Surgical site infections in neonates and infants: is antibiotic prophylaxis needed for longer than 24 h?

PURPOSE: The purpose is to determine whether use of perioperative antibiotics for more than 24 h decreases the incidence of SSI in neonates and infants. METHODS: We studied neonates and infants who had clean-contaminated or contaminated gastrointestinal operations from 1996 to 2006. Patient- and operation-related variables, duration of perioperative antibiotics, and SSI within 30 days were ascertained by retrospective chart review. In assessing the effects of antibiotic duration, we controlled for confounding by indication using standard covariate adjustment and propensity score matching. RESULTS: Among 732 operations, the incidence of SSI was 13%. Using propensity score matching, the odds of SSI were similar (OR 1.1, 95% CI 0.6-1.9) in patients who received ≤24 h of postoperative antibiotics compared to >24 h. No difference was also found in standard covariate adjustment. This multivariate model identified three independent predictors of SSI: preoperative infection (OR 3.9, 95% CI 1.4-10.9) and re-operation through the same incision, both within 30 days (OR 3.5, 95% CI 1.7-7.4) and later (OR 2.3, 95% CI 1.4-3.8). CONCLUSION: In clean-contaminated and contaminated gastrointestinal operations, giving >24 h of postoperative antibiotics offered no protection against SSI. An adequately powered randomized clinical trial is needed to conclusively evaluate longer duration antibiotic prophylaxis.

Risk factors for parenteral nutrition­associated liver disease following surgical therapy for necrotizing enterocolitis: A Glaser Pediatric Research Network Study [corrected].

OBJECTIVE: The aim of the study was to prospectively determine risk factors for the development of parenteral nutrition-associated liver disease (PNALD) in infants who underwent surgery for necrotizing enterocolitis (NEC), the most common cause of intestinal failure in children. PATIENTS AND METHODS: : From February 2004 to February 2007, we diagnosed 464 infants with NEC, of whom 180 had surgery. One hundred twenty-seven patients were available for full analysis. PNALD was defined as serum direct bilirubin ≥ 2 mg/dL or ALT ≥ 2 × the upper limit of normal in the absence of sepsis after ≥ 14 days of exposure to PN. RESULTS: Median gestational age was 26 weeks and 68% were boys. Seventy percent of the cohort developed PNALD and the incidence of PNALD varied significantly across the 6 study sites, ranging from 56% to 85% (P = 0.05). Multivariable logistic regression analysis identified small-bowel resection or creation of jejunostomy (odds ratio [OR] 4.96, 95% confidence interval [CI] 1.97-12.51, P = 0.0007) and duration of PN in weeks (OR 2.37, 95% CI 1.56-3.60, P < 0.0001) as independent risk factors for PNALD. Preoperative exposure to PN was also associated with the development of PNALD; the risk of PNALD was 2.6 (95% CI 1.5-4.7; P = 0.001) times greater in patients with ≥ 4 weeks of preoperative PN compared with those with less preoperative PN use. Breast milk feedings, episodes of infection, and gestational age were not related to the development of PNALD. CONCLUSIONS: The incidence of PNALD is high in infants with NEC undergoing surgical treatment. Risk factors for PNALD are related to signs of NEC severity, including the need for small-bowel resection or proximal jejunostomy, as well as longer exposure to PN. Identification of these and other risk factors can help in the design of clinical trials for the prevention and treatment of PNALD and for clinical assessment of patients with NEC and prolonged PN dependence.

Congenital diaphragmatic hernia: endothelin-1, pulmonary hypertension, and disease severity.

