Urogenital sinus in the female child.

A retrospective review of the urogenital sinus occuring in female children suggests five readility identifiable groups with predictable clinical associations.     

The Use of Diaphragmatic Pedicle Grafts for Reconstructive Procedures in the Esophagus and Tracheobronchial Tree

The use of well vascularized, denervated grafts of diaphragmatic muscle for repair of injury to the esophagus and tracheobronchial tree is described. The techniques for mobilization of the grafts to different areas of the chest and neck are outlined and illustrated by reference to patients with spontaneous esophageal perforation, bronchopleural fistula, and injury to the posterior tracheal membrane at the thoracic inlet. Diaphragmatic muscle is strong, elastic, well vascularized, and resistant to necrosis, with good regenerative capacity. These attributes make it the ideal material for reconstructive work in the chest and neck.     

Mustard's operation for transposition of the great arteries complicated by juxtaposition of the atrial appendages.

Six patients were seen with complete left-sided juxtaposition of the atrial appendages (LJAA) in a series of 366 consecutive Mustard operations for transposition of the great arteries. All 6 patients had situs solitus of the viscera and atria with concordant atrioventricular relationship. D-transposition was present in 3 patients and L-transposition in the other 3; all 6 had associated cardiac anomalies. The diagnosis of LJAA was first made at operation, although angiocardiographic recognition is possible. The size of the right atrium is small in the presence of this anomaly, and direct cannulation of the venae cavae and enlargement of the pulmonary venous atrium are therefore recommended.     

Assessment of fecal incontinence by manometric and radiological techniques

An assessment by anorectal manometry and defecograms has been made of 11 children born with anorectal agenesis (supralevator) in whom several surgical procedures and clinical measures were used in an attempt to minimize their incontinence.     

Experience with fibrin seal (Tisseel) in operations for congenital heart defects

Tisseel (human fibrin seal) was used in 38 children with complex congenital heart defects (2.8% of all operations for congenital heart defects) between July, 1980, and August, 1983. Control of bleeding was excellent in 31 patients, good in 6 patients, and unsatisfactory in 1 patient. Best results were achieved when Tisseel was applied on collagen substrate. If bleeding was particularly difficult to control, a combination of Tisseel with pharmacological hypotension (sodium nitroprusside) was used. We recommend the use of Tisseel to preseal Dacron conduits and to control bleeding from multiple suture lines (conduits, arterial switch operations, complex reconstructions of the pulmonary arteries), near major coronary arteries, from Gore-Tex patches in high-pressure systems, and from the raw areas of the heart at reoperation.     

Reoperation for complictions after inflow correction of transposition of the great arteries: technical considerations

A right anterolateral thoracotomy through the fifth or sixth intercostal space is described as an easier approach for reoperations after the Mustard procedure for transportation of the great arteries. Advantages of this approach compared with repeated sternotomy include easier cannulation of the superior and inferior vena cava; less extensive dissection of adhesions, resulting in decreased postoperative bleeding; reduced risk of injury to the coronary asteries and the right phrenic nerve; and improved exposure of intracardiac pathways and the tricuspid valve.     

Infradiaphragmatic Total Anomalous Pulmonary Venous Drainage: Surgical Treatment and Long-Term Results

Since 1971, 20 infants (1 day to 3 months old) with infradiaphragmatic total anomalous pulmonary venous drainage (TAPVD) have undergone repair at The Hospital for Sick Children, Great Ormond Street, London. All patients were seen with severe cyanosis (mean partial pressure of arterial oxygen, 35.8 mm Hg) and pulmonary edema. All had a persistent ductus arteriosus. Right-sided pressures were equal to or greater than systemic in 17 patients.During surgical repair, the confluence of the pulmonary veins was anastomosed to the left atrium. The descending vein was ligated in 11, divided in 4, and left open in 5 patients. Atrial septal defects were closed with a patch in 6 and sutured in 14. Eight patients died (40%). Acidosis on admission was significantly greater among nonsurvivors (mean pH, 7.29 ± 0.02) than survivors (mean pH, 7.37 ± 0.02) (p < 0.05).Twelve survivors are well four months to 8 years after operation. All are asymptomatic and none requires cardiac medication. Six have undergone repeat catheterization, demonstrating normal pressures and no shunts. We conclude that the mortality of patients with infradiaphragmatic TAPVD depends mainly on the condition of the child on admission.     

Early and late results of surgical correction of pulmonary artery sling.

Since 1976, we have operated on 4 children with pulmonary vascular sling. They were 5, 12, 19, and 54 months old. All patients were seen initially with severe stridor. Diagnosis was made by barium swallow in all 4. Each child was operated on through a left thoracotomy; the left pulmonary artery was dissected deep between the trachea and esophagus. Systemic heparinization and microsurgical techniques were used. All patients improved after operation. Radioisotope pulmonary ventilation/perfusion scans were performed 9, 9, 14, and 21 months after operation. Decreased ventilation was noted in the right upper lobe of one scan; the other three ventilation scans were normal. Perfusion scans showed good patency of both pulmonary arteries in all 4 patients. It is concluded that pulmonary vascular slings should be treated surgically as soon as diagnosed. Delayed operation can lead to severe tracheomalacia, as demonstrated in our 54-month-old patient.     

