Surgical site infections in neonates are independently associated with longer hospitalizations.

OBJECTIVE: There is limited data characterizing the risk and impact of surgical site infection (SSI) in neonates; this makes it difficult to identify factors that increase neonatal SSI risk and to determine how SSI affects outcomes in this special population. STUDY DESIGN: The American College of Surgeons National Surgical Improvement Program Pediatric (NSQIP-P) collected data on children undergoing surgery at children's hospitals from 2012 to 2014. Neonates undergoing general surgical procedures were characterized with regard to demographic characteristics and comorbidities. Perioperative variables such as wound class, type of surgery and length of operation were also evaluated. RESULTS: Seven thousand three hundred and seventy-nine neonates were identified in the NSQIP-P participant user file. The overall SSI rate was 2.6%. Only wound class and length of surgery were significantly associated with SSI. Furthermore, SSI was independently associated with longer length of stay, even after adjusting for covariates. CONCLUSIONS: This is the largest study to date analyzing SSI in neonates. We found that perioperative variables have a more significant impact on SSI than patient factors, suggesting that operation-related characteristics are influencing SSI. Furthermore, neonates with SSI are more likely to have prolonged hospitalizations even after adjusting for patient comorbidities.

Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup Study.

PURPOSE: To determine whether the 3-year event-free survival (EFS) of children with completely resected immature teratomas is greater than 85%. PATIENTS AND METHODS: Patients with immature teratomas treated at Pediatric Oncology Group or Children's Cancer Group institutions were eligible. Pathology was centrally reviewed to confirm diagnosis and tumor grading. Follow-up included physical examination, measurement of tumor markers (alpha fetoprotein and human chorionic gonadotropin), and imaging. All patients were monitored for events, defined as tumor recurrence, second malignancy, or death. RESULTS: Seventy-three children (median age, 7.8 years) with extracranial immature teratomas were enrolled on study. Primary tumor sites included ovarian (n = 44), testicular (n = 7), and extragonadal (n = 22). However, on review, 23 patients had foci of yolk sac tumor (n = 21) or primitive neuroectodermal tumor (n = 2), whereas 50 had pure immature teratomas. Twenty-five patients had increased alpha fetoprotein (n = 18), human chorionic gonadotropin (n = 5), or both (n = 2); nine had foci of yolk sac tumor on review. Pathology review identified 23 patients with grade 1, 29 with grade 2, and 21 with grade 3 immature teratomas. With a median follow-up of 35 months, the overall 3-year EFS was 93% (95% confidence interval, 86% to 98%), with 3-year EFS of 97.8%, 100%, and 80% for patients with ovarian, testicular, and extragonadal tumors, respectively. Only four of 23 patients with immature teratoma and malignant foci developed recurrence, suggesting that surgical resection followed by close observation are effective treatment. Overall, five patients had disease recurrence 4 to 7 months from diagnosis, and four (80%) are disease free after platinum-based therapy. The fifth patient has residual tumor after cisplatin, etoposide, and bleomycin treatment requiring further therapy. CONCLUSION: Surgical excision is safe and effective treatment for 80% to 100% of children with immature teratoma.

Immature teratomas in children: pathologic considerations: a report from the combined Pediatric Oncology Group/Children's Cancer Group.

