Sacrococcygeal teratoma: late recurrence warrants long-term surveillance.

BACKGROUND: Sacrococcygeal teratoma (SCT) is a rare childhood malignancy. Although overall survival is favorable, recurrent tumors are associated with poor outcomes. As most recurrences occur within 3 years of presentation, the utility of long-term surveillance is uncertain. METHODS: Patients with SCTs evaluated and managed by our pediatric surgery department between 1986 and 2013 were included. Details pertaining to laboratory values, operative findings, tumor histology, management, recurrence, and outcomes were recorded and analyzed. RESULTS: During the study period, 40 children with the diagnosis of SCT were managed by our practice. Five (13%) developed a recurrence. The median age at the initial resection was day of life two (range day of life 0-2.5 years). The median time to recurrence was 5 years (range 5 months-15 years). Among those with recurrences, mature teratoma was the most common histological type on the initial resection (n = 3), with yolk sac and immature teratomas comprising the other two. At the time of recurrence, three patients had mature teratomas, and all are alive and well following resection. Two patients had yolk sac tumors at the time of recurrence and both died. CONCLUSION: SCT can recur many years after the initial resection. Our findings suggest that all patients with SCT should be closely followed into adulthood.

Closer Look at the Nutritional Outcomes of Patients After Primary Repair of Congenital Diaphragmatic Hernia.

OBJECTIVES: We hypothesize that the patients after primary repair of congenital diaphragmatic hernia (CDH) can have poor nutritional outcomes and plan to identify risk factors to further stratify these patients. METHODS: Retrospective cohort of patients who had primary repair of CDH between 2000 and 2014 and had follow-up at our institution. Z scores (weight for age and weight for length) were calculated using the World Health Organization and Centers for Disease Control and Prevention growth standards. RESULTS: For the 67 patients in the cohort, the median age at the time of repair was 3 days (interquartile range 2-5) and at the time of discharge was 20.5 days (interquartile range 16-30). Fifteen percent of the patients required supplemental tube feeding for inadequate oral intake and 69% required fortified feedings for inadequate growth at discharge (4 patients subsequently needed gastrostomy tube placement). The median z scores at discharge were -1.0 (-2.1 to -0.3) and -1.2 (-2.3 to -0.5) in weight for age and weight for length, respectively. The risk factors for low z scores included open repair and longer periods of postoperative intubation or hospitalization. The z scores were similar at 6 months of age compared to discharge, but then statistically improved at 12 months of age. CONCLUSIONS: Patients with primary repair of CDH are at risk for poor nutritional outcomes at the time of hospital discharge and require follow-up to ensure adequate growth. Patients at highest risk are those who had an open repair and had prolonged intubation or hospitalization.

Tumor metrics and morphology predict poor prognosis in prenatally diagnosed sacrococcygeal teratoma: a 25-year experience at a single institution.

PURPOSE: Some fetuses with sacrococcygeal teratoma (SCT) develop hydrops, but there is no consensus on an appropriate prognostic marker for poor prognosis. The purpose of this study is to establish predictors of poor prognosis in fetuses with SCT. METHODS: A retrospective review of patients with prenatally diagnosed SCT from 1986 to 2011 was performed. Patients with outcome data and ultrasound exams before 32 weeks gestational age (GA) were included (n=37). Tumor volume-to-fetal weight ratio (TFR) and tumor morphology were assessed as sonographic predictors of poor prognosis. RESULTS: Twelve patients (32%) had good prognosis, and twenty-five patients (68%) had poor prognosis. All patients with poor prognosis had a morphology score ≥ 3, which is a significant predictor of poor prognosis (p <0.0001). TFR was assessed, and a receiver operating characteristic (ROC) analysis identified a cutoff value of 0.12 before 24 weeks GA and 0.11 before 32 weeks GA as predictors for poor prognosis. TFR is a significant predictor of poor prognosis (p<0.0001). CONCLUSIONS: Patients with cystic SCT all had good prognosis. TFR >0.12 was validated as a sonographic predictor of poor prognosis. TFR and tumor morphology can be used to counsel expectant families with prenatally diagnosed SCT regarding prognosis.

