Background. A common site for neonatal intestinal obstruction is the duodenum. Delayed establishment of enteral nutritional autonomy continues to challenge surgeons and, since early institution of nutritional support is critical in postoperative newborns, identification of patients likely to require alternative nutritional support may improve their outcomes. Therefore, we aimed to investigate risk factors leading to delayed establishment of full enteral nutrition in these patients. Methods. 87 patients who were surgically treated for intrinsic duodenal obstructions from 1998 to 2012 were reviewed. Variables were tested as potential risk factors. Median time to full enteral nutrition was estimated using the Kaplan-Meier method. Independent risk factors of delayed transition were identified using the multivariate Cox proportional hazards regression model. Results. Median time to transition to full enteral nutrition was 12 days (interquartile range: 9–17 days). Multivariate Cox analysis identified three significant risk factors for delayed enteral nutrition: gestational age (GA) ≤ 35 weeks (P < .001), congenital heart disease (CHD) (P = .02), and malrotation (P = .03). Conclusions. CHD and Prematurity are most commonly associated with delayed transition to full enteral nutrition. Thus, in these patients, supportive nutrition should strongly be considered pending enteral nutritional autonomy.

Purpose: The purpose of this study was to evaluate recurrence and survival outcomes in pediatric adrenal cortical neoplasms.Methods: A 90-year retrospective review of children with adrenal cortical neoplasms was performed using multivariate Cox regression analysis to identify factors associated with recurrence and tumor-related mortality.Results: The evaluable cohort included 29 patients. Twenty-seven underwent resection. Twenty-two (81%) had localized disease, and 5 (19%) had locally advanced disease (all received chemotherapy and 2 of 5 were cured). Two patients presenting with metastatic disease died despite treatment. There were 4 recurrences; all patients died. Tumor-related mortality was 24% (7/29). Kaplan-Meier freedom from recurrence was 85% at 1 year (95% confidence interval, 75%-95%). Multivariate Cox regression revealed that older age (P = .01), higher mitotic rate (P = .005), and necrosis (P < .001) were independent predictors of tumor-related death. Higher mitotic rate (P = .007) and larger tumor size (P = .03) were significant predictors of tumor recurrence.Conclusion: Risk factors for poor outcomes in patients with adrenocortical tumors include older age, higher mitotic rate, higher percent necrosis, and larger tumor size. Therefore, the presence of these factors may warrant consideration of adjuvant chemotherapy, even in the absence of advanced disease. (C) 2011 Elsevier Inc. All rights reserved.

Background/Purpose: Severe neonatal pulmonary hypoplasia incurs mortality rates approaching 71% to 95%. We sought to determine the utility of serial amnioinfusions through a subcutaneously implanted intraamniotic catheter to prevent pulmonary hypoplasia in fetal obstructive uropathy.Methods: Fetal lambs (n = 32) were divided into 3 groups. Group I (n = 12) underwent a sham operation, group II (n = 15) underwent a complete urinary tract obstruction via ligation of the urachus and urethra with a subcutaneous tunneled intraamniotic port-a-cath without amnioinfusions, and group III (n = 5) underwent a creation of a complete urinary tract obstruction with a port-a-cath as described in group II with serial amnioinfusions. Lung tissue was analyzed by lung volume to body weight ratios and stereology. Statistical analysis was performed by analysis of variance and Bonferroni comparisons (P < .05).Results: Obstructed fetuses grossly had smaller lungs than treated and control animals. Lung volume to body weight ratios were statistically significant between groups. Airspace fractions were comparable between groups I and III (average = 0.53 and 0.55, respectively), although both were significantly greater than group II (average = 0.48) (P = .049).Conclusions: Serial amnioinfusions through an intraamniotic port-a-cath prevented pulmonary hypoplasia in an ovine model of complete obstructive uropathy. The use of an easily accessible device for amnioinfusions may be a viable option to treat oligohydramnios. (C) 2011 Elsevier Inc. All rights reserved.

Juvenile granulosa cell tumor (JGCT) is an uncommon gonadal stromal tumor that occurs rarely in the testis. We report a newborn boy with bilateral intra-abdominal JGCT presenting with abdominal distention and respiratory distress at birth. He was taken to the operating room emergently, and 2 large masses connected by gubernacula to the inguinal canals were resected. Associated abnormalities included a constitutional chromosome 4 abnormality, polymicrogyria, and renal cysts. This report describes a rare presentation of JGCT with abdominal compression and expands the literature to include bilateral testicular involvement. Additionally, it is the 1st report of JGCT associated with a chromosome 4 abnormality, highlighting a genetic region that may be important in JGCT development.