RATIONALE: Endothelin-1 (ET1) is dysregulated in pulmonary hypertension (PH). It may be important in the pathobiology of congenital diaphragmatic hernia (CDH). OBJECTIVES: We hypothesized that ET1 levels in the first month would be higher in infants with CDH who subsequently expired or were discharged on oxygen (poor outcome). We further hypothesized that ET1 levels would be associated with concurrent severity of PH. METHODS: We sampled plasma at 24 to 48 hours, and 1, 2, and 4 weeks of age in 40 prospectively enrolled newborns with CDH. We performed echocardiograms to estimate pulmonary artery pressure at less than 48 hours of age and weekly to 4 weeks. PH was classified in relationship to systemic blood pressure (SBP): less than 2/3 SBP, 2/3 SBP-systemic is related to pressure, or systemic-to-suprasystemic pressure. MEASUREMENTS AND MAIN RESULTS: ET1 levels at 1 and 2 weeks were higher in infants with poor outcome compared with infants discharged on room air (median and interquartile range: 27.2 [22.6, 33.7] vs. 19.1 [16.1, 29.5] pg/ml, P = 0.03; and 24.9 [17.6, 39.5] vs. 17.4 [13.7, 21.8] pg/ml, P = 0.01 at 1 and 2 weeks, respectively). Severity of PH was significantly associated with increasing ET1 levels at 2 weeks (16.1 [13.7, 21.8], 21.0 [17.4, 31.1], and 23.6 [21.9, 39.5] pg/ml for increasing PH class, P = 0.03). Increasing severity of PH was also associated with poor outcome at that time (P = 0.001). CONCLUSIONS: Infants with CDH and poor outcome have higher plasma ET1 levels and severity of PH than infants discharged on room air. Severity of PH is associated with ET1 levels.

Risk factors for intestinal failure in infants with necrotizing enterocolitis: a Glaser Pediatric Research Network study.

OBJECTIVE: To determine risk factors for intestinal failure (IF) in infants undergoing surgery for necrotizing enterocolitis (NEC). STUDY DESIGN: Infants were enrolled in a multicenter prospective cohort study. IF was defined as the requirement for parenteral nutrition for >or= 90 days. Logistic regression was used to identify predictors of IF. RESULTS: Among 473 patients enrolled, 129 had surgery and had adequate follow-up data, and of these patients, 54 (42%) developed IF. Of the 265 patients who did not require surgery, 6 (2%) developed IF (OR 31.1, 95% CI, 12.9 - 75.1, P < .001). Multivariate analysis identified the following risk factors for IF: use of parenteral antibiotics on the day of NEC diagnosis (OR = 16.61, P = .022); birth weight < 750 grams, (OR = 9.09, P < .001); requirement for mechanical ventilation on the day of NEC diagnosis (OR = 6.16, P = .009); exposure to enteral feeding before NEC diagnosis (OR=4.05, P = .048); and percentage of small bowel resected (OR = 1.85 per 10 percentage point greater resection, P = .031). CONCLUSION: The incidence of IF among infants undergoing surgical treatment for NEC is high. Variables characteristic of severe NEC (low birth weight, antibiotic use, ventilator use, and greater extent of bowel resection) were associated with the development of IF.

Vascular endothelial growth factor isoform and receptor expression during compensatory lung growth.

BACKGROUND: Vascular endothelial growth factor (VEGF) is required for blood vessel formation during lung growth and repair. Alteration of VEGF isoform expression has been demonstrated in response to fetal tracheal occlusion and in models of lung injury. The purpose of this study was to investigate VEGF expression during compensatory lung growth in the mouse. METHODS: Under general anesthesia, adult mice underwent left thoracotomy with (n = 5) or without (sham, n = 5) pneumonectomy. The right lungs were harvested at 1, 3, and 7 d after the operation. Lung-to-body weight ratio as well as total DNA and protein content were measured. VEGF protein expression was analyzed by Western blot and ELISA. VEGF isoform expression was evaluated using semi-quantitative PCR followed by Imagequant optical densitometry. Values were compared by Student's t-test and ANOVA using Fisher's protected least significant difference post-hoc test where appropriate. RESULTS: Compensatory lung growth was observed as measured by increases in right lung-to-body weight ratio and in DNA and protein content. Total VEGF RNA and protein expression did not change after pneumonectomy. However, on post-operative day 1, there was a decrease in the relative percentage of VEGF188 mRNA (P < 0.01), and an increase in the relative percentage of VEGF164 mRNA (P = 0.05). At 3 d postpneumonectomy, low relative VEGF188 expression persisted (P < 0.05), VEGF164 expression normalized, and relative VEGF120 expression increased (P < 0.01). Isoform expression in the pneumonectomy animals was identical to sham animals by the seventh d. There were no differences observed in VEGF receptor expression. CONCLUSION: During compensatory lung growth, we have observed an early postoperative reversion of VEGF isoform expression to the pattern seen during fetal lung development and in lung injury models.

Increasing prevalence of gastroschisis: population-based study in California.

