A case of pulmonary artery sling associated with long-segment funnel trachea and bronchus suis

An infant with pulmonary sling syndrome associated with an abnormal branching of the trachea, bronchus suis, complicated by long segment intrathoracic tracheal stenosis (ie, “funnel trachea”) and concomitant tracheomalacia underwent reoperation at 5 months of age. We review the anatomy of this rare entity and describe the surgical technique to avoid the residual stenosis.     

Intrapericardial lipoma with stenosis of the left bronchus and pulmonary artery]

In a 1-year-6 month-old girl with asthma, a chest magnetic resonance imaging revealed an intrapericardial lipoma at the site of the transverse sinus behind the great arteries. The tumor compressed the left bronchus and pulmonary artery resulting in the stenosis. Under cardiopulmonary bypass, the tumor was successfully removed. The stenosis of the left bronchus and pulmonary artery were released.     

Management of pulmonary artery sling (anomalous left pulmonary artery arising from right pulmonary artery): a conservative approach.

An anomalous left pulmonary artery arising from a right pulmonary artery and passing between the trachea and oesophagus was seen in seven patients over a period of 20 years. All of them had main airway obstruction. Surgical division and re-anastomosis of the anomalous artery in front of the trachea produced an unsatisfactory result in the first two patients. The only surgical procedure undertaken in the subsequent five patients was division of a ligamentum arteriosum; this formed part of a compressing ring in one patient. Three patients, one of whom had associated tracheobronchial stenosis, became completely asymptomatic. Two others, both with tracheobronchial stenosis, still have symptoms; the older has shown considerable improvement, while the younger is only 7 months old. It is suggested that a more conservative approach to the management of this anomaly is justified. In patients with associated tracheobronchial stenosis, symptoms are most probably the result of that anomaly. Surgical division of the anomalous artery is unlikely to be beneficial, and death from postoperative complications frequently occurs. Surgical division of the anomalous artery in patients with extrinsic tracheal compression should be considered only if life is threatened, as spontaneous improvement can be anticipated. Recent advances in respiratory care should assist in the conservative management of distressed infants.     

Bronchogenic cyst in association with hypoplsia of the left pulmonary artery.

Bronchogenic cysts are congenital foregut anomalies which may cause significant respiratory symptoms in infants and children.^1,3,5 Compression of the tracheobronchial tree can lead to overinflation, atelectasis and life-threatening respiratory disease. The case presented is unique in that it was associated with hypoplasia of the left pulmonary artery.     

Cloaca, the most severe degree of imperforate anus: experience with 195 cases.

OBJECTIVE: To provide a follow-up of 195 patients with cloacal malformations seen by the author from 1959 to 1998. SUMMARY BACKGROUND DATA: Cloaca, which occurs in approximately 1 of 50,000 births, is the most complex type of imperforate anus with confluence of the rectum, vagina, and bladder in a urogenital sinus. Functional results for the bowel, the genital tract, and the urinary tract were formerly poor. Cloacal exstrophy, which is an even more complex spectrum of malformations, was uniformly fatal until 1960. In addition to imperforate anus, these babies have an omphalocele, two exstrophic bladders, between which there is an open cecum, and a blindly ending colon hanging down in the pelvis from the cecum. Although both of these diagnoses contain the word "cloaca," which is Latin for sewer, they are really two separate entities in terms of surgical management. Cloaca and cloacal exstrophy in most cases are very different anatomic problems. However, there are variants that are like a hybrid, which is the rationale for reporting together an experience with both entities. METHODS: Records were reviewed of 154 patients with cloaca and 41 patients with cloacal exstrophy to assess anorectal function, urinary continence, and sexual function where available. RESULTS: Follow-up was available in 141 cloaca patients: 82 have spontaneous bowel movements and satisfactory control, 38 use enemas to evacuate, 9 have a colostomy, 7 have fecal soiling, and 5 are too recently operated to evaluate. Regarding urinary control, 83 void spontaneously, 40 catheterize to empty, 4 have urinary diversion, 1 has a continent diversion, 5 patients are wet, and 8 are too recently operated to judge. Twenty-four patients are now adults, 17 of who have experienced coitus and 7 have not. Seven have had babies, all except one by cesarean section. Results of surgery for cloacal exstrophy are not as good, but are encouraging nonetheless for an anomaly that was uniformly fatal before 1960. Of the 41 cloacal exstrophy patients being followed, 7 have not undergone surgery. Fifteen have a colostomy; 19 had pull-through of the colon, but 3 were subsequently reversed for fecal incontinence. Most depend on enemas to evacuate. Urinary dryness was attained in 30 patients, usually by intermittent catheterization of the bladder, which was augmented with small bowel or stomach or both. Only three void voluntarily. Fifteen of the completed long-term patients wear no bag. Only three of the completed patients wear two bags. The rest have one bag. CONCLUSIONS: Imperforate anus and associated malformations in cloaca and cloacal exstrophy are not hopeless problems. A reasonable lifestyle can be achieved for most of these children with comprehensive surgical planning.     

