Reno-portal anastomosis as an approach to pediatric kidney transplantation in the setting of inferior vena cava thrombosis.

In pediatric renal transplantation in the setting of IVC thrombosis, the retrohepatic IVC or gonadal veins are often used for outflow. However, if use of systemic venous outflow is unsuccessful, options become limited. We report the use of the portal vein for venous outflow in kidney retransplantation in the setting of IVC thrombosis. The patient is a 19-month-old male who developed end-stage renal failure at seven months of age secondary to hypotension after spontaneous rupture of an accessory renal vein. The IVC was occluded during emergent laparotomy, and the patient developed extensive IVC thrombosis. The first two transplant attempts used the retrohepatic IVC for venous outflow. Despite good initial flow, in both instances the renal vein thrombosed on post-operative day 1. In an unsuccessful salvage attempt of the second transplant, a reno-portal anastomosis was performed. With few options for vascular access, a third transplant was attempted. The reno-portal stump from the second transplant was used for outflow. The patient recovered well from his third transplant (creatinine 0.6 mg/dL 35 months post-surgery), demonstrating that the portal vein can be used for outflow in cases of extensive IVC thrombosis.

Multivisceral transplantation using a 2.9 kg neonatal donor.

Prematurity and very low birthweight have often been considered relative contraindications to neonatal organ donation. Organ procurement from neonatal donors is further complicated by unclear guidelines regarding neonatal brain death. We report a successful case of multivisceral transplantation using a graft from a 10-day-old, 2.9 kg, neonatal donor born at 36 6/7 wk in a 3.2 kg, three month old with intestinal and liver failure secondary to midgut volvulus. There was immediate liver graft function with correction of recipient coagulopathy, but delayed normalization of laboratory values and delayed return of bowel function. At six-yr post-transplant follow-up, the patient has normal intestine and liver function. Her last histologically confirmed rejection episode was 30 months prior to last follow-up. This case suggests that multivisceral grafts from very young or small neonatal donors may be transplanted successfully in selected cases. We propose a re-examination of the brain death guidelines for premature and young infants to potentially increase the availability of organs for infant recipients.

Stretched and sheared microcatheter retained after onyx embolization of infantile myofibromatosis.

We describe a rare neurointerventional complication, namely a stretched and sheared microcatheter, extending 52 cm from its point of retention within an Onyx cast in an infant patient's neck mass, to the groin. The tumor was an unusual manifestation of infantile myofibromatosis and prior attempts at resection had proven impossible due to bleeding. Recommendations regarding microcatheter selection, diagnostic workup, and management of the ensuing complication are given.

Life threatening tension pneumoperitoneum from intestinal perforation during air reduction of intussusception.

We present a case report of a child with intussusception who underwent air reduction which was complicated by bowel perforation. Life threatening tension pneumoperitoneum developed rapidly and immediate needle decompression was life saving in this case. The pathophysiology of hyperacute abdominal compartment syndrome is discussed.

Staged reconstruction for middle aortic syndrome.

BACKGROUND/PURPOSE: Middle aortic syndrome is a rare condition that involves narrowing of the abdominal aorta and its visceral branches. The authors propose staged vascular repair to minimize renal ischemia and facilitate use of native arterial tissue for reconstruction. METHODS: Three adolescents (age 8(1/2), 12(1/2), 13(1/2)) presented with severe hypertension. Subsequent evaluation showed coarctation of the abdominal aorta extending above the celiac axis. All 3 patients had bilateral renal artery stenoses. There also were tight stenoses of the celiac or superior mesenteric arteries. In the first stage the right renal artery stenosis was relieved. In the youngest patient, this was accomplished by balloon angioplasty. However, in the other 2, right renal autotransplantation was performed to the right iliac vessels using end-to-side anastomoses of the renal artery and vein. Cold perfusion was used. The second stage was performed 2 to 5 months later via a thoracoabdominal approach in 2 patients. A Dacron tube graft was utilized from above the coarctation to the iliac bifurcation. The left renal arteries were detached and anastomosed end to side to the bypass graft. In 1 child there were actually 3 separate renal arteries that required reimplantation. In the youngest patient the aortic narrowing was relieved by a long Dacron patch aortoplasty and interposition of an internal iliac artery graft to the left renal artery. RESULTS: All 3 patients recovered well and returned to full activities. There was no measurable rise of BUN or serum creatinine postoperatively. Postoperative renal scans showed good renal perfusion bilaterally. Follow-up results 2 to 10 years later continue to show well functioning reconstructions. CONCLUSION: A staged approach is an effective reconstruction for children with middle aortic syndrome which minimizes risk to renal function.