OBJECTIVE: To evaluate time trend of gastroschisis and examine the epidemiological risk factors for gastroschisis. STUDY DESIGN: This population-based study analyzed the active surveillance data from the California Birth Defects Monitoring Program from 1987 to 2003. RESULTS: The overall birth prevalence of gastroschisis was 2.6 cases per 10,000 births (908 cases in >3.5 million births). In the adjusted analysis, by using the age of 25 to 29 years as the reference, mothers aged 12 to 15 years had a 4.2-times greater birth prevalence (95% CI, 2.5-7.0), and fathers aged 16 to 19 years and 20 to 24 years had 1.6- and 1.5-times greater birth prevalence (95% CI, 1.1-2.1 and 1.2-1.8), respectively. Compared with non-Hispanic whites and US-born Hispanic, both foreign-born Hispanics and blacks had adjusted prevalence ratio of 0.6 (95% CI, 0.5-0.7 and 0.4-0.9, respectively). In addition, nulliparity was also associated with gastroschisis. Independent of maternal age, paternal age, and maternal ethnicity, the birth prevalence increased 3.2-fold (95% CI, 2.3-4.3) during the 17-year study period. CONCLUSIONS: The birth prevalence of gastroschisis continues to increase in California, and young, nulliparous women are at the greatest risk of having a child with gastroschisis.

Does congenital diaphragmatic hernia associated with bronchopulmonary sequestration portend a better prognosis?

INTRODUCTION: Congenital diaphragmatic hernia (CDH) continues to be a devastating disease in the newborn population, with well-documented morbidity and mortality. Bronchopulmonary sequestration is a separate congenital defect that has been associated with CDH. While the association of sequestration with CDH has been reported to be as high as 30-40%, the prognosis associated with the two simultaneous defects is unknown. We reviewed our experience to evaluate if prognosis was better in the CDH infants with associated bronchopulmonary sequestration. METHODS: Institutional approval was obtained. Our institutional database was examined from August 1995 to August 2005, identifying all mothers carrying fetuses with pulmonary masses and/or CDH and all neonates treated with bronchopulmonary sequestration and/or CDH. Patients who had both CDH and sequestration were identified by prenatal ultrasound reports, postnatal radiographs, and operative and pathology reports. RESULTS: 16 patients were identified in the fetal or neonatal period with concomitant diagnoses of CDH and bronchopulmonary sequestration. Of those proceeding to delivery, 6 expired and 6 survived. The presence of liver herniation and low lung-to-head ratio on antenatal ultrasound correlated with mortality. However, 2 patients survived with very low lung-to-head ratio that would usually be associated with 100% mortality at our institution. Two diagnoses of bronchopulmonary sequestration were reversed after final pathology revealed liver tissue. CONCLUSION: Given the limited series, we cannot conclude that bronchopulmonary sequestration confers an anatomic advantage to patients that have CDH. We did observe survivors in this group that, given their antenatal predictors of CDH severity, would ordinarily have dismal prognosis. The presence of a sequestration may be protective in a subset of patients with severe CDH, or may confound our antenatal predictors of disease severity in these patients.

Congenital diaphragmatic hernia associated with spinal anomalies.

OBJECTIVE: Increased mortality is seen in patients with congenital diaphragmatic hernia who have associated anomalies, such as cardiac defects. We reviewed our series of patients with congenital diaphragmatic hernia and spinal anomalies to evaluate if their prognosis was altered. METHODS: We examined our institutional database from August 1995 to August 2005, examining 679 cases of congenital diaphragmatic hernia, identifying all fetuses and newborns with congenital diaphragmatic hernia and spinal anomalies. Patients who had both congenital diaphragmatic hernia and spinal anomalies were identified by prenatal ultrasound reports, postnatal radiographs, operative notes, and pathology reports. RESULTS: Seven patients were identified in the fetal or neonatal period with concomitant diagnoses of congenital diaphragmatic hernia and spinal anomalies. All patients had normal karyotype analysis. Six of these patients had both defects diagnosed using prenatal ultrasonography. All patients died before hospital discharge. CONCLUSION: Although the numbers are limited in our series, patients with congenital diaphragmatic hernia and spinal anomalies have a dismal prognosis. This finding has significant implications for prenatal counseling. LEVEL OF EVIDENCE: III.

Reliability of the lung-to-head ratio as a predictor of outcome in fetuses with isolated left congenital diaphragmatic hernia at gestation outside 24-26 weeks.