新生儿动脉导管瘤合并左肺动脉严重压迫1例

新生儿动脉导管瘤发生率较低,以肺动脉压迫为表现的病例更为罕见。本例是1例以肺栓塞为表现的动脉导管瘤患婴,外科治疗后病情平稳。     

Anomalous origin of the left coronary artery from the pulmonary artery. Surgical repair of an unusual form

The authors present a case of a 24-year-old woman with anomalous left coronary artery originating from the pulmonary artery. Surgical correction was performed by the direct transposition of the anomalous left coronary artery from the pulmonary artery to the ascending aorta, without interposed graft. Pre- and postoperative evaluations, including angiograms and cycloergometer stress test are presented. The advantages of this technique are discussed, emphasizing the creation of a two-coronary system without the use of prosthetic, arterial or venous grafts. In the present case the left coronary artery originated from the right posterior sinus of the pulmonary artery. The patient is doing well 23 months after operation.     

Surgical correction of congenital left coronary artery-main pulmonary artery fistula in association with anomalous right coronary artery.

The association of a left coronary artery-main pulmonary artery fistula and an anomalous right coronary artery originating from the main pulmonary artery is the subject of this report. This unique combination of congenital cardiac anomalies establishes a double coronary steal from the left coronary artery, which hemodynamically represents the sole source of myocardial perfusion. The left coronary artery-main pulmonary artery fistula was closed and the coexisting anomalous right coronary artery reimplanted into the anterior aspect of the ascencing aorta. A dual coronary supply was therefore established and thus eliminated the potential threat of total myocardial ischemia should the left coronary artery become critically compromised. Patency of both the left coronary artery and the transplanted right coronary artery was documented 1 year postoperatively by aortic root angiography.     

Anomalous origin of left coronary artery from pulmonary artery in association with intracardiac lesions. Report of two cases.

Two cases are presented to illustrate the diagnostic and therapeutic dilemma when an anomalous origin of the left coronary artery from the pulmonary artery occurs with a hemodynamically significant intracardiac lesion. Hemodynamic alterations resulting from the associated intracardiac lesion may obscure the clinical features of the coronary arterial abnormality, and the appearance of normal coronary arterial distribution with left ventricular or aortic root angiography may create a false impression of a normal origin of the coronary arterial supply. A high index of suspicion may be the only lead to the detection of an anomalous origin of the left coronary artery from the pulmonary artery in association with an intracardiac lesion. In addition to the intracardiac lesion known to be associated with coronary arterial abnormalities, the question of anomalous coronary supply should be raised by the finding of poor left ventricular function or mitral insufficiency after operation (unexplainable by other causes), by the development of poor myocardial function during cardiac catheterization or anesthesia, or by the significant enlargement of a coronary artery seen during left ventriculography or supravalvular aortography.     

Sleeve resection of the bronchus and the pulmonary artery for pulmonary lesions

The tissue preserving resections for non-small-cell bronchial carcinoma can be grouped into three main categories: I. bronchoplastic procedures, II. angioplastic procedures, and III. concomitant broncho- and angioplastic procedures, and into the subgroups, standard and extended sleeve resection. The indications are; elderly patients, impaired respiratory reserve, limited tumour growth, and palliative surgery. The analysis of 229 cases yielded follow-up data in 192. The estimated 5 years survival rate was 34 per cent, 19 per cent and 14 per cent in categories I, II and III, respectively. The decrease in survival was due to a greater tumour burden. The operative mortality rate was 8.9 per cent in category I and 17 per cent in category III, such being comparable with standard or extended pneumonectomy, respectively. Surgical techniques and postoperative complications are discussed. Presented at the 82nd Annual Congress of the Japan Surgical Society, 1982, Chiba, Japan     

The prenatal diagnosis of imperforate anus with rectourinary fistula: dilated fetal colon with enterolithiasis.

The finding of extraluminal calcifications is commonly reported and usually indicates intrauterine intestinal perforation with intraperitoneal extravasation of meconium, most often associated with intestinal obstruction and/or atresias. Intraluminal calcification of meconium is more rare and appears to result from the mixing of stagnant urine and meconium in utero. The presence of the intraluminal calcifications in a dilated loop of intestine, particularly with an associated urinary tract abnormality, should suggest a rectourinary fistula. Two cases of prenatally diagnosed imperforate anus with rectourinary fistulae are reported.