Nutritional and other postoperative management of neonates with short bowel syndrome correlates with clinical outcomes.

OBJECTIVE: To determine correlates of clinical outcomes in patients with short bowel syndrome (SBS). METHODS: Retrospective medical record review of neonates treated between 1986 and 1998 who met our criteria for SBS: dependence on parenteral nutrition (PN) for at least 90 days after surgical therapy for congenital or acquired intestinal diseases. RESULTS: Thirty subjects with complete data were identified; 13 (43%) had necrotizing enterocolitis, and 17 (57%)had intestinal malformations. Mean (SD) residual small bowel length was 83 (67) cm. Enteral feeding with breastmilk (r = -0.821) or an amino acid-based formula (r = -0.793) was associated with a shorter duration of PN, as were longer residual small bowel length (r = -0.475) and percentage of calories received enterally at 6 weeks after surgery(r = -0.527). Shorter time without diverting ileostomy or colostomy (r = 0.400), enteral feeding with a protein hydrolysate formula (r = -0.476), and percentage of calories received enterally at 6 weeks after surgery (r = -0.504) were associated with a lower peak direct bilirubin concentration. Presence of an intact ileocecal valve and frequency of catheter-related infections were not significantly correlated with duration of PN. In multivariate analysis, only residual small bowel length was a significant independent predictor of duration of PN, and only less time with a diverting ostomy was an independent predictor of peak direct bilirubin concentration. CONCLUSIONS: Although residual small bowel length remains an important predictor of duration of PN use in infants with SBS, other factors, such as use of breast milk or amino acid-based formula, may also play a role in intestinal adaptation. In addition, prompt restoration of intestinal continuity is associated with lowered risk of cholestatic liver disease. Early enteral feeding after surgery is associated both with reduced duration of PN and less cholestasis.

Use of a gracilis muscle flap to facilitate delayed ileal pouch-anal anastomosis.

Failure of an ileal pouch-anal anastomosis may result in unsuccessful completion of the anastomosis or removal of an ischemic pouch. We report a technique for preservation of the muscular wall of the rectum after mucosal dissection, which allowed a successful delayed pull-through.

Pediatric lung transplantation and "lessons from Green Surgery.".

During the past decade, lung transplantation has emerged as the definitive treatment for children with end-stage lung disease. Pediatric transplantation presents unique challenges with respect to diagnostic indications, donor-recipient size disparities, perioperative management, and growth. Lessons from the early development of cardiac surgery at the University of Minnesota (Green Surgical Service) provide a useful model for novel surgical challenges. Since 1990, 25 lung transplantations have been performed at our institution, including 4 heart-lung, 3 single-lung, 17 bilateral-lung, and 1 living-related lobar allograft. Age at transplantation ranged from 7 months to 27 years. The most common indication was cystic fibrosis. Given the limited donor pool, size disparities between donor and recipient were frequent. Excessive donor size was addressed by parenchymal reduction. Accommodation of small donor allografts was facilitated by elective cardiopulmonary bypass and pulmonary vasodilation using inhaled nitric oxide. Epidural anesthesia was routinely used for postoperative pain management and to enhance good pulmonary hygiene. Immunosuppression is presently achieved using cyclosporine, mycophenolate mofetil, and corticosteroids. Monitoring for rejection is accomplished with spirometry and transbronchial biopsies. Bronchial complications in 2 patients required placement of Palmaz stents. The living-related allograft was performed in a previous bone marrow transplant recipient obviating the need for long-term immunosuppression. The potential for growth of mature lung parenchyma postoperatively was studied and verified in a sheep model. Our experience parallels that of other frontiers such as early cardiac surgery in which medical and technologic innovations can be applied in a supportive environment to permit surgical progress.

Quality-of-life assessment after ileoanal pull-through for ulcerative colitis and familial adenomatous polyposis.