Pediatric germ cell tumors (n = 135) with a major component of immature teratoma (IT) registered on Pediatric Oncology Group/Children's Cancer Group treatment protocols from 1990 to 1995 were reviewed. Sixty cases were pure IT with no malignant component and 75 were mixed tumors with a major component of IT. Foci of yolk sac tumor (YST) were present in all 75 mixed tumors; additional malignant components were present in 15. The IT component was as follows: 47% grade 3, 29% grade 2, 24% grade 1. There were no significant correlations between tumor grade and patient age by specific subsets or overall (all p > 0.10). Significant correlations were detected between stage and the presence of foci of YST (p = 0.0145) and grade and the presence of foci of YST (p < 0.001). Serum alpha-fetoprotein concentrations were elevated at diagnosis in 96% of ovarian tumors with foci of YST and were mildly elevated (< 60 ng/dL) in only 16% of tumors without YST. Overall 2- to 6-year survival rate was 96% and was related to the presence of YST. Central pathologic review revealed aspects of morphologic diagnosis that were most frequently misinterpreted by contributing pathologists. These included the classification of differentiating tissues as immature and the failure to recognize two well-differentiated patterns of YST (the hepatoid pattern resembling fetal liver and the well-differentiated glandular pattern resembling fetal lung or intestine). Such foci were often overlooked. The authors conclude that the presence of microscopic foci of YST, rather than the grade of IT, per se, is the only valid predictor of recurrence in pediatric IT at any site.

Monotherapy with cyclosporine for chronic immunosuppression in pediatric liver transplant recipients.

Children who survive liver transplantation (LT) suffer the adverse effects of life-long immunosuppression. In an attempt to minimize these effects, we have instituted a program of tapering immunosuppression, resulting in chronic monotherapy for children after LT. Seventy-three children ages 4 months to 19 years received LT between January 1987 and December 1992. Patient survival was 85% (62/73), with graft survival of 73%, at one year. Triple therapy with prednisone, cyclosporine, and azathioprine begun at transplant was tapered as follows: 1-2 mg/kg prednisone at discharge was reduced by .2 mg/kg every 2 months until a .2 mg/kg total was reached. Alternate-day steroids (.2 mg/kg) were begun at 1 year and discontinued at 1.5 years. AZA (1 mg/kg) was begun posttransplant and discontinued after any serious viral illness or by 1 year. Currently 37 survivors are > 18 months post-LT and were considered candidates for monotherapy. Monotherapy was attempted in 28 (76%), and 25 of these remain on monotherapy an average of 2 years later. All have normal liver function. After monotherapy and alternate-day steroids were achieved, 66% of children < 5th percentile for height at the time of transplant improved to greater than the 5th percentile. There were 3 (11%) patients who rejected while on monotherapy an average of 1.15 years after it was started. These patients had the following predisposing factors that decreased cyclosporine levels and led to rejection: common bile duct stricture, chronic and intermittent antibiotic administration for urinary tract infection, and noncompliance. In the 9 potential candidates not tapered to monotherapy, 6 have had recurrent acute or chronic rejection; 2 of these now receive FK506. We conclude that the majority of stable pediatric LT recipients may be safely tapered to chronic cyclosporine monotherapy. Increased growth is a major benefit of decreased steroid dosing in these children. Cyclosporine absorption and adequate levels are crucial for success of this approach.

Congestive heart failure and absent femoral pulses in newborns without coarctation of the aorta.

Two infants with thrombosis of the abdominal aorta are discussed. In each case the presentation was indistinguishable from that in coarctation of the aorta, with heart failure and absent femoral pulses. Surgery in one infant successfully relieved the obstruction. The diagnosis may not be suspected from the history. Aggressive management is indicated.

Surgical management of aplasia cutis congenita.

A child born without scalp, or dura to cover the brain (aplasia cutis congenita) was successfully treated by a multidiscipline team. A coexisting rupture omphalocele forced a change in treatment from the currently recommended regimen of mandatory early scalp closure. Homograft skin was used to protect the brain during the time the omphalocele was treated and skin flaps were delayed. Fluoroscein dye was utilized to determine flap viability and predicted ischemia until after a third delaying procedure was performed. The successful outcome suggests that the present philosophy of early surgical closure being essential for survival in infants with large cranial defects can be altered and, in fact, permanent full-thickness flaps may be designed, tested for viability, and delayed while homograft skin protects the infant's brain from infection and thrombosis.

Renal pelvic tumors in childhood.