OBJECTIVE: The purpose of this study was to investigate the relationship between lung-to-head ratio (LHR) and gestational age (GA) in fetuses with isolated left congenital diaphragmatic hernia and to determine the applicability and reliability of LHR to predict postnatal outcome beyond 24-26 weeks of gestation. STUDY DESIGN: The institutional review board approved this retrospective review of the University of California, San Francisco, Fetal Treatment Center database for cases with left congenital diaphragmatic hernia who were referred between March 1995 and June 2004. LHR was determined at the initial evaluation. One hundred seven live-born fetuses at 20-34 weeks of gestation (excluding cases that were lost to follow-up, with factors that potentially could influence the LHR measurement or postnatal outcome, or that were terminated electively). RESULTS: The median GA at LHR measurement was 25.6 weeks; the median LHR was 1.01; the median GA at birth was 37.7 weeks; and the overall survival rate was 59% (64/107). The median LHR of nonsurvivors was significantly lower than that of survivors, but neither GA at LHR measurement nor at delivery was significantly different between the groups. Multiple logistic regression analysis confirmed LHR to be an independent predictor of postnatal survival, and receiver-operator characteristic curve analysis demonstrated that an LHR of > or = 0.97 has the highest performance in predicting postnatal survival. When fetuses were grouped by GA at initial LHR measurement to determine reliability of LHR, specifically with respect to GA, in the 26-34 and 24-26 weeks of gestation groups, median LHR of survivors was significantly higher than that of nonsurvivors, and receiver-operator characteristic curve analysis confirmed LHR to be a reliable predictor of postnatal survival. However, for fetuses at 20-24 weeks of gestation, there was a trend toward a higher LHR in survivors, although this did not reach statistical significance. CONCLUSION: A significant positive linear relationship exists between LHR and GA at the time of measurement, such that LHR reliably predicts postnatal survival in fetuses with left congenital diaphragmatic hernia at 24-34 weeks of gestation and less reliable at 20-24 weeks. However, given the limitations of a retrospective, cross-sectional study, further prospective longitudinal studies that will investigate the change of LHR with GA and its association with fetal outcome are necessary.

Congenital hyperinsulinism in an infant caused by a macroscopic insulin-producing lesion.

Congenital hyperinsulinism is the most common cause of persistent neonatal hypoglycemia. Severe congenital hyperinsulinism is most often due to inactivating mutations in either the ABCC8 or KCNJ11 genes, which encode the SUR1 and Kir6.2 proteins, respectively--the two components of the ATP-sensitive K+ (KATP) channel; neonatal hypoglycemia due to macroscopic insulin-producing pancreatic lesions or adenomas are extremely rare. KATP channel hyperinsulinism is classified as diffuse or focal, the latter being associated with paternally-derived mutations of ABCC8 or KCNJ11 and somatic loss of heterozygosity of the maternal alleles. KATP channelopathies usually produce microscopic intra-pancreatic lesions and are typically unresponsive to drug therapy, requiring > 95% pancreatectomy for diffuse disease and occasionally more limited pancreatic resection for focal disease; macroscopic pancreatic lesions and adenomas are focally excised. We describe a 1 month-old infant with severe congenital hyperinsulinism who had a macroscopic insulin-producing pancreatic lesion successfully treated with focal lesion enucleation.

Outcomes of multigestational pregnancies affected by congenital diaphragmatic hernia.

BACKGROUND/PURPOSE: The prognosis for multiple vs singleton pregnancies affected by congenital diaphragmatic hernia (CDH) is not known. To improve the counseling of families with multiple gestation pregnancies complicated by CDH, we examined outcomes of a consecutive series of CDH cases occurring in multiple gestation pregnancy referrals. METHODS: Clinical characteristics and morbidity and mortality data were gathered for a consecutive series of infants with CDH from 16 multiple gestation pregnancies. Outcomes were compared to a cohort of 91 patients with CDH from singleton pregnancies. Multivariate regression was also used in an attempt to determine whether multiple gestation pregnancy was independently predictive of subsequent long-term adverse outcomes. RESULTS: Four pregnancies were lost to follow-up, and 1 underwent selective reduction. Overall mortality for live-born multiple gestation fetuses affected by CDH was 30% and was 8% for unaffected siblings. No pregnancy was concordant. Clinical features were not different between the case series and control infants, except median gestational age at delivery, which was significantly lower for the multigestational infants (34 [range, 32-36] vs 38 [range, 28-41] weeks) (P = .02). Long-term morbidity was comparable between cases and controls. CONCLUSIONS: In terms of mortality, outcomes of multigestational pregnancies affected by CDH are no worse than for CDH pregnancies in general. Long-term risk may depend more on CDH severity rather than the presence of multiple fetuses.