BACKGROUND/PURPOSE: The ileoanal pull-through procedure (IAP) is gaining increasing favor and use in the surgical treatment of children with ulcerative colitis (UC) and familial adenomatous polyposis (FP). Although physiological studies have been performed to assess the outcome of these children, no long-term quality-of-life assessment after the procedure has been performed. METHODS: Forty-three patients were identified who had an IAP at our institution in the last 10 years and were at least 6 months postsurgery. Thirty-four were contacted, and 32 agreed to participate in the survey, which was approved by the Human Studies Committee. Participants completed the standardized Medical Outcome Study Short Form-36 (SF-36), which has well-established normative values. Several supplemental questions were prepared in a similar format dealing with issues specific to the ileoanal pull-through procedure. RESULTS: Of the 32 participants, 19 (59%) were girls and 26 (81%) had ulcerative colitis. Mean age at the time of survey was 18.1 years with 12 less than 18 years and 20 > or =18 years. Data from the latter group could be compared with national normative values for this age. The study group was not statistically different from age-appropriate US population normal values on all assessable scales of physical and mental health in the SF-36 survey including physical functioning, role limitations-physical, bodily pain, general health, vitality, social functioning, role limitations-emotional, and mental health (all P>.05 or mean difference SD units <0.8). The supplemental questionaire demonstrated little adverse effect of the surgery. There was limited consumption of medications to control bowel frequency and little restriction of activity because of the frequency of bowel movements or fear of incontinence. The surgical scar was the sole negative factor of significance. CONCLUSIONS: The ileoanal pull-through procedure is an excellent surgical option for children with ulcerative colitis or familial adenomatous polyposis, and it produced minimal, if any, adverse effects on their long-term quality of life.

Lung growth after reduced size transplantation in a sheep model.

BACKGROUND: The growth of mature allografts is a critical issue in pediatric lung transplantation. This study explores the architectural changes of mature sheep lung when submitted to two different compensatory growth forces: either transplantation into a neonatal host or expansion in an otherwise empty adult hemithorax. METHODS: Right upper lobes (RUL) (mean+/-SEM, 66.7+/-1.9 kg) from 4- to 5-year old (adult sheep) were transplanted into newborn (n=6) lambs (5.4+/-0.3 kg, 5+/-2 days old) that were then allowed to survive for 45 days. Changes in pulmonary volume and architecture were measured before and after transplantation. Allografts were compared with both normal adult RUL (n=10) and adult (65.8+/-2.2 kg and 4 to 5 year old) RUL that remained in situ for 45 days after resection of the corresponding middle and lower lobes (n=6). Statistical differences were analyzed using two-sample and paired t tests. RESULTS: In adult animals, RUL remaining in the otherwise empty hemithorax compensated by an 85% increase in volume (251.5+/-18.7 ml vs. 466+/-32.8 ml) (P<0.0001). Concomitant increases in total internal alveolar surface area (48%) and alveolar size were prominent. The number of alveoli per volume decreased proportionately to the increases in volume (P<0.0001). There was no significant change in the calculated number of alveoli (345.6+/-40.5 x 10(6)) compared with the normal adult RUL (402.4+/-40.7x10(6)) (P=0.37). Transplant recipients received a reduced-size normal adult RUL (49%) in volume (125.3+/-21.5 ml). Allografts 45 days after transplantation showed a 73% increase in volume (216.4+/-21.3 ml) (P<0.0001) with a parallel (83%) increase in total internal alveolar surface area (P=0.008). The number of alveoli per volume remained constant (P=0.21) despite the increase in volume. There was therefore a significant increase in the calculated number of alveoli from before transplantation (172.5+/-35.9x 106) compared with that observed 45 days after transplantation (389.7+/-77.7x10(6)) (P=0.012). CONCLUSIONS: We conclude that mature sheep RUL parenchyma compensates with dilation of the respiratory structures in the adult animal, whereas there is alveolar multiplication when transplanted into newborn recipients.

Enteric duplications of the pancreatic head: definitive management by local resection.

