Heart transplantation in children

/NTERNATIONAL ABSTRACTS

Complement Activation During Prolonged Extracorporeal Membrane Oxygenation. S.H. Westfall, C. Stephens, IC Kesler, et al. Surgery 110:887-891, (November), 1991. This study measures complement activation in newborn patients with respiratory failure treated with extracorporeal membrane oxygenation (ECMO) over a period of 96 hours. Twenty-one of these infants were assigned either to a control group managed without steroids or a group receiving 30 mg/kg of intravenous methylprednisolone before ECMO. Steroids significantly shortened the time on ECMO and the time of ventilator management after ECMO. However, they did not affect survival or total hospital time. Surprisingly, steroids were found to enhance paradoxical activation of C3 and C5. The authors conclude that the inverse relationship between complement activation and a shortened ECMO course indicates a more probable benefit of steroids to lung repair rather than an important role of complement activation in lung surgery.--Thomas F. TracT, Jr Studies in Fetal Wound Healing. M. Longaker, E. Chiu, S. Adzick, etaL Ann Surg 213:292-296, (April), 1991. Midgestation fetal wound healing proceeds without scaring or fibrosis. The fetal wound has been shown to be rich in extracellular hyaluronic acid (HA). This study compares fetal and adult wounds for concentrations of extracellular HA using a refined [35]streptavidin hyaluronic acid-binding protein assay. Adult wound HA peaked early and was undetectable by 3 days postoperatively. Fetal wound HA remained significantly elevated from 7 to 21 days. The prolonged presence of HA provides an environment promoting a highly ordered matrix in fetal wounds. Collagen is rapidly deposited in a manner indistinguishable from unwounded tissues. HA may mediate chemical signals within the wound allowing collagen deposition. Clinically, it may be possible to manipulate wound healing by the prolonged application of HA or HA-protein complexes.--Edward G. Ford The Fetus as a Patient. M. Harrison and S. Adzick. Ann Surg 213:279-291, (April), 1991. The authors present the current state of the art of fetal surgery including the rationale for intervention, the development of open operative techniques, details of the operative techniques, and preoperative and postoperative care. Eighteen women have undergone open fetal surgery for congenital diaphragmatic hernia, sacrococcygeal teratoma, bladder outlet obstruction, and cystic adenomatoid malformation. Fifteen procedures were technically successful and resulted in a viable fetus. The success of the fetal surgery program is related to a team effort, ongoing research activity, and establishment of procedures and personal expertise in animal models.--Edward G. Ford THORAX

Congenital Tracheal Stenosis With Unilateral Pulmonary Agenesis. T.R. Weber, R.H. Connors, and T.F. TracT. Ann Surg 213:70-74, (January), 1991. Congential tracheal stenosis with unilateral pulmonary agenesis is rare and generally fatal. Repair is difficult because the stenosis may involve long (or total) segments of the trachea. This series of five patients details reconstruction with rib cartilage graft tracheoplasty (RCGT). One patient was reconstructed with segmental resection and end-to-end anastomosis. Of the four patients undergoing RCGT, one required ECMO support. All patients survived the operative procedure; three are long-term survivors (3 to 6 years). One patient died of viral pneumonitis with copious secretions complicating ventilatory management. One patient died of

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aortotracheal fistula. The authors detail preoperative and postoperative evaluations and care and discuss specifics of the operative procedure.--Edward G. Ford An Experimental Model for the Surgical Correction of Tracheomalacia.A.R Shaha, C. Burnett, T. DiMaio, et al. Am J Surg 162:417-420, (October), 1991. Tracheomalacia, resulting from weakness of anterior cartilaginous support, is a significant tracheal complication for which there is no perfect reconstruction. In the case of patients with tracheomalucia there is significant morbidity due to prolonged intubation or tracheostomy for prolonged stenting and support. In this study, tracheomalacia was created in eight dogs by submucosal resection of six to seven rings of tracheal cartilage. The tracheal walls were then reconstructed with polytetrafluoroethylene (PTFE) grafts that had ringed supports. Reconstruction was performed at the time of resection. At 4 to 6 months, the tracheas were excised and evaluated. One dog died postoperatively due to erosion of the graft into the tracheal lumen. The authors report no evidence of tracheal narrowing. Despite histological evidence ofa submucosal inf/ammatory response and fibrosis, there was good incorporation of the graft in the tracheal wall. Future success with this technique may relate to delayed versus immediate reconstruction, or the ability to use these ringed grafts as external stents when sutured to soft tracheal cartilage. This study did not address tracheal growth with these grafts in place.--Thomas F. TracT, .11" HEART A N D GREAT VESSELS