Magnetic mini-mover procedure for pectus excavatum III: safety and efficacy in a Food and Drug Administration-sponsored clinical trial.

PURPOSE: The magnetic mini-mover procedure (3MP) uses magnetic force to gradually remodel pectus excavatum deformity. A magnet is implanted on the sternum and coupled with an external magnetic brace. Under Investigational Device Exemption and Institutional Review Board approval, we performed a pilot study of safety, probable efficacy, and cost-effectiveness of this new treatment of an orphan disease using an implantable pediatric device. METHODS: Ten otherwise healthy patients, ages 8 to 14 years, with severe pectus excavatum (pectus severity index [PSI] > 3.5) underwent 3MP treatment (mean, 18.8 ± 2.5 months). Safety was assessed by postimplant and postexplant electrocardiograms and monthly chest x-rays. Efficacy was assessed by change in pectus severity index as measured using pretreatment and posttreatment computed tomographic scan. Cost of 3MP was compared with that of standard procedures. RESULTS: The 3MP device had no detectable ill effect. Device weld failure or malpositioning required revision in 5 patients. Average wear time was 16 h/d. Pectus severity index improved in patients in the early or mid puberty but not in patients with noncompliant chest walls. Average cost for 3MP was $46,859, compared with $81,206 and $81,022 for Nuss and Ravitch, respectively. CONCLUSION: The 3MP is a safe, cost-effective, outpatient alternative treatment for pectus excavatum that achieves good results for patients in early and midpuberty stages.

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.

Long-term surgical outcomes in congenital diaphragmatic hernia: observations from a single institution.

BACKGROUND/PURPOSE: Surgical complications are common in survivors of congenital diaphragmatic hernia (CDH), but little is known about long-term incidence patterns and associated predictors. METHODS: A cohort of 99 CDH survivors was prospectively followed at a single-institution multidisciplinary clinic. Data were gathered regarding the adverse surgical outcomes of hernia recurrence, chest and spinal deformity, and operative small bowel obstruction (SBO), and then were retrospectively analyzed in relation to perinatal and perioperative markers of disease severity to determine significant predictors. Statistical methods used included univariate and multivariate regression analysis, hazard modeling, and Kaplan-Meier analysis. RESULTS: At a median cohort age of 4.7 (range, 0.2-10.6) years, 46% of patients with patch repairs and 10% of those with primary repairs had a hernia recurrence at a median time of 0.9 (range, 0.1-7.3) years after repair. Chest deformity was detected in 47%. Small bowel obstruction and scoliosis occurred in 13%. Recurrence and chest deformity were significantly more common with patch repair, liver herniation, age at neonatal extubation greater than 16 days, oxygen requirement at discharge, and prematurity. The strongest predictor of SBO was patch repair. Multivariate analysis showed that patch repair was independently predictive of recurrence and early chest deformity (odds ratios of 5.0 and 4.8, confidence intervals of 1-24 and 1-21, P < .05). Use of an absorbable patch was associated with the highest risk of surgical complications. CONCLUSIONS: For long-term survivors of CDH, specific perinatal and operative variables, particularly patch repair, are associated with subsequent adverse surgical outcomes.

Magnetic mini-mover procedure for pectus excavatum II: initial findings of a Food and Drug Administration-sponsored trial.