A capillary hemangioma in the renal pelvis of a twelve-year-old child is presented. The diagnosis and management of renal pelvic tumors in childhood is reviewed. We believe this pathologic entity should be included in the differential diagnosis of hematuria and ureteropelvic junction obstruction in a child.

Gastroesophageal reflux in the infant with cystic fibrosis.

The association of gastroesophageal reflux and its sequelae in the infant with cystic fibrosis has gone virtually unnoticed. Eight of 40 newly diagnosed infants with cystic fibrosis seen over a 24 month period had significant gastroesophageal reflux, characterized by vomiting (7 infants), recurrent pneumonia (7 infants), and failure to thrive (4 infants). Gastroesophageal reflux was demonstrated by a combination of barium swallow, scintiscan, manometry, and esophagoscopy. Three infants had rapid and permanent alleviation of symptoms after standard medical therapy; in five infants, therapy failed and they required a Nissen fundoplication. Three infants required postoperative ventilatory support for 1, 2, and 5 days. No tracheostomies were required. Postoperative hospital stay averaged 12 days (range 5 to 30 days). There were no complications or perioperative deaths. All children had complete relief of their preoperative symptoms. The group that required surgery presented earlier (mean 7 weeks of age) to the cystic fibrosis center than either the medically treated group (mean 5 months of age) or the group free of gastroesophageal reflux symptoms (5 1/2 months of age). Also, postoperative hospitalization time markedly decreased from 50 percent of 577 combined patient days preoperatively to 4 percent of 1,639 days postoperatively; this 4 percent then paralleled what was seen in the group without gastroesophageal reflux (3 percent of 19,966 combined patient days) and the group controlled medically (5 percent of 1,897 days). The pulmonary manifestations of cystic fibrosis are extremely variable, and evaluation of the effect that any intervention has on the natural history of the disease is difficult. Nonetheless, we believe that even this small series suggests that gastroesophageal reflux and its complications can significantly alter the courses of some children with cystic fibrosis. Gastroesophageal reflux should be managed as aggressively as it is in any child with reflux, and a successful and safe reduction of symptoms can be expected with intensive management.

The suture fistula technique in the repair of selected cases of esophageal atresia.

Correction of esophageal atresia with tracheoesophageal fistula can present a difficult problem when distance and tension between upper and lower esophageal segments are too great for primary anastomosis. Alternatives have included staged procedures with upper pouch elongation or interpositions. In 1974, Shafer and David described an operation in which the lower segment was divided, closed, and then merely apposed to the unopened upper pouch. A central "necrosing suture" was placed through the lumina of both segments. Spontaneous fistulization between the two segments occurred postoperatively, and the resultant channel was then progressively dilated with woven silk bougies. This technique has been employed at Babies Hospital in five children. The results were excellent in four. One child failed to fistulize, and following a complicated postoperative course, died at home from accidental dislodgement of his tracheostomy tube. All five children required antireflux procedures but ultimately did well with regard to esophageal function.

Computed tomographic scanning in children.

Computed tomographic scanning (CT) has been effectively utilized in evaluating the pediatric patient. Our experience with CT of the chest and abdomen in 19 patients has been described. The advantages offered by CT include: a unique anatomic display, the lack of morbidity, and an acceptable radiation dosage. Few technical disadvantages exist. Further application of this unique technique can be predicted.

Is age less than 1 year a high-risk category for orthotopic liver transplantation?