BACKGROUND/PURPOSE: Enteric duplications can occur throughout the entire alimentary tract. When they occur in the pancreatic head, they present a formidable challenge in both diagnosis and treatment. Surgical management has ranged from simple drainage to local excision or radical resection (eg, Whipple procedure). The authors propose that with identification of the local anatomic relationships, definitive management can be achieved by complete local resection of the cyst mucosa. METHODS: The authors have treated four patients, ages 13 months to 4 years for enteric duplication cysts within the pancreatic head. RESULTS: Clinical presentations were quite varied, including pancreatitis (n = 2), gastritis (secondary to increased production of gastrin), and recurrent pleural effusion with high amylase content. Two of the four cysts had been drained initially using Roux-en-Y cystenterostomies. Our preoperative imaging studies included abdominal ultrasonography, endoscopic retrograde cholangiopancreatography (ERCP), angiography, computerized tomography (CT) or magnetic resonance imaging (MRI) scanning. When necessary intraoperatively, ultrasonography was used for cyst localization and transduodenal pancreatography to define precise ductal relationships. All four duplication cysts were completely excised, including two that communicated directly with the main pancreatic duct. One of the latter extended into the pleural cavity and required a thoracotomy for complete excision. Pathological exam of the excised cysts demonstrated gastric, duodenal, or respiratory mucosa. All four patients have remained entirely asymptomatic during a follow-up of 2 to 7 years postoperatively. CONCLUSION: The authors conclude that complete local resection of enteric duplication cysts in the pancreatic head can be performed for definitive management, avoiding the complications of more radical procedures.

The use of expandable metallic stents for acute tracheal stenosis in the growing lamb.

PURPOSE: Expandable metallic stents (Palmaz stents) have been used in the treatment of tracheobronchial obstruction in children and adults. The authors investigated their utility in the management of acute airway stenosis in a growing animal model. METHODS: A model for tracheal stenosis was developed in young lambs (mean age, 4 weeks; mean weight, 8.6 kg). Via an anterior tracheotomy, a circumferential mucosal injury to the trachea was produced with electrocautery in 31 lambs. In the control group (n = 10) no further intervention was used. In the treatment groups, either serial balloon dilatation of the stricture was performed (n = 6), or expandable metallic stents were inserted across the stricture (n = 15). All animals were monitored daily for signs of respiratory distress. Body weights, fluoroscopic airway measurements and rigid bronchoscopy were performed at least weekly. RESULTS: The average weekly rate of airway growth was 8.2% +/- 5.5% of the tracheal cross-sectional area (CSA). All animals displayed severe stenosis (mean, 90.2% +/- 7.5% of CSA) within 13.1 +/- 4 days after the injury. All animals in the control group had severe respiratory distress, weight loss and died within 14.6 +/- 2.8 days after injury. Serial balloon dilatation of the stricture alone failed to relieve symptoms in all six animals in this group, who died within 20 +/- 1 days after the injury, despite two to three dilatations each. With placement of expandable metallic stents, only 3 of 15 lambs died (two of pneumonia, one of iatrogenic perforation). The remaining 12 remained symptom-free and gained weight during a 2-month follow-up period. However, fluoroscopic examination showed partial collapse of the stents in all of these animals (mean, 44.7% +/- 21.6% of CSA) requiring an average of 2 +/- 0.7 bronchoscopic dilatations. Pathological evaluation showed more pronounced granulation tissue in the stented animals. CONCLUSIONS: The authors conclude that expandable metallic stents provide an effective tool in the management of acute tracheal stenosis. However, airway growth, tissue reaction, and the mechanical properties of the stent require close monitoring and stent adjustment.

Pulmonary blood flow distribution during partial liquid ventilation.

Regional pulmonary blood flow was investigated with radiolabeled microspheres in four supine lambs during the transition from conventional mechanical ventilation (CMV) to partial liquid ventilation (PLV) and with incremental dosing of perfluorocarbon liquid to a cumulative dose of 30 ml/kg. Four lambs supported with CMV served as controls. Formalin-fixed, air-dried lungs were sectioned according to a grid; activity was quantitated with a multichannel scintillation counter, corrected for weight, and normalized to mean flow. During CMV, flow in apical and hilar regions favored dependent lung (P < 0.001), with no gradient across transverse planes from apex to diaphragm. During PLV the gradient within transverse planes found during CMV reversed, most notably in the hilar region, favoring nondependent lung (P = 0.03). Also during PLV, flow was profoundly reduced near the diaphragm (P < 0.001), and across transverse planes from apex to diaphragm a dose-augmented flow gradient developed favoring apical lung (P < 0.01). We conclude that regional flow patterns during PLV partially reverse those noted during CMV and vary dramatically within the lung from apex to diaphragm.