PURPOSE: The Magnetic Mini-Mover Procedure (3MP) uses a magnetic implant coupled with an external magnet to generate force sufficient to gradually remodel pectus excavatum deformities. This is an interim report of the evolution of the 3MP during a Food and Drug Administration-approved clinical trial. METHODS: After obtaining Institutional Review Board approval, we performed the 3MP on 10 otherwise healthy patients with moderate to severe pectus excavatum deformities (age, 8-14 years; Haller index >3.5). Operative techniques evolved to improve ease of implantation. Patients were evaluated monthly by a pediatric surgeon and orthotist. Electrocardiograms were performed pre- and postoperatively. Sternal position was documented by pre- and postprocedure computed tomographic scan, interval chest x-ray, depth gauge, and interval photographs. RESULTS: There was no detectable effect of the static magnetic field on wound healing or cardiopulmonary function. No detectable injuries and minimal skin changes resulted from brace wear. Operative techniques evolved to include a custom sternal punch and a flexible guide wire to guide the posterior plate into position behind the sternum, reducing outpatient operating time to one-half hour. In 9 patients, the procedure was performed as an outpatient basis; and 1 patient was observed overnight. Three patients required evacuation of retained pleural air postoperatively, and 2 required an outpatient revision. A custom-fitted orthotic brace (Magnatract) was extensively modified to increase user friendliness and functionality while incorporating several novel functions: a screw displacement mechanism so patients can easily self-adjust magnetic force, a miniature data logger to measure force and temperature data every 10 minutes, and an interactive online Web portal for remote patient evaluation. All attempts to quantitate sternal position (radiographic, fluid volume, and depth gauge) were inadequate. Visual assessment remains the best indicator. CONCLUSIONS: In this interim report, the 3MP appears to be a safe, minimally invasive, outpatient, cost-effective alternative treatment of pectus excavatum. Outcomes will be reported upon the completion of this phase II clinical trial.

Thoracoscopic resection of foregut duplication cysts.

BACKGROUND: Foregut duplications are rare entities that include both esophageal and bronchogenic cysts. The diagnosis of foregut duplication cyst is made most often from an incidental finding on chest radiograph, or due to respiratory compromise due to mass effect or infection. Treatment consists of complete resection. Recurrences are associated with incomplete resection. Six cases of foregut duplication cysts are presented that were resected thoracoscopically. MATERIALS AND METHODS: From May 1998 to April 2003, six patients underwent thoracoscopy for resection of foregut duplication cyst. One patient required conversion to open thoracotomy due to esophageal perforation. The distribution of cysts was 4 on the left and 2 on the right; all procedures were performed with three or four ports. Single lung ventilation was used in three patients. The masses were removed via a port site after intrathoracic decompression. Chest tubes were placed in all patients, and most were removed within 12 hours. RESULTS: Five of six cases underwent successful thoracoscopic resection. Pathology demonstrated esophageal duplication cyst in three patients and bronchogenic cyst in the other three patients. Average hospital stay was 5.5 days. Complications included aspiration pneumonia and chest tube dislodgment. There were no deaths, and no recurrences. CONCLUSION: Thoracoscopic resection is a safe and effective method of treating foregut duplications. Outcomes have been good with little short-term morbidity and no mortality. Morbidity and cosmesis are improved by avoiding thoracotomy. Thoracoscopic resection should be considered the first-line therapy for these benign masses.

Congenital teratoma: a clinicopathologic study of 22 fetal and neonatal tumors.

Extragonadal teratoma is the most common congenital tumor. The prognostic significance of the grade of immaturity and the presence of small foci of conventional yolk sac tumor (YST) in fetal and neonatal teratomas have not been determined. We report detailed histologic studies of 22 congenital teratomas, including eight tumors resected in utero for developing hydrops, and correlate the histologic features with initial serum alpha-fetoprotein (AFP) levels and clinical outcome. All fetal tumors that required in utero intervention were grade 3 immature teratomas, with admixed conventional YST in 44%. Among tumors resected postnatally, those presenting in utero were more commonly immature (71% vs. 50%). All initial post-surgical serum AFP levels were high, as expected in a neonate. No correlation was found between AFP elevation above the mean for gestational age and the presence of YST, hepatic differentiation, or immature endodermal glands in the tumor. Among 15 survivors with follow-up, 5 patients had malignant mixed germ cell tumors (immature teratoma with foci of conventional YST) and 5 had immature teratomas with foci of hepatic differentiation or immature endodermal glands with subnuclear vacuoles (so-called "well-differentiated YST"). No patient has developed recurrent or metastatic disease after treatment by complete surgical excision alone (mean follow-up, 37.6 months). The clinical behavior of congenital teratomas is determined predominantly by whether or not the tumor can be completely resected and in our study did not correlate with the grade of the teratoma or with the presence or absence of foci of hepatic tissue, immature intestinal glands, or foci of conventional YST.

