson’s, van Gierson’s, Gomory’s methods, and with orcein for elastic fibers. In the first group with double aortic arch, relative silver absorptive fiber changes were found. These alterations were expressed by intensification of different degrees in intervesical septa mostly around small blood vessels. The above alterations were strongly marked among older children, above 1 yr of age
a
b
@J
respiratory
Cl
digestive
q
cardiac Fig. 1.
Prominent clinical symptoms.
CONGENITAL
VASCULAR
123
RINGS
heart
26
a
b
C
renal nervous
13
a
b
c
others Fig. 2.
Total 47
Associated anomalies.
7
bc
llsD
7
P
rota137
Fig. 3.
Associated heart lesions.
and among children with coexisting congenital heart lesions as PDA and VSD. In 16 children no histological alteration was found. In the second group with postesophageal subclavian artery, right sided aortic arch, and posterior arterial duct, inflammatory alterations of interstitial nature were found with thickening and swelling of intervesical septa, endothelial vessel proliferation, and inflammatory groupings consisting of lymphocytic-type uninuclear cells (Fig. 4) and distinct increasing silver absorptive fibers.
Fig. 4. Thickened walls of pulmonary endothelial cells, X,450,H and E.
alveolus
infiltrated
by lymphocytes
and
proliferated
ZDEBSKA
124
ET AL.
RESULTS
Out of 34 children in whom the ring was divided, 31 survived. Pulmonary infections as well as dysphagia and circulatory problems subsided. Control x-rays done from 3 yr to 6 mo after the operation revealed slightly indented esophagus in only 10 children. One child with double aortic arch and Down’s Syndrome died 3 wk after release of the ring with a severe pneumonia. Two other children with multiple associated anomalies such as severe tetralogy of Fallot, common superior vena cava, situs inversus, and transposition of atria died with signs of brain edema. One child with double aortic arch and tetralogy of Fallot returned two yr after the division of the ring and systemic-pulmonary anastomosis because of insufficient shunt. A second anastomosis was performed with good results. CONCLUSIONS
The increasing number of children with vascular rings operated during last 3 yr result from the fact that more attention is paid to recurrent signs of respiratory and esophageal symptoms and the use of contrast examinations of esophagus and angiocardiography. If in these children an accompanying malformation of the circulatory system is encountered, it should be simultaneously corrected or other palliative surgery should be performed, depending on the age of child and the type of accompanying heart anomaly. REFERENCES I. Cole WH: Double aortic arch. New York. Appieton-Century-Crofts. 1956, p 133 2. Cooley DA. Hallman CL: Surgical treatment of congenital heart disease. Philadelphia. Foboger 1966, p 13 3. Giiycka et al: Wady rozwojowe &.tku aorty. Pediatria Polska 1:47, 1972 4. Gross RE: Present status of surgery for treatment of congenital cardiovascular malformations. Bull, NY Acad Med 33:305, 1957 5. Gross RE: Arterial malformation which cause compression of the trachea or esophagus. Circulation I I : 124, I955 6. Mustard WT et al.: Cardiovascular surgery in first year of life. J Thorac Cardiovasc Surg 9:764, 1970 7. Gerbode F et al: Surgery lesions of the heart and great 1970
of congenital vessels, 1:14,
8. Downes
JJ et al: Acute
respiratory
in infants foliowing cardiovascular Thorac Cardiovasc Surg 1:59, 1970 9. Hallman
GL,
Coley
DA:
aortic vascular ring. Surgical Arch Surg 88:666, I964 10. Izukowa T, Scott fifth aortic arch in men. Il90-1195, 1973 1 I. Mahoney Surgery
failure
surgery.
J
Congenital
consideration.
ME: Persistens left Brit Heart J 35:ll.
E, Manning
JA: Vascular
ring.
55:15, 1964
12. Mustard
WT: Vascular
ring. Ped Surg
427, 1962. 13. Gross RE: Thoracic surgery J Thor Cardiovasc 2: 152, 1964 14. Van Praagh fifth arterial arch 241279, 1969
for infants.
R, Van Praagh S: Persistens in men. Amer Jour Cardio!
1: