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Biomedical subjects

E Kiely

Publications and source records attributed to E Kiely.

34 records · Page 2Linked to original sources

Aortopexy for tracheomalacia in oesophageal anomalies.

Forty-eight patients with repaired congenital oesophageal anomaly underwent aortopexy for significant tracheomalacia between 1980 and 1990. Indications for aortopexy included recurrent apnoea/cyanosis in 31, near fatal episodes in 16, recurrent respiratory distress and infection in 20 and worsening stridor in 15. Gastro-oesophageal reflux was noted in 30 patients, recurrent fistula in 6 and oesophageal stricture in 14. Aortopexy cured near fatal episodes in all patients and resulted in improvement of airway obstruction in 95%. The procedure failed in 2 patients due to unrecognised bronchomalacia and phrenic nerve palsy respectively. Aortopexy is the primary procedure of choice for significant tracheomalacia when associated with near fatal episodes and significant airway obstruction.

Airway Obstruction↗

Intravascular haemolysis in association with necrotising enterocolitis.

Severe haemolysis is an unusual occurrence in association with necrotising enterocolitis (NEC). Activation of the Thomsen-Friedenreich (T) antigen on the neonatal erythrocytes is proposed as the mechanism. This haemolytic process is precipitated by transfusion with serum-containing blood products, which should therefore be avoided. Once haemolysis has become established the outcome is usually fatal. This report describes the management of four neonates who had established haemolysis complicating severe NEC. T activation was confirmed in two infants, but could not be tested for in the other two. Exchange transfusion abolished the haemolysis in two patients, who survived. Despite reduced use of blood products the other two infants died. We believe that exchange transfusion was essential in the successful management of the infants who survived, and recommend early exchange transfusion when haemolysis complicates NEC.

Antigens, Tumor-Associated, Carbohydrate↗

Fibrous tissue tumours.

Thirty-three patients, ranging in age from newborn to 12 years, with fibrous tissue tumours, were treated at the Hospital for Sick Children, Great Ormond Street, London, from 1970 to 1984. Seven infants presented with tumours at birth. In 42% of cases, the tumour was noted during the 1st year of life. The anatomical distribution of the lesions was: upper extremity 6, lower extremity 5, head and neck 10, trunk 6, thoracic cavity 2 (heart 1), abdominal cavity 4 (pelvis, bladder, small bowel mesentery, and canal). Three patients had multiple tumours. Seven of the tumours were classified as sarcomas. Complete excision was possible in 28 patients, following initial biopsy in six patients of whom three were treated preoperatively with radiotherapy and/or chemotherapy. There were two deaths, one in an infant with a huge resectable pelvic tumour and the other in a child with cardiac fibroma. Recurrences occurred in 14 patients (48%), in nine of whom the recurrences were multiple. The mean time interval to recurrence was 13.8 months.

Adolescent↗

Response of the benign hypertrophied prostate to treatment with an LHRH analogue.

Twenty patients with outflow tract obstruction secondary to benign prostatic hypertrophy were treated with an LHRH analogue, buserelin, for up to 6 months. Despite maintaining castrate levels of testosterone and achieving a significant reduction in tissue levels of dihydrotestosterone, no significant improvement was seen in flow rate or residual volume.

Buserelin↗

Diagnosis and treatment of venous leakage: a curable cause of impotence.

Sixteen of 149 patients complaining of impotence of a non-neurogenic or endocrine aetiology and with a penile brachial index of greater than 0.7 failed to achieve an erection following intracavernosal injection of 30 mg papaverine and 1 mg phentolamine. All 16 were shown to have a significant venous leak using digital subtraction cavernosography. Nine of the 13 so far operated upon have had a full return of potency. Repeat cavernosography in three failures showed persistent leakage into crural veins in two and non-ligated superficial veins in one. Impotent men with a normal penile brachial index who fail to respond to papaverine and phentolamine have a venous leak, a curable cause of impotence. Surgical access should allow ligation of all identified leaking veins.

Adult↗

Esophageal atresia: five year experience with 148 cases.

One hundred forty-eight infants with abnormalities of the esophagus treated over a 5-year period were reviewed: 87% comprised esophageal atresia with distal tracheoesophageal fistula. The survival rate for infants in risk group A was 100%, for risk group B, 86%, and for risk group C, 73%. There were six infants with associated anomalies incompatible with survival. Anastomotic leaks occurred in 21% of cases, strictures in 18% and recurrent tracheoesophageal fistula in 12%. The use of braided silk for the repair was associated with a high complication rate. There was a distinct advantage in delaying surgery pending improvement in aspiration pneumonia. Low birth weight was not considered a contraindication for primary repair. Emergency ligation of the fistula was considered a more appropriate procedure than gastrostomy for the infant with associated severe respiratory distress syndrome in whom mechanical ventilation was providing difficult. Congenital cardiac anomalies were the single most common cause of mortality and correction of these anomalies should be pursued aggressively. There were definite advantages of preoperative endoscopy, particularly in identifying proximal fistulae. Elective postoperative ventilatory support for infants with a very tense anastomosis was successful in preventing leaks in five infants. There were no advantages to routine gastrostomy. Severe gastroesophageal reflux and tracheomalacia demand aggressive management. Mortality was directly related to the severity of associated congenital anomalies.

Abnormalities, Multiple↗

Gastric transposition for esophageal replacement in children.

Esophageal replacement by total gastric transposition was performed on 34 infants (32 with esophageal atresia) in the 5.5-year period from January 1981 to June 1986. There were three deaths (9%), two occurring in the early postoperative period, with the third occurring 1 year after surgery from persistent chronic respiratory problems. Fourteen infants had a totally uncomplicated course and have not required further admissions. Thirteen infants had early postoperative problems including six with delayed gastric emptying, four with anastomotic strictures requiring dilatation, and two with radiologic anastomotic leaks. Four late complications consisted of two adhesion intestinal obstructions, a perforation related to a jejunal feeding tube, and a child in whom malabsorption subsequently developed. An excellent result has been obtained in 24 infants, four doing well with only minor problems with feeding, and two are fair experiencing persisting difficulties. These results compare favorably with a large previous experience in colon interposition.

Abnormalities, Multiple↗

One hundred consecutive central venous catheters in children.

A prospective study of central venous catheterisation for parenteral treatment was carried out. The catheters were used for a variety of purposes, the majority for intravenous feeding alone. Three types of catheter were used - fine silastic tubing, Vygon Leader Caths and cuffed Hickman and Broviac catheters. The mean catheter life was 42 days with a range of 1-400 days. Sixty-two catheters were removed prior to completion of treatment. In this series the Hickman catheters were remarkably trouble-free.

Adolescent↗

Management of esophageal atresia.

A total of 303 infants with esophageal atresia and/or tracheoesophageal fistula were treated over 10 years (1980-1989). The overall survival rate was 86.5%. Associated anomalies were identified in 51.8% of patients, the most common being cardiac malformations which affected 24.4% of infants and was responsible for the majority of deaths. The dramatic increase in survival of infants with esophageal atresia in the past half century is due to improvements in pre- and postoperative management, meticulous operative technique to reduce anastomotic complications, and aggressive treatment of associated congenital anomalies.

Anastomosis, Surgical↗

Congenital alveolar capillary dysplasia and associated gastrointestinal anomalies.

Congenital alveolar capillary dysplasia is a rare cause of irreversible pulmonary hypertension with 100% mortality. We present three cases of congenital alveolar capillary dysplasia with associated gastrointestinal abnormalities. Three full-term neonates presented with pulmonary hypertension needing ventilatory support by oscillation. Of the three, two neonates subsequently needed extracorporeal membrane oxygenation. Abdominal distension associated with bilious aspirates was the gastrointestinal manifestation. One child had duodenal atresia and anorectal anomaly, one with intestinal malrotation and the other with a rare combination of intestinal malrotaion and total colonic Hirschsprung's disease. All three infants succumbed to pulmonary hypertension at mean age 34 days. The etiopathogenesis and pathology of this condition are discussed with a comprehensive review of the literature.

Capillaries↗