Background/Purpose: We aimed to determine whether the profile of matrix metalloproteinase (MMP) activity in fetal urine correlates with the degree of kidney damage in the setting of congenital obstructive uropathy.Methods: Fetal lambs underwent either a sham operation or creation of a complete urinary tract obstruction. Necropsies were performed before term, when urinary MMP profiling was performed by zymography; and kidney damage was assessed histologically by multiple semiquantitative analyses and histomorphometric measurements.Results: There was a significant correlation between inner medullary thickness and MMP-9 (P=.005) and 63-kd MMP-2 (P=.019) activities. In like manner, the only MMPs associated with kidney fibrosis were MMP-9 and 63-kd MMP-2. Matrix metalloproteinase-9 activity was a highly significant independent predictor of the total combined kidney fibrosis score (P < .001) as well as of higher fibrosis grades in each of 6 kidney areas analyzed (all with P < .01). The activity of 63-kd MMP-2 correlated significantly with higher fibrosis in select areas.Conclusions: In a fetal ovine model, urinary MMP activity correlates with the degree of kidney damage. The presence of MMP-9 (in particular) and that of 63-kd MMP-2 are independent predictors of severity. Prenatal urinary MMP profiling may enhance patient stratification and counseling in the setting of congenital obstructive uropathy. (C) 2010 Elsevier Inc. All rights reserved.

Background/Purpose The diagnostic evaluation, patient stratification, and prenatal counseling for congenital obstructive uropathy remain sub-optimal. Matrix metalloproteinase (MMP) expression profiles are emerging as a valuable diagnostic tool in assorted disease processes. We sought to determine whether congenital obstructive uropathy impacts MMP expression in fetal urine. Methods Fetal lambs (n = 25) were divided in two groups: group I (n = 12) underwent a sham operation and group II (n = 13) underwent creation of a complete urinary tract obstruction. Gelatin zymography panels for 4 MMP species were performed on fetal urine in both groups at comparable times post-operatively. Statistical analysis was by the Fisher's exact test (P < .05). Results Overall fetal survival was 80% (20/25). A variety of significant differences in MMP expression between the two groups were identified. The following profiles were present only in obstructed animals: any MMP other than MMP-2 (P = .029), including any MMP other than 63 kDa and 65 kDa (P = .009); 2 or more MMPs excluding MMP-2s (0.029); and 3 or more MMPs (P = .029). Conclusions Limited matrix metalloproteinase expression is present in the urine of normal ovine fetuses. Fetal obstructive uropathy impacts urinary MMP expression in various distinguishable patterns. Prenatal urinary MMP profiling may become a practical and valuable diagnostic tool in the evaluation of congenital obstructive uropathy.

PURPOSE:Primary lung tumors in children are rare. A wide range of histopathologic tumor types occurs. The incidence of these lesions and their outcomes are still largely unknown. This study aims to determine the incidence of different primary lung tumors in children and to contribute data leading to the development of evidence-based treatment models. METHODS:A single institution retrospective review was performed with institutional review board approval. Patients were included if they had primary, nonhematologic lung tumors. Simple squamous papillomas subjected to endoscopic biopsy and not resected, and vascular lesions associated with multisystem lesions, such as hereditary hemorrhagic telangiectasia, were excluded. Medical records and pathologic material for patients from 1918 to 2008 were reviewed. RESULTS:Forty patients were identified (23 boys, 17 girls) with a mean age of 9.6 years (range, 3 months to 19 years). Fourteen distinct histopathologic tumor types were identified. The most common tumor types were carcinoid (8), inflammatory myofibroblastic tumor (7), and pleuropulmonary blastoma (6). Rare pediatric lung tumors including small cell carcinoma, adenocarcinoma, and pulmonary capillary hemangiomatosis were also seen. The mortality rate was 17.5% (7) in our series. Chemotherapy was used in 23% (9) and radiation in 20% (8) of the patients. Of the 33 survivors, 28 had follow-up with a median duration of 29.5 months (mean, 63.2 months; range, 1-471 months). CONCLUSIONS:Primary lung tumors in children are rare and histopathologically diverse. The tumor spectrum involves many types not seen in adults, and unlike adults, patients rarely have a history of exposure to external predisposing factors. Although complete resection remains the standard for treatment of most tumors, addition of adjuvant therapy is dependent on both tumor stage and histopathologic type.