In utero repair of gastroschisis in the sheep (Ovis aries) model.

OBJECTIVE: Gastroschisis is associated with inflammatory changes in the exposed bowel which leads to intestinal dysmotility after postnatal repair. The insult is a combined effect of amniotic fluid exposure and mechanical constriction. We hypothesized that in utero anatomic repair is possible in a sheep model, and that it may halt the inflammatory damage caused by both mechanisms. METHODS: Gastroschisis was surgically created in mid-gestation (day 75) in 8 sheep fetuses. On gestational day 100, 2 fetuses underwent open fetal gastroschisis repair, where the eviscerated bowel was returned to the peritoneal cavity, and the abdominal wall was primarily closed. All fetuses were harvested at 135 days of gestation. RESULTS: Six fetuses survived the initial operation, and both fetuses that underwent gastroschisis repair survived to term. At 100 and 135 days of gestation, the eviscerated bowel showed progressive signs of inflammation and peel development. The gross and microscopic inflammatory changes in the gastroschisis bowel at 100 days of gestation were completely resolved at term following in utero repair. CONCLUSION: In utero anatomic repair of gastroschisis is possible in mid-gestation in the fetal lamb model, and it appears to ameliorate the inflammatory process.

Conflicts in wound classification of neonatal operations.

BACKGROUND/PURPOSE: This study sought to determine the reliability of wound classification guidelines when applied to neonatal operations. METHODS: This study is a cross-sectional web-based survey of pediatric surgeons. From a random sample of 22 neonatal operations, participants classified each operation as "clean," "clean-contaminated," "contaminated," or "dirty or infected," and specified duration of perioperative antibiotics as "none," "single preoperative," "24 hours," or ">24 hours." Unweighted kappa score was calculated to estimate interrater reliability. RESULTS: Overall interrater reliability for wound classification was poor (kappa = 0.30). The following operations were classified as clean: pyloromyotomy, resection of sequestration, resection of sacrococcygeal teratoma, oophorectomy, and immediate repair of omphalocele; as clean-contaminated: Ladd procedure, bowel resection for midgut volvulus and meconium peritonitis, fistula ligation of tracheoesophageal fistula, primary esophageal anastomosis of esophageal atresia, thoracic lobectomy, staged closure of gastroschisis, delayed repair and primary closure of omphalocele, perineal anoplasty and diverting colostomy for imperforate anus, anal pull-through for Hirschsprung disease, and colostomy closure; and as dirty: perforated necrotizing enterocolitis. CONCLUSIONS: There is poor consensus on how neonatal operations are classified based on contamination. An improved classification system will provide more accurate risk assessment for development of surgical site infections and identify neonates who would benefit from antibiotic prophylaxis.

Determination of risk factors for deep venous thrombosis in hospitalized children.

PURPOSE: Our objective was to determine the time trend and risk factors for deep venous thrombosis (DVT) of the lower extremities among pediatric inpatients. METHODS: This cross-sectional study used the data from the Health Care Cost and Utilization Project Kids' Inpatient Database for the years of 1997, 2000, and 2003 to estimate the DVT prevalence and crude and adjusted prevalence ratios. Patients between the ages of 1 and 17 years and who were hospitalized for at least 4 days were included. RESULTS: The weighted prevalence of DVT was 4.2 per 1000 hospital discharges (95% confidence interval [CI], 3.4-3.7). Independent of age, the prevalence of DVT was significantly greater in 2000 and 2003 compared to 1997, prevalence ratio (PR) of 1.2 and 1.4 (95% CI, 1.1-1.3 and 1.2-1.4). Using only the 2003 database, adjusted analysis revealed that patients at highest risk were those in the age range of 15 to 17 years (PR, 2.0; 95% CI, 1.6-2.4) and with the following comorbid conditions: obesity (PR, 2.1; 95% CI, 1.5-2.8), inflammatory bowel disease (PR, 1.8; 95% CI, 1.2-2.7), hematologic malignancy (PR, 2.5; 95% CI, 2.0-3.1), and thoracoabdominal (PR, 1.8; 95% CI, 1.6-2.2) or orthopedic (PR, 2.2; 95% CI, 1.7-2.8) operations. Predictors not associated with DVT included sex and diagnosis of trauma. CONCLUSIONS: The discharge diagnosis of DVT of the lower extremities has significantly increased since 1997. In addition, teenagers with underlying disorders are at highest risk for DVT.