Intra-abdominal sacrococcygeal mature teratoma or fetus in fetu in a third-trimester fetus.

This case report describes a fetus with a large intra-abdominal, complex, vascular mass, displacing the normal intra-abdominal organs due to its large size. Extension to the presacral area was also identified, facilitating the diagnosis of a sacrococcygeal teratoma. Because of its highly mature nature, this was considered a fetus in fetu which was totally intra-abdominal.

Esophageal replacement in children who have caustic pharyngoesophageal strictures.

Caustic injury to the upper aerodigestive system with scarring of the pharynx, hypopharynx, and esophagus is a challenging reconstructive problem. The authors report on seven patients who required total esophageal replacement from the pharynx to the stomach. Injury occurred from alkali in six and acid in one. Age at injury ranged from 14 months to 14 years (mean, 4.5 years.) in five boys and two girls. Time from injury to esophageal replacement was 6 months to 10 years (mean, 3.5 years). Two required pharyngeal reconstruction before and one after esophageal replacement. Six patients had an isoperistaltic right or transverse colon interposition. One who had gastric necrosis had an ileo-right colonic substernal interposition with creation of a jejunal reservoir. Results of barium swallows showed intact anastomoses in all patients. There were no leaks. Most had some degree of mild to moderate aspiration, and one who had left vocal cord paralysis had initially massive aspiration. Three patients currently eat regular diets; four eat but still require supplemental tube feeds. The authors conclude that children who have hypopharyngeal scarring and obliterated esophageal inlet can undergo a successful colonic esophageal replacement with high proximal pharyngocolic anastomosis.

Congenital diaphragmatic hernia--a tale of two cities: the Boston experience.

Infants with congenital diaphragmatic hernia (CDH) show a wide range of anatomic and physiological abnormalities, making it difficult to compare the efficacy of management protocols between institutions. The purpose of this study was twofold: (1) to analyze the results of treatment of CDH in a large tertiary care pediatric center using conventional mechanical ventilation (CMV) with extracorporeal membrane oxygenation (ECMO) as rescue therapy, and (2) to compare these results with those of a parallel study by a similar large urban center that used high-frequency oscillating ventilation (HFOV) as rescue therapy without ECMO. All patients who had CDH diagnosed within the first 12 hours of life and were referred for treatment before repair (between 1981 and 1994) were included in the analysis (n = 196). CMV was used initially in all patients, with conversion to ECMO for refractory hypoxemia or hypercapnea. Between 1981 and 1984, ECMO was not available. Between 1984 and 1987, ECMO was offered postoperatively. Between 1987 and 1991, ECMO was offered preoperatively. In all three groups, aggressive hyperventilation and alkalosis was the norm. Since 1991, permissive hypercapnia has been used. HFOV was used in three patients as stand-alone therapy with one survivor. Twenty patients died without repair: Ten had other lethal anomalies, eight died before ECMO could be instituted, and two died of ECMO-related complications. Overall, 104 patients (53%) survived and 92 (47%) died. Ninety-eight patients (50%) received ECMO, and 43 (44%) survived. Survivors had significantly higher 1- and 5-minute Apgar scores and higher postductal Po2s than did nonsurvivors. Associated anomalies were present in 39%, who had a significantly lower survival than those with isolated CDH. Antenatal diagnosis and side of the defect had no impact on outcome. Survival was not improved with the institution of ECMO or delayed repair but rose significantly to 69% (84% with isolated CDH, P = .007) with the introduction of permissive hypercapnea. Autopsy results from nonsurvivors showed other lethal anomalies and significant barotrauma as the primary causes of death. Comparisons between the Boston and Toronto series showed similar patient demographics and no significant differences in survival in any time period. The two series differed in the number of associated anomalies, their impact on survival, and in the prognosis of right-sided CDH. From the individual and combined analyses the authors concluded: (1) CMV with ECMO as rescue produced an overall survival in CDH patients equivalent to CMV with HFOV in a parallel series, (2) neither HFOV nor ECMO has significantly improved outcome in CDH patients, (3) institution of permissive hypercapnia has resulted in a significant increase in survival, and (4) the leading causes of death in CDH patients appear to be associated anomalies and pulmonary hypoplasia, which are currently untreatable. Barotrauma, which may contribute in up to 25% of deaths in CDH patients is avoidable.