BACKGROUND Undifferentiated (embryonal) sarcoma (UES) of the liver is a malignant hepatic neoplasm accounting for 7% of pediatric hepatic tumors. Current use of multimodal therapy, including chemotherapy and surgery, has greatly improved survival. Tumor rupture is uncommon and, prior to the adjuvant use of sarcoma based chemotherapy regimens, was thought to be poor prognostic sign. CASE PRESENTATION AND DISCUSSION A 10-year-old girl presented with acute worsening of abdominal pain while being worked up for liver mass. At surgery, she was found to have a ruptured, 20 x 15 x 5 centimeter (cm) UES, the largest reported ruptured UES to be resected primarily. The size and position of her tumor required very aggressive surgery for complete resection. She subsequently received adjuvant chemotherapy and radiation. Twenty-four months following surgery she is doing well with no evidence of disease. CONCLUSION Despite tumor rupture, complete tumor resection gives the patient the best chance of long-term survival. Aggressive surgery is warranted if the tumor can be completely resected.

Purpose: Heterotaxy syndrome (HS) patients often present with congenital heart disease and intestinal malrotation. Controversy exists regarding the management of these patients. Risk of midgut volvulus, morbidity from elective operations, and overall prognosis must be weighed when considering a Ladd procedure on asymptomatic HS/intestinal malrotation patients.Methods: This is a retrospective review comparing HS and non-heterotaxy syndrome (NHS) patients undergoing a Ladd procedure at Children's Hospital Boston (Mass) from January 1997 to September 2007.Results: Thirty-one I-IS and 51 NHS patients were identified. After a Ladd procedure, HS patients remained in the hospital 12.9 days, had a 9.7% risk of small bowel obstruction, and a 9.7%, in-hospital mortality. If allowed to develop abdominal symptoms, 27% of HS patients with intestinal malrotation had a midgut volvulus at Surgery. Intra-HS group comparison demonstrated longer hospital stays in symptomatic patients (P = .01). Mortality was greater in the HS than NHS patients, but deaths were related to cardiac disease and not to the Ladd procedure.Conclusions: Elective Ladd procedures are well tolerated by HS patients. Given the risk of midgut volvulus and in light of improved survival beyond infanthood, once identified, HS patients with asymptomatic malrotation should be offered a prophylactic Ladd procedure. (C) 2009 Elsevier Inc. All rights reserved.

Mesenteric lipoblastoma is a rare tumor and, its presentation as a bowel obstruction with possible midgut volvulus has only been reported once before. A 7-year-old girl presented with nausea and vomiting but a benign abdominal examination. Upper gastrointestinal contrast study demonstrated possible malrotation with midgut volvulus. During emergency laparotomy, segmental small bowel volvulus secondary to a large mesenteric lipoblastoma was found. The lipoblastoma was resected with a segment of small bowel. Resection margins were negative for tumor, and the patient is doing well with no evidence of recurrence.

Hypopharyngeal perforation secondary to blunt trauma is a rare injury. It can be managed operatively or expectantly without clear criteria for either approach. Here, we present a case of a 17-year-old adolescent boy who had a hypopharyngeal tear from direct blunt trauma to the anterior neck received during a high school football game. Physical examination demonstrated cervical crepitus, and neck radiograph revealed air in the retropharyngeal space. Rigid endoscopy diagnosed a 3-cm linear tear in the posterior hypopharynx. The tear was repaired transorally using laparoscopic instruments and visualized by a rigid endoscope, followed by anterior neck dissection and drain placement. Postoperatively, he was kept nil per os and received intravenous antibiotics. He was discharged home and returned to the football field the same fall.

Purpose: The rarity and histopathologic diversity of primary pancreatic neoplasms in children have made it difficult to predict prognosis and to develop optimal management protocols.Methods: A 90-year (1918-2007), single institution, retrospective review of all patients with neoplastic pancreatic masses was performed.Results: Eighteen patients were identified with 7 distinct histopathologic subtypes. The most common were gastroenteropancreatic neuroendocrine, solid pseudopapillary, and acinar tumors. There were 6 benign and 12 malignant tumors. Six patients had disease outside the pancreas at their initial operation. There were 7 deaths (41%), 2 related to the initial operation, 3 from disease progression, 1 from a small bowel obstruction, and 1 from necrotizing pancreatitis. Five deaths were in patients with extrapancreatic disease found at initial operation. The median duration of follow-up for the 10 survivors was 41 months.Conclusion: In adults, pancreatic ductal adenocarcinoma is by far the most common histopathologic subtype, with other subtypes more common in children. Stage is an important prognostic factor. Long-term disease-free survival in childhood pancreatic malignancies is achievable with complete surgical resection, prognosis, and adjuvant treatment, depending on the histopathologic type. (C) 2009 Elsevier Inc. All rights reserved.

Congenital H-type rectovaginal fistulas and single ectopic bilateral ureters are each rare malformations. We describe a baby girl with a congenital rectovaginal fistula diagnosed 2 years after correction of single ectopic bilateral ureters. To our knowledge, this is the first association of these entities. Repair of fistula was complicated by recurrence, requiring a second procedure. The recommended operation for this anomaly requires separating the suture lines on the vagina and rectum. The practice of simply oversewing and then buttressing the suture lines is probably not sufficient.