Thoracoscopic versus open resection for congenital cystic adenomatoid malformations of the lung.

PURPOSE: This study evaluated the potential advantages of thoracoscopy compared to thoracotomy for resection of congenital cystic adenomatoid malformations (CCAM). METHODS: We conducted a retrospective chart review of consecutive cases of CCAM resection at University of California San Francisco Children's Hospital from January 1996 to December 2006. RESULTS: Thirty-six cases of postnatal CCAM resections were done over the past 10 years; 12 patients had thoracoscopic resections, whereas 24 patients had open resections. Patients in the thoracoscopic group had significantly longer operative time (mean difference of 61.3 minutes; 95% confidence interval [CI], 30.5-92.1) but shorter postoperative hospital stay (mean difference of 5.7 days; 95% CI, 0.9-10.4) and duration of tube thoracostomy (mean difference of 2.6 days; 95% CI, 0.7-4.5) and lower odds of postoperative complications (odds ratio of 9.0 x 10(-4); 95% CI, 8.0 x 10(-6)-0.1). In the subgroup analysis of only asymptomatic patients, the thoracoscopy group still had a significantly shorter hospital stay (mean difference of 2.8 days; 95% CI, 0.7-4.8). There was also a pattern for reduced complications in the thoracoscopy group (OR, 0.13; 95% CI, 0.02-1.0; P = .05). The average hospital costs were similar in both groups. With a conversion rate of 33% (6/18), patients with a history of preoperative respiratory symptoms had a higher incidence of conversion than those who were asymptomatic (66.7% vs 0%, P = .005). These four patients had a history of pneumonia. CONCLUSION: Minimally invasive resection of CCAM results in longer operative time but shorter hospital stay, potentially reduced complications, and no additional hospital costs. Thoracoscopic lobectomy in patients with a history of pneumonia is challenging and a risk factor for conversion to thoracotomy.

Indication for pediatric muscle biopsy determines usefulness.

PURPOSE: Diagnostic skeletal muscle biopsy is an invasive procedure used for evaluation of neuromuscular disorders. We hypothesize that the yield of biopsy varies with its indication or suspected diagnosis. METHODS: Retrospective review of all muscle biopsies was performed at an academic tertiary care center between January 1, 1996, and August 1, 2006. RESULTS: A total of 142 muscle biopsies were performed on 127 children. Mean age at biopsy was 5.3 years (median, 3.3; range, 8 days-21 years) with 48% female. Follow-up was maintained for a mean 3.4 years (median, 2.2; range, 1 month-10.4 years). Specific pathological diagnoses were obtained from 33 (23%) of 142. Changes in therapy resulted from 11 (8%) of 142 biopsies. Treatment changed for all patients with prebiopsy suspicion of inflammatory or neoplastic processes (7/7, 100%); these accounted for 7 (64%) of 11 patients with treatment changes. Thirteen patients underwent multiple biopsies, with 1 (8%) patient's treatment adjusted as a result. Fifteen neonates (<100 days) underwent a total of 17 biopsies; none changed treatment plans. CONCLUSIONS: Muscle biopsy for neuromuscular disorders is inconsistently useful. Specific diagnoses are occasionally revealed, but treatment changes are infrequent except in those patients with suspected neoplastic or inflammatory disease. Ongoing study is required to determine the most rational indications for this procedure.

Fetal intervention for mass lesions and hydrops improves outcome: a 15-year experience.