Levatorplasty: A secondary operation for fecal incontinence following primary operation for anorectal agenesis.

A procedure is described which combines the posterior liberation of the levator with the levator plication. This operation was performed in 15 children, born with anorectal agenesis, all of whom were incontinent of feces after primary operation. Four of the 15 children became fully continent following levatorplasty, seven patients now have only occasional soiling, and four patients continue to soil persistently. In all the cases, a good correlation was found between bowel control after levatorplasty and anal canal pressures. Normal resting anal canal pressure was as significant as the voluntary squeeze pressure in relation to continence.     

Postoperative Angiographic Assessment of Modified Blalock-Taussig Shunts Using Expanded Polytetrafluoroethylene (Gore-Tex)

Thirty-six of 87 modified Blalock-Taussig shunts done with expanded polytetrafluoroethylene (Gore-Tex) were restudied angiocardiographically. In 7 patients the study was carried out within 1 month of the shunt operation because the patients failed to make satisfactory clinical progress. Two shunts were occluded and 1 was itenosed; all 3 were in neonates. The remaining 29 patients were reinvestigated electively between 5 and 29 months postoperatively and had a 97% shunt patency rate. Because of the rather high incidence of irregular or stenosed shunts among neonates with 4 mm conduits, we now prefer to use a larger conduit even in this age group.     

Tracheal compression by the aortic arch following right pneumonectomy in infancy

Uneventful pneumonectomy was carrried out in a 10-month-old infant for hypoplastic right lung associated with esophageal origin of the right main bronchus. Eight months after operation, symptoms of tracheal compression began. This compression was due to the aortic arch, which was stretched across the lower trachea following the displacement of the heart to the posterior right chest after pneumonectomy. Successful relief of the airway obstruction was achieved by inserting a 20 mm woven Dacron graft between the ascending and the descending aorta, with division of the aortic arch between the left carotid and the left subclavian arteries. An aortogram performed two years after operation confirmed good patency of the graft and normal flow through the descending aorta. The child remains well two years after operation but continues to have mild residual tracheomalacia and limited exercise tolerance, compatible with the presence of only one lung.     

Iatrogenic ileal atresia secondary to clamping of an occult omphalocele.

Clamping of an occult omphalocele has led to complete division of an entrapped loop of ileum in two instances. The proximal end has been sealed in the process, producing an iatrogenic ileal atresia. The clinical picture in both instances differed from that usually found with ileal atresia. Escape of a small amount of meconium from the transected cord was noted in one, and the stump of the cord in the other appeared red and engorged. Clamping the umbilical cord routinely at least 5 cm from the abdominal wall is recommended.     

Is there a decline in neonatal surgery?

The apparent severe fall in admissions to the neonatal surgical unit in the Sheffield Children's Hospital, after corrections have been made for known changes in the disposal of case material in the region, is almost completely in line with the falling curve of the birth rate. The constancy of this curve is due to the fact that the reduction in cases of neural tube defects is offset by an increase in other neonatal surgical problems.     

Vertebral anomalies associated with esophageal atresia and tracheoesophageal fistula with reference to the initial operative mortality

In a series of 40 patients with esophageal atresia and tracheoesophageal fistula, the incidence of musculoskeletal anomalies was 45% (18 patients). An extra mesodermal segment was found in 15 patients (13 paired thoracic vertebrae and 13 paired ribs in nine patients; six lumbar vertebrae in 6 patients). The mortality in grade A infants with an extra mesodermal segment was significantly higher than in those infants with a normal spine (p > 0.01). It is suggested that segmentation or abnormal segmentation of the embryo may be important as a cause of esophageal atresia.     

The clinical features and treatment of fetus in fetu: two case reports and a review of the literature.

The case reports of two children with the condition of “fetus in fetu” are added to the 18 cases already in the literature.     

Ongoing cirrhosis after successful porticoenterostomy in infants with biliary atresia.

Thirty-one patients were treated by portoenterostomy procedures for extrahepatic biliary atresia. In 20, there was unequivocal evidence of bile drainage. Eleven of these 20 were restudied from 3 to 23 mo postoperatively by liver biopsy. The clinical condition of most of these infants was satisfactory. Despite absence of clinical jaundice, normal rose bengal scan, and normal liver function studies, eight showed progressive degrees of hepatic fibrosis when compared to the results of the biopsy taken at the time of portoenterostomy. Two patients showed stable histology and one, after progressing on a second biopsy, had normal liver histology on a third study. The implications of progressive hepatic histopathology despite bile drainage are discussed.