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Case of the fall season
Case of the Fall Season Sandra
K. Fernbach
T
A bronchogram was performed (Fig. 1). Shortly thereafter the patient was discharged. She returned 3 mo later with a 2-day history of otitis media and an upper respiratory infection. She died a few hours after admission.
HIS 3-mo-old girl was referred for the evaluation of multiple congenital anomalies. She was the product of an uncomplicated pregnancy. The family history was negative. Physical examination revealed a comfortable well-nourished infant. Musculoskeletal anomalies were limited to the right lower extremity, and included a congenital band deformity of the thigh, a clubfoot, an extra small toe, and a partially cleft great toe. Cardiovascular findings included an apical beat at the right fourth intercostal space, heart sounds heard best to the right of the sternum, and a harsh systolic murmur that radiated through the entire precordium. No cyanosis was noted.
From the Department of Diagnostic Radiology, Children’s Memorial Hospital, Northwestern University Medical School. Chicago, III. Sandra K. Fernbach, M.D.: Department of Radiology, Children’s Memorial Hospital. Reprint requests should be addressed to Sandra K. Fernbath. M.D., Department of Radiology, Children’s Memorial Hospital, 2300 Children’s Plaza, Chicago, 111.60614. 0 1981 by Grune & Stratton. Inc. 0037-198X/81/1604-0003$01.00/0
Fig. 1.
Seminars in Roenrgenology,
Vol. XVI, No. 4 (October),
1981
239
240
SANDRA
DIAGNOSIS: ASPLENIA SYNDROME BILATERAL TRACHEAL BRONCHI
WITH
In addition to dextrocardia, there is a horizontal liver and symmetry of the bronchial tree. The branching pattern present is not that of a normal right lung nor of a normal left lung. At autopsy, a dextroposed bilocular heart (single atrium and single ventricle), transposition of the great vessels, and patent ductus arteriosus with aberrant termination in the left subclavian vein were found. The tracheal origin of both upper lobe bronchi was confirmed; each lung was trilobed. No splenic tissue was present despite a thorough exploration of the abdominal cavity. There was also malrotation of the small intestine, and a bicornuate uterus. This child was autopsied in 1948, 7 yr before lvemark described the constellation of abnormalities associated with a lack of splenic tissue. He discussed the embryologic basis for the connection between conotruncal anomalies and anomalies of the interatrial and interventricular septum in patients with specific visceral anomalies, including absence of the spleen. The asplenia syndrome is also known by the eponym lvemark syndrome. Cardinal features of the asplenia syndrome are absence of the spleen, complex cardiac and great vessel malformations, and symmetrical lobation of the liver and lungs. The malpositioned abdominal contents include a midline gallbladder and malrotation of the bowel. As there is a lack of normal left-sided structures and symmetry of right-sided organs, this syndrome is also known as right isomerism. The lack of splenic tissue produces a characteristic hematologic pattern (Howell-Jolly bod-
K. FERNBACH
ies in a peripheral blood smear) and, more importantly, leaves the child vulnerable to bacterial infections. Death in early childhood is usually due to the severe cardiac malformation or to infection. More recently, antibiotic therapy and sophisticated cardiac surgery has extended the expected life. A right upper lobe tracheal bronchus is seen normally in cattle, swine, and various other fourlegged mammals. In man, tracheal bronchi are uncommon. Two types of tracheal bronchus are described. The more common displaced bronchus supplies one or more segments in a normally segmented upper lobe; the rarer supernumerary bronchus is an accessory bronchus, present in addition to the normal complement. While generally asymptomatic, tracheal bronchi are not universally so. There may be infection or tumor in the segment supplied by the aberrant bronchus. Hypoplastic changes in the trachea above or below the tracheal bronchus may cause respiratory symptoms. Ventilation and perfusion lung scan abnormalities have been reported. Several complications may arise in a patient following endotracheal intubation: intubation of the aberrant bronchus to the exclusion of the remaining lung, or occlusion of the tracheal bronchus with segmental atelectasis. This case was included in a review of malformations of the respiratory tract by Holinger et al in 1952. Although the absent spleen was appreciated, the significance of the cardiac anomalies and the symmetric lobation of the lungs was not. No record was made of lobation of the liver, nor its symmetry, although the latter is clearly seen on the radiograph.
REFERENCES I. Holinger PH, Johnston KC, Paschet VN, et al: Congenital malformations of the trachea, bronchi and lung. Ann Oto Rhino Laryngol 1952; 61:l 159-80. 2. lvemark BI: Implications of agenesis of the spleen on the pathogenesis of cone-truncus anomalies in childhood. Acta Paediatr Stand 1955; Suppl 104:44.
3. Carilli AD, The SHS, Agress H, et al: Tracheal bronchus with regional ventilation and perfusion abnormalities. Chest 1980; 78:343-6. 4. Forde WJ, Finby N: Roentgenographic features of asplenia; a teratologic syndrome of visceral symmetry. AJR 1961; 86:523-33.