Patterns of Ductal Tissue in Coarctation of the Aorta in the First Three Months of Life. G.A. Russell, P.J. Berry, K. Watterson, et aL J Thorac Cardiovasc Surg 102:596-601, (October), 199l. The presence of residual ductal tissue following repair of coarctation of the aorta is cited as one reason for recurrence of coarctation. This study defined the presence and pattern of ductal tissue in aortic segments from 23 patients less than 3 months of age who underwent aortic resection for coarctation. In this group, extensive excision of the coarctation included a wide margin of descending aorta beyond the ductus arteriosus. Histological examination of 22 specimens demonstrated a circumferential sling of ductal tissue that extended out from the ductus arteriosus and surrounded the aorta at the level of the eoarctation shelf. In 15 specimens one or two tongue-like prolongations of ductal tissue extended distally from the circumferential sling. Incomplete excision of ductal tissue was found in 11 specimens. These patterns of ductal tissue may have significant implications for restenosis, especially with subclavian flap aortoplasty which tends to occur in infants whose operations are performed at less than 4 to 6 weeks of age when there is still time for further maturation of ductal tissue. Also, restenosis tends to occur at the apex of the flap which is where residual ductal tissue is present according to this study. This study also demonstrates evidence that may impact on the success of balloon angioplasty for coarctation of the aorta.--Thomas F. TracT, Jr Heart Transplantation in Children. W. Merrill, W. Frist, J. Stewart, et aL Ann Surg 213:393-400, (May), 1991. Cardiac transplantation (OCT) in infants and children is unique because of the complex congenital abnormalities of the recipient, necessity to limit steroid use to prevent growth arrest, and the technical danger of repeated endomyocardial biopsies. Sixteen infants and children underwent OCT for complex congenital heart disease or cardiomyopathy (hypoplastic left heart [5], critical aortic stenosis and small left ventricle [1], complex cyanotic heart disease [5], idiopathic myopathy [3]). The mean waiting list time was 29 -+

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INTERNATIONAL ABSTRACTS

28 days. Patients ;with hypoplastic left hearts were transplanted under deep hypothermia and circulatory axrest;all others underwent moderate hypothermia and bypass. Donors were matched to body weight and ABO group. Two of five patients with hypoplastic left heart disease died in the operating room. One of six patients with complex cyanotic disease died at 1 week with right ventricular failure and persistently elevated pulmonary vascular resistance. There were no late deaths; survival is 79% -+ 11%. Sixty-two percent of patients were free of rejection at 1 month, and 26% at 3 months. Twenty episodes of infection occured, with no deaths (pulmonary [4], urinary tract [4], blood-borne [3], central line [1], other [7]). There were no fungal infections. Hypertension requiring treatment is noted in 6 of 12 long-term follow-ups. Linear growth was achieved in all eight surviving neonates and children under 6 years old by excluding prednisone from the immunosuppressive regimen. These children are treated with CsA and AZA. AZA is discontinued at 1 year of age in the neonate OCT patients. All long-term survivors are free of symptoms related to OCT and engage in normal activities for age.--Edward G. Ford A L I M E N T A R Y TRACT Zollinger-Ellison Syndrome in Children: A 25-Year FollowUp. S.D. Wilson. Surgery 110:696-703, (October), 1991. This is a report of the follow-up of five young patients who underwent total gastrectomy for Zollinger-Ellison syndrome (ZES). These patients were first examined in a report to the central surgical section 26 years prior to this study. In that study, three children who had less than total gastrectomy died with complications of gastric acid hypersecretion. In this group of five patients, however, only one patient was dead from tumor 14 years after total gastrectomy. In the remaining four patients growth and activity were near normal, and all had proven metastatic islet cell carcinoma during their course with lymph nodes positive in three patients and liver metastases in two patients. Only one patient has a normal serum gastrin and no apparent tumor. The author concludes that gastrinoma appears to be less aggressive in children, and that total gastrectomy may be preferable to lifelong medical management with the gastric acid inhibitors in children and young adults.--Thomas F. Tracy, Jr Mid-Ileal Stricture and Spinal Injuries Sustained by a Seven Year Old Child Restrained by a Lap Seat Belt Involved in a Motor Vehicle Accident. V. Sripathi and PM. King. Aust N Z J Surg 61:640-641, (August), 19~1. 7-Year-old girl, who was a rear seat passenger in a motor vehicle accident, was thrown forward and sustained a lower abdominal crushing injury due to a lap seat. At initial admission a crushed fracture third lumbar vertebrae was diagnosed, but she was judged fit for discharge the following day. Two weeks later she developed a subacute bowel obstruction from a stricture in her midileum. This was excised at laparotomy, and histopathological examination showed disruption of the muscular areas with dense fibrosis. The authors attribute this to the compression from the lap seat belt causing an ischaemic stricture. This injury has not previously been reported. Patricia Davidson Changing Trends in Necrotizing Enterocolitis. 1 Grosfeld, H. Cheu, M. Schlatter, et al. Ann Surg 214:300-307, (September), 1991. Necrotizing enterocolitis (NEC) still affects 1% to 2% of all infants in neonatal intensive care units. This report details changing trends in the patient population presenting with NEC and emphasizes factors resulting in significantly improved survival. Three hundred two infants were treated for NEC from 1972 to