The aim of this study was to determine if age less than 1 year is a high risk group for orthotopic liver transplantation (OLT). Retrospective analysis was done of patients with liver failure who received OLT. Comparison was made between patients aged < 1 year and > 1 year with regard to survival, allograft survival, hepatic artery thrombosis, and medical status at OLT. Between January 1, 1987 and September 30, 1991, 46 children received OLT. Fifteen (35%) were < 1 year (average age, 7.93 months). Survival in children < 1 year was 80% and children > 1 year was 91%. Allograft survival in children < 1 year was 57% (21 allografts required for 12 survivors) and 78% in children > 1 year (37 allografts required for 29 survivors). Retransplantation was required in 5 of 15 children < 1 year (33%) and in 5 of 29 children (17%) > 1 year. Medical status in children < 1 year was similar to medical status in children > 1 year at the time of transplant. Children with chronic stable liver disease represented 60% of children < 1 year and 60.1% of children > 1 year. Children requiring hospitalization represented 26% of children < 1 year and 29% of children > 1 year. Children in intensive care represented 13% of children < 1 year and 11% of children > 1 year. Survival for all status groups was similar. Hepatic artery thrombosis occurred in one child < 1 year and in 2 children > 1 year. No statistical difference (chi 2 analysis) was found by age between the categories evaluated.(ABSTRACT TRUNCATED AT 250 WORDS)

Successful splenorenal shunt for emergent control of variceal bleeding in a small child.

A 8.6-kg, 10-month-old boy with idiopathic cavernous transformation of the portal vein developed variceal hemorrhage refractory to nonoperative management. A distal splenorenal shunt was performed while the bleeding was controlled with balloon tamponade. Hemorrhage was successfully controlled with no recurrent bleeding and the shunt remains patent at 1 year of follow-up.

Familial retroperitoneal fibrosis.

Retroperitoneal fibrosis is an unusual cause of obstructive uropathy in the pediatric population. The etiology is unknown although there are laboratory and clinical associations with various autoimmune diseases. Familial associations have not been reported in children. A family is reported where two siblings have idiopathic retroperitoneal fibrosis. In addition, these sisters and the father manifest clinical laboratory evidence for systemic immunologic diseases. These young girls represent the first patients to illustrate both autoimmune and familial characteristics of this disease. These cases support the concept that retroperitoneal fibrosis is a local manifestation of a systemic immune disease.

Pyelonephritis following pediatric renal transplant: increased incidence with vesicoureteral reflux.

The association between pyelonephritis and vesicoureteral reflux (VUR) following pediatric renal transplantation is unclear. To understand the relationship of vesicoureteral reflux with urinary tract infection (UTI) and pyelonephritis, 67 patients were evaluated for reflux and pyelonephritis. Sixty-seven pediatric patients, aged 2 to 18 (39 males and 28 females) underwent renal transplantation. Beginning in 1982, all patients underwent voiding cystourethrography or radionuclide voiding studies 1 to 3 months postoperatively to assess the incidence of VUR. Techniques of ureteroneocystostomy (UNC) included the Leadbetter-Politano (L-p) in 39 cases, and two different modifications of the LICH (herein called LICH-1 and LICH-2) in 30 cases. Urinary cultures were performed routinely. Pyelonephritis was considered present in any patient with UTI and increased serum creatinine or fever greater than 38.5. VUR occurred in 36% of patients; highest in LICH-1 (79%), intermediate in L-P (22%), and lowest in LICH-2 (9%). VUR was not statistically significantly higher in females (43%) v males (31%). UTI occurred in 37% of patients. The difference in incidence between females (54%) and males (26%) was significant (P less than .05). The frequency of UTI in patients with VUR was 46% v 33% in patients without reflux (NS). However, pyelonephritis that occurred in 16% of cases overall was present in 82% of UTIs in patients with reflux v 14% of UTIs in patient without reflux (P less than .01). Pyelonephritis is significantly increased in pediatric renal transplant patients with UTI was have VUR. A nonrefluxing UNC is advocated in all patients. All renal transplant patients should have routine monitoring of urinary cultures and should be evaluated of VUR posttransplant.

Respiratory complications in cervical thymic cysts.