K. Fernbach
T
A bronchogram was performed (Fig. 1). Shortly thereafter the patient was discharged. She returned 3 mo later with a 2-day history of otitis media and an upper respiratory infection. She died a few hours after admission.
HIS 3-mo-old girl was referred for the evaluation of multiple congenital anomalies. She was the product of an uncomplicated pregnancy. The family history was negative. Physical examination revealed a comfortable well-nourished infant. Musculoskeletal anomalies were limited to the right lower extremity, and included a congenital band deformity of the thigh, a clubfoot, an extra small toe, and a partially cleft great toe. Cardiovascular findings included an apical beat at the right fourth intercostal space, heart sounds heard best to the right of the sternum, and a harsh systolic murmur that radiated through the entire precordium. No cyanosis was noted.
From the Department of Diagnostic Radiology, Children’s Memorial Hospital, Northwestern University Medical School. Chicago, III. Sandra K. Fernbach, M.D.: Department of Radiology, Children’s Memorial Hospital. Reprint requests should be addressed to Sandra K. Fernbath. M.D., Department of Radiology, Children’s Memorial Hospital, 2300 Children’s Plaza, Chicago, 111.60614. 0 1981 by Grune & Stratton. Inc. 0037-198X/81/1604-0003$01.00/0
Fig. 1.
Seminars in Roenrgenology,
Vol. XVI, No. 4 (October),
1981
239
240
SANDRA
DIAGNOSIS: ASPLENIA SYNDROME BILATERAL TRACHEAL BRONCHI
WITH
In addition to dextrocardia, there is a horizontal liver and symmetry of the bronchial tree. The branching pattern present is not that of a normal right lung nor of a normal left lung. At autopsy, a dextroposed bilocular heart (single atrium and single ventricle), transposition of the great vessels, and patent ductus arteriosus with aberrant termination in the left subclavian vein were found. The tracheal origin of both upper lobe bronchi was confirmed; each lung was trilobed. No splenic tissue was present despite a thorough exploration of the abdominal cavity. There was also malrotation of the small intestine, and a bicornuate uterus. This child was autopsied in 1948, 7 yr before lvemark described the constellation of abnormalities associated with a lack of splenic tissue. He discussed the embryologic basis for the connection between conotruncal anomalies and anomalies of the interatrial and interventricular septum in patients with specific visceral anomalies, including absence of the spleen. The asplenia syndrome is also known by the eponym lvemark syndrome. Cardinal features of the asplenia syndrome are absence of the spleen, complex cardiac and great vessel malformations, and symmetrical lobation of the liver and lungs. The malpositioned abdominal contents include a midline gallbladder and malrotation of the bowel. As there is a lack of normal left-sided structures and symmetry of right-sided organs, this syndrome is also known as right isomerism. The lack of splenic tissue produces a characteristic hematologic pattern (Howell-Jolly bod-
K. FERNBACH
ies in a peripheral blood smear) and, more importantly, leaves the child vulnerable to bacterial infections. Death in early childhood is usually due to the severe cardiac malformation or to infection. More recently, antibiotic therapy and sophisticated cardiac surgery has extended the expected life. A right upper lobe tracheal bronchus is seen normally in cattle, swine, and various other fourlegged mammals. In man, tracheal bronchi are uncommon. Two types of tracheal bronchus are described. The more common displaced bronchus supplies one or more segments in a normally segmented upper lobe; the rarer supernumerary bronchus is an accessory bronchus, present in addition to the normal complement. While generally asymptomatic, tracheal bronchi are not universally so. There may be infection or tumor in the segment supplied by the aberrant bronchus. Hypoplastic changes in the trachea above or below the tracheal bronchus may cause respiratory symptoms. Ventilation and perfusion lung scan abnormalities have been reported. Several complications may arise in a patient following endotracheal intubation: intubation of the aberrant bronchus to the exclusion of the remaining lung, or occlusion of the tracheal bronchus with segmental atelectasis. This case was included in a review of malformations of the respiratory tract by Holinger et al in 1952. Although the absent spleen was appreciated, the significance of the cardiac anomalies and the symmetric lobation of the lungs was not. No record was made of lobation of the liver, nor its symmetry, although the latter is clearly seen on the radiograph.
REFERENCES I. Holinger PH, Johnston KC, Paschet VN, et al: Congenital malformations of the trachea, bronchi and lung. Ann Oto Rhino Laryngol 1952; 61:l 159-80. 2. lvemark BI: Implications of agenesis of the spleen on the pathogenesis of cone-truncus anomalies in childhood. Acta Paediatr Stand 1955; Suppl 104:44.
3. Carilli AD, The SHS, Agress H, et al: Tracheal bronchus with regional ventilation and perfusion abnormalities. Chest 1980; 78:343-6. 4. Forde WJ, Finby N: Roentgenographic features of asplenia; a teratologic syndrome of visceral symmetry. AJR 1961; 86:523-33.