1990, 184 of which required operative intervention. Follow-up is divided into two groups, the early period (1972 to 1982, n = 176) and the recent period (1983 to i990; n = 126). There were no significant group differences in gender, number of patients referred from outside institutions (70% v 77%), number of patients admitted with NEC (66% v 68%), or the number developing NEC while hospitalized for other reasons (34% v 32%). The groups were also similar as to maternal age, complications of pregnancy, incidence of multiple births, resuscitation at birth, incidence of congenital heart disease, and the need forventilator support. Significant differences were seen in the recent group being of lower birth weight (1,505 v 1,645 g), of earlier gestional age (30.4 v 32.4 weeks), later symptom onset (15.7 v 10 days), and less hyaline membrane disease (70% v 36%). Ninety percent of patients had received enteral intake (6% breast milk) before developing symptoms in both groups. Seven percent developed NEC without enteral feeds. Most frequent clinical findings (in order of frequency) were abdominal distention, abdominal tenderness, acidosis, blood in stool, bilious gavage, abdominal wall erythema, and oliguria. Bilious gavage, abdominal tenderness and abdominal wall erythema were less common in the recent group. The incidence of blood in the stool was similar in both groups. Severe acidosis and oliguria were more common in the early group. The diagnosis of NEC was confirmed by recumbent abdominal radiographs; pneumatosis, pneumoperitoneum, and portal vein air were observed equally in both groups. Patients were operated on earlier in the recent group (2.6 v 3.8 days), but the number of patients operated on was similar in both groups. Recent group infants showed improved survival in patients weighing < 1,000 g and those between 24 and 27 weeks' gestation. Improvements in survival were seen in both the medically treated group (68% to 98%) and the surgical group (51% to 75%). The authors review the numerous proposed etiologies for NEC, the absolute and relative indications for surgical intervention, and the numerous technology-related events that may have added to the improved outlook for very small infants with NEC.--Edward G. Ford Microvillus Inclusion Disease. ZM. Rhoads, R.C. Vogler, S.~ Lacey, et al. Gastroenterology 100:811-817, (March), 1991. Microvillus inclusion disease (MID) is an inherited defect of the brush border membrane that results in severe congenital diarrhea. The pathognomonic features of MID are hypoplastic villus atrophy with electron microscopic findings of intracellular brush border inclusions and densely packed apical cytoplasmic vesicular bodies. The authors describe an infant with this syndrome whose basal stool output was greater than 125 mL/kg/d, which was not altered by treatment with clonidine or octreotide. A section of jejunum was mounted in Ussing chambers for determination of transepithelial sodium and chloride fluxes. With respect to both sodium and chloride, the excised jejunum was in a net secretory state. A glucose-electrolyte solution was administered intrajejunally in this infant and this did not significantly change stool output, suggesting that all of the solution was absorbed. The authors speculate that subtotal enterocolectomy could have decreased purging by 66% in this infant. The parents opted not to have the infant's bowel resected, and she eventually died 8 weeks after ostomy placement, from Klebsiella pneumoniae septicemia.--Richard R. Ricketts Intussusception in Newcastle in a 25 Year Period. V.T. Kany, I W . Bear, I.S. Reid, et al. Aust N Z J Surg 61:608-613, (August), 1991. This paper reviews intussusception over a 25-year period in the City of Newcastle, Australia (population 500,000). The study cov-