Thymic cysts are considered uncommon lesions in the differential diagnosis of pediatric neck masses. They have been described as asymptomatic and of little clinical consequence. Recent reports have stressed the possibility of respiratory compromise associated with these lesions. We reviewed our experience with cervical thymic cysts with emphasis on respiratory problems. Ten pediatric patients underwent surgery and were found to have cervical thymic cysts. Ages ranged from newborn to 14 years. There were four boys and six girls. Two were found to have the thymic cysts at time of neck exploration for Grave's disease and hyperparathyroidism. Of the remaining eight patients, all had mobile cystic masses, located anterior to but extending beneath the lower third of the sternocleidomastoid muscle. The size of the mass ranged from 3.0 to 8.5 cm. Preoperative diagnosis included cystic hygroma/branchial cleft cyst (five), lymphoma (one), teratoma (one), and thymic cyst (one). All had a history of rapidly developing neck mass. Seven of the eight gave a history of upper respiratory tract infection (URI) prior to the development of the mass. Five had imaging studies that showed tracheal compression. Three of these required airway management in the early postoperative period. All were excised through a neck incision, with two requiring sternal extension. Histology showed cholesterol crystals, Hassall's corpuscles, and giant cell reaction diagnosis of thymic cysts. There has been no recurrence and no permanent respiratory sequela in the ten patients. Cervical thymic cysts are benign lesions that may be more common than literature suggests.(ABSTRACT TRUNCATED AT 250 WORDS)

Transplantation for acute hepatic failure in children.

BACKGROUND/PURPOSE: Acute liver failure in the pediatric population is a rare but highly lethal health problem. Sometimes it is difficult to predict who will benefit from liver transplantation. The authors report on their experience in the past 8 years at a pediatric transplant center. METHODS: A retrospective chart review was performed on all children referred to the liver transplant (TX) service with the diagnosis of acute hepatocellular dysfunction (AHD) from 1988 to 1996. Presentation, chemistries, and clinical course were evaluated. Statistical analysis was performed using analysis of variance. RESULTS: Twenty-six children underwent evaluation. Seventeen patients fulfilled the criteria for fulminant hepatic failure (FHF). Eleven patients recovered without TX, 14 received a TX, and one died awaiting TX. Of those that received a TX, four died in the early postoperative period and 10 survived (mean follow-up of 4.2 years). There was a wide range in most laboratory values. Serum bilirubin levels, ammonia levels, and coagulation parameters, however, reached statistical significance in patients requiring transplant. The most consistent discriminators of need for transplantation and outcome were neurological findings and multisystem organ failure. Children who recovered without TX had no seizures and minimal encephalopathy. Of the 15 children who were recommended for TX, six had seizures and all had encephalopathy, 12 having grade III or IV. All five nonsurvivors had respiratory failure early in their clinical course, and four of five nonsurvivors also had renal failure. CONCLUSIONS: There is significant overlap in the presentation and laboratory findings of children who present with AHD or FHF. Neurological status was an important discriminator of need for transplantation. Patients who presented with multisystem organ failure, including renal failure and respiratory failure, had 100% mortality rate despite liver transplantation.

Characterization and treatment of biliary anastomotic stricture after segmental liver transplantation.

BACKGROUND/PURPOSE: Biliary anastomotic strictures (BAS) after left lateral segment liver transplantation (LLST) may cause graft dysfunction, sepsis, and patient mortality. A review of the authors' experience was performed to better characterize the risk factors and corrective management. METHODS: The medical records of 9 children who underwent a LLST in whom a BAS developed from 1989 to the present were reviewed retrospectively. RESULTS: Seventy-five of 199 liver transplants (38%) at the authors' institution since 1989 have been LLST. BAS developed in 12% of these cases. BAS were diagnosed less than 12 months after transplantation in 4 children (mean, 7.5 months; range, 5 to 11 months) and greater than 12 months in 5 children (mean, 37 months; range, 14 to 72 months). Early strictures (<12 months) were associated with hepatic artery thrombosis (n = 1), and posttransplant bile leak (n = 1) and ducts from segment II and III exiting separately from the left lateral segment (n = 2). The diagnosis of BAS was heralded by episodes of liver biopsy-proven cholangitis in all patients and confirmed radiographically. Seven children underwent successful biliary exploration and revision of the hepaticojejunostomy. Two of these children ultimately required retransplantation secondary to chronic graft rejection. CONCLUSIONS: BAS in LLST are a source of significant morbidity and should be considered in children after LLST who present with cholangitis. Surgical correction is possible in most cases.