PURPOSE: The natural history of certain prenatally diagnosed masses is well known. Large thoracic mass lesions can evolve one of 2 ways, either to regress and cause minimal morbidity, or to progress and enlarge, often resulting in hydropic changes in the fetus. This nonimmune hydrops carries a dismal prognosis, with nearly all fetuses expiring before or shortly after birth. However, hydrops associated with fetal mass lesions can be halted and even reversed with fetal intervention and treatment of the underlying defect. We examined our patients with fetal mass lesions to evaluate survival after intervention. METHODS: Institutional approval was obtained by the Committee on Human Research. A retrospective review was performed of 294 fetuses evaluated over 15 years with large mass lesions. All patients were evaluated for evidence of fetal hydrops using ultrasound criteria. Patients were divided according to type of intervention. Primary outcome measure was 30-day survival after birth. RESULTS: (1) Patients without fetal hydrops did not undergo fetal intervention and survived to 30 days after birth (167/172, 97%). (2) Patients with fetal mass lesions that developed hydrops fared poorly with no intervention (1/33 survival, 3%), whereas fetuses undergoing prenatal intervention fared much better (15/30 open, 50%; 3/10 percutaneous, 30%). (3) Four patients with hydropic congenital cystic adenomatoid malformation (n = 3) or pulmonary sequestration (n = 1) received steroids in preparation for surgery but underwent no intervention, and the patients survived the neonatal period. CONCLUSION: Fetuses with prenatal diagnoses of masses not associated with hydrops have excellent prognosis with survival higher than 95%. Nonimmune hydrops associated with prenatal diagnosis of a fetal mass is a devastating complication with less than 5% survival. Open resection of a mass causing hydrops resulted in 50% survival, with reversal of hydrops in a group with near-uniform fatality. Further investigation is warranted regarding the use of minimally invasive prenatal therapies including steroid administration for hydropic fetuses.

Surgical treatment of childhood recurrent pancreatitis.

BACKGROUND/PURPOSE: Surgical intervention that improves pancreatic ductal drainage is a reasonable treatment strategy for recurrent pancreatitis in children. METHODS: This study was approved by the Committee on Human Research (San Francisco, CA). A retrospective chart review was performed on children aged 0 to 17 years given the International Classification of Diseases, Ninth Revision coding diagnosis of chronic pancreatitis who underwent surgical intervention from 1981 to 2005. RESULTS: From 1981 to 2005, 32 children were treated for the diagnosis of chronic pancreatitis. The etiologies were obstructive (n = 13), idiopathic (n = 10), hereditary (n = 6), medications (n = 2), and infection (n = 1). Fifteen patients underwent 17 operations for chronic pancreatitis, including Puestow (n = 9), cystenterostomy (n = 2), Whipple (n = 1), distal pancreatectomy (n = 1), Frey (n = 1), DuVal (n = 1), excision of enteric duplication cyst (n = 1), and pancreatic ductal dilation (n = 1). The mean age at presentation of patients undergoing surgery was 6.0 +/- 4.1 years (mean +/- SD). The mean time from presentation to operation was 3.3 +/- 3.3 years. There were no deaths after surgical intervention. Of 15 patients, 2 (13%) required rehospitalization within 90 days of surgery, one for bowel obstruction, the other for splenic infarction. The median length of stay postoperatively was 8 days (range, 5-66 days). CONCLUSIONS: Chronic pancreatitis in children differs markedly in etiology when compared with adults. In most cases seen in our institution, chronic pancreatitis resulted from ineffective ductal drainage. These disorders are amenable to surgical decompression, which, ultimately, can prevent disease recurrence.

Fetal surgery for congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) continues to account for significant mortality in neonates. Advances in postnatal therapies have reduced mortality rates in the less severely affected infants, though surviving infants continue to display significant comorbid conditions. The history of fetal treatment for CDH is a microcosm for the development of fetal therapy in general. This paper traces this history from early clinical observations and imaging advances to experimental animal studies and finally to safe human application and development of clinical trails. Encouraging recent results and improved outcomes lend credence to the idea than prenatal interventions may give clinicians and families more hope.

Monochorionic twin gestation complicated by a sacrococcygeal teratoma with hydrops--optimal timing of delivery allows for survival of the unaffected twin.

Sacrococcygeal teratomas are the most common tumors found in newborns and have a favorable outcome when diagnosed late in gestation. Solid, highly vascularized tumors diagnosed early in gestation have a higher incidence of hydrops fetalis and fetal demise. The following is a case report of a monochorionic twin gestation complicated by a solid highly vascular sacrococcygeal teratoma and the development of hydrops in the affected twin. In this case, serial ultrasound evaluations and timing of delivery optimized the outcome of the unaffected twin.