Initial experience with complex laparoscopic biliary surgery in children: biliary atresia and choledochal cyst.

BACKGROUND: Laparoscopic approaches are possible for many pediatric surgical diseases, including complex biliary disorders. The authors describe their early experience with laparoscopic procedures for biliary atresia and choledochal cysts. METHODS: A retrospective review was performed with Institutional Review Board (IRB) approval of children undergoing laparoscopic Kasai portoenterostomy (n = 2) or laparoscopic excision of a choledochal cyst with Roux-en-y biliary reconstruction (n = 3). The authors report the operative technique for these procedures and present their initial results. RESULTS: All procedures were performed with 4 to 5 trocars using 3- and 5-mm ports. Excision of the fibrous biliary remnant (biliary atresia) or of the cyst (choledochal cysts) was performed laparoscopically in all cases. The Roux-en-y limb was created through a 1-cm extension of the umbilical port site. Laparoscopic biliary reconstruction was performed successfully in 4 of 5 patients. In one child, the proximal extent of the choledochal cyst was at the bifurcation of the hepatic duct, and the biliary reconstruction was performed via an open incision with separate anastomoses of the right and left ducts. The gallbladder was used as a handle for retraction of the liver for portal visualization, as in a cholecystectomy, and was resected at the end of the procedure. All children with choledochal cysts have normal bilirubin values. One of 2 children who underwent laparoscopic Kasai has a normal postoperative bilirubin level, whereas the other child did not drain bile and underwent a successful liver transplantation. Mean length of surgery was 338 minutes; mean length of stay was 5.5 days. There were no significant postoperative complications. Cosmesis was excellent in all cases. CONCLUSIONS: Laparoscopic approaches for children with biliary atresia and choledochal cysts are possible. Excellent visualization of the portal structures can be achieved laparoscopically with adequate retraction of the liver. Further follow-up is needed to determine broader application.

Antimicrobial prophylaxis for gastrointestinal procedures: current practices in North American academic pediatric programs.

BACKGROUND: Guidelines for the use of antibiotic prophylaxis in children are based on a small number of studies that assess the risk of infection associated with performing endoscopic procedures. The American Heart Association (AHA) and the American Society of Gastroenterological Endoscopy (ASGE) have established guidelines that identify conditions and procedures that place a child at greater risk for infectious complications. Because data on bacteremia and sepsis associated with endoscopy in children are very limited, we reviewed the practices of 15 large academic pediatric gastroenterology services to see if patterns of practice and safety could be determined. METHODS: A questionnaire was sent to 15 academic gastroenterology centers in the United States and Canada asking about antibiotic prophylaxis for endoscopic procedures for children with six conditions. These included three conditions related to congenital heart disease based on negligible, moderate, and high risk for endocarditis; immune compromise; the presence of a central venous line; and the presence of a ventriculo-peritoneal shunt. Six procedures were evaluated, including esophagogastroduodenoscopy with biopsy, flexible sigmoidoscopy or colonoscopy with biopsy, endoscopic retrograde cholangiopancreatography, esophageal sclerotherapy, esophageal dilation, and percutaneous endoscopic gastrostomy (PEG) tube placement. RESULTS: The patterns of reported practice generally conform to the AHA and ASGE guidelines. The six conditions and six procedures yielded 36 response categories for the participating centers. The majority of centers reported routine use of antibiotic prophylaxis in about half (17) of the response categories, which represented three distinct situations. These included children with congenital heart disease having moderate or high risk for bacterial endocarditis for almost all procedure categories and children undergoing PEG tube placement regardless of underlying condition. In all other combinations of underlying conditions and procedures, the majority of centers did not use routine prophylaxis. The majority of centers did not use antibiotic prophylaxis for cardiac conditions with a negligible risk of infectious complication or for children with immunocompromise, central venous lines, or ventriculo-peritoneal shunts for any procedure except PEG placement. CONCLUSIONS: These results indicate that the routine use of antibiotic prophylaxis is limited in pediatric academic centers to a few very specific conditions and procedures. The results also provide indirect evidence that the risk of infectious complications associated with endoscopic procedures appears to be exceedingly low.