Simple technique for determination of the correct length of percutaneous tunnelled catheters in neonates and children.

A simple technique for determining the correct catheter length in percutaneous tunnelled catheters in infants and young children has been devised that virtually guarantees accurate catheter tip placement. Sixty-six patients, aged newborn to 5 years (mean, 1.6 years) have successfully undergone this technique. It is safe, simple, precise, quick, and cost effective. It requires only a hemostat, a suture, and the supplies provided in the prepackaged catheter kit. This technique should be used whenever a percutaneous technique for accessing the vein is used and fluoroscopy is available.

Rejection after pediatric liver transplantation is not the limiting factor to survival.

Liver transplantation became a very successful therapy when cyclosporin A was introduced as an immunosuppressive agent. In an effort to evaluate the contribution of difficult or uncontrolled allograft rejection to mortality, the authors determined the most important factors that limited survival after liver transplantation. Eighty-two children received a total of 94 liver transplants from January 1987 to the present. Patients' records were reviewed for complications that contributed to morbidity and mortality and to assess which complications were preventable. Twelve patients died (15%), nine within 6 weeks of liver transplantation. The chief contributing cause of death was hepatic artery thrombosis (one patient), brain death after liver transplantation for fulminant hepatic failure (two patients), primary allograft nonfunction or dysfunction (two patients), allograft rejection (three patients), or other problems (four patients). Overall, hepatic artery thrombosis occurred in 3.1%, allograft rejection in 57%, fulminant hepatic failure in 7%, and donor organ dysfunction in 7%. Allograft rejection contributed directly to the cause of death in three children (4%). The authors conclude that few deaths after pediatric liver transplantation are caused by failure of immunosuppression. A high survival rate can be achieved after transplantation by eliminating the correctable complications that most frequently occur in the early postoperative period.

Isolated liver transplantation for liver failure in patients with short bowel syndrome.

The ultimate prognosis for patients with short bowel syndrome (SBS) has become progressively more favorable over the past decade. Advances in long-term total parenteral nutrition (TPN) have allowed this group of patients to meet nutritional needs while the process of intestinal adaptation occurs. Unfortunately, a subgroup of patients with SBS have hepatic failure (HF), most often secondary to TPN-induced cholestasis. Combined small bowel and liver transplantation (LT) offers a sound anatomic solution for cases of HF with SGS, but it remains experimental at this time. We propose that an isolated LT is a viable alternative mode of therapy for the patient with HF and SBS. The following characteristics were reviewed for five patients with SBS and HF who underwent LT: age at transplantation, weight, liver function, survival, intestinal length, volume of feeding before surgery, and current feeding tolerance and liver function. Four boys and one girl, aged 5.5 to 15 months (average, 11.9), had LT. The total bilirubin level at the time of transplantation was 14.4 to 37 mg/dL (average, 24.7). The patients weighed between 3.8 and 12 kg (average, 8.0), and feeding tolerance ranged from no enteric to complete enteric feeding (average, < 33% of calories by enteric feeding). Bowel loss was attributed to necrotizing enterocolitis in two cases, volvulus in two, and birth hypoxia in one. Bowel length ranged from 60 to 120 cm (average, 88.6). Four children (80%) survived LT, and the average follow-up period was 9.3 months. Three (75%) are home; one is on combined hyperalimentation and enteral feeding, and two are on full enteric feeding. One remains in a chronic care facility, on combined enteral and intravenous feeding. The average daily enteral feeding now comprises more than 70% of caloric requirements. The total bilirubin level is .6 to .8 mg/dL (average, .71). Isolated LT for HF in the patient with SBS effectively restores liver function, allowing time for further intestinal adaptation.