Osteosarcoma is the most common osseous malignancy of childhood. Vascular reconstruction during resection is uncommon. However, when required, thrombosis is a major cause of failed limb salvage surgery. A 19-year-old patient required complex vascular reconstruction in conjunction with lower extremity osteosarcoma resection. Preoperatively, his case was complicated by an ipsilateral popliteal deep vein thrombosis presenting during neoadjuvant chemotherapy. At the time of resection, to increase vascular graft flow, a distal arteriovenous fistula was created between the posterior tibial artery and the greater saphenous vein. This case illustrates a patient with a complex extirpation procedure requiring concomitant vascular reconstruction. This technique should be considered when limb salvage with vascular resection is being attempted in a high-risk patient.

Congenital pyloric atresia (CPA) is a rare disorder that has traditionally been diagnosed in the postnatal period. With improvements in ultrasound technology and increasing use of MRI, CPA is now diagnosed with increasing accuracy in utero. This also allows for identification of concomitant anomalies, which greatly affects infant survival. In addition, antenatal diagnosis of CPA and associated anomalies allow for family counseling and planning of treatment for the perinatal period including early referral to a center with pediatric surgical services. Here, we present a case of CPA with preoperative diagnosis using ultrasound and MRI.

Focal nodular hyperplasia (FNH) is a benign, poorly understood hepatic tumor that is rare in children. Although there is no evidence for malignant degeneration, FNH can occur adjacent to a malignancy. Here, the case of a 4-year-old boy with a hepatic mass and history of stage IV neuroblastoma is presented. Initial imaging and core-needle biopsy were consistent with FNH. However, after left lateral segmentectomy, pathologic examination revealed a malignant tumor most consistent with small cell undifferentiated hepatoblastoma as well as 3 foci of FNH in the surrounding parenchyma.

Fetal urinary tract obstruction can result in renal dysplasia, oligohydramnios, and pulmonary hypoplasia. Various surgical approaches have been used to create a complete obstructive uropathy though with variable success particularly in female fetuses and significant mortality in both males and females. Our objective was to create an improved ovine obstructive uropathy model in both male and female fetuses during the second trimester to study lung hypoplasia and renal function. Ewes (n=11) at 90-100 days gestation (term=140-145 days) underwent hysterotomy. 64% singletons, 36% twins. One fetus per ewe underwent creation of obstructive uropathy (7 males and 4 females). In the male fetus, a paramedian incision is made below the umbilical cord and the urachus and distal urethra are ligated. Females require two incisions. One midline incision made at the pubic symphysis to ligate the proximal urethra and a second in the midline below the umbilical cord to ligate the urachus. Serial ultrasounds are performed and fetuses are sacrificed between 130-135 gestational days. Ultrasounds confirmed bladder distention, hydronephrosis, and fetal viability. Mortality occurred in 4/11 (36%) from fetal demise. Successful completion of the model at necropsy demonstrates a grossly dilated bladder and multi-cystic kidneys (Figure 1, Figure 2).Figure 2View Large Image Figure ViewerDownload (PPT) We demonstrate a successful complete obstructive uropathy model in male and female fetal lambs. Differing techniques are needed due to anatomic differences and allow for minimal fetal trauma. This refined ovine model has utility in studying potential fetal interventions in decreasing the mortality from renal dysplasia and pulmonary hypoplasia.

The CD8 coreceptor enhances T cell function by stabilizing the TCR/peptide/MHC complex and/or increasing T cell avidity via interactions with the intracellular kinases Lck and LAT. We previously reported a CD4(+) T cell (TIL 13831), which recognizes the tumor-associated Ag tyrosinase in the context of HLA-A2. To determine Whether CD8 independent tumor cell recognition is a property of the TCR, we used retroviral transduction to express the TIL 13831 TCR in the CD8(-) murine lymphoma, 58 alpha(-)/beta(-). Immunofluorescent staining of TCR-transduced cells with human TCR Vbeta subfamily-specific and mouse CD3-specific Abs confirmed surface expression of the transferred TCR and coexpression of mouse CD3. Transduced effector cells secreted significant amounts of IL-2 following Ag presentation by tyrosinase peptide-pulsed T2 cells as well as stimulation with HLA-A2(+) melanoma lines compared with T2 cells alone or HLA-A2(-) melanoma cells. Further analysis of TCR-transduced clones demonstrated a correlation between T cell avidity and cell surface expression of the TCR. Therefore, the TIL 13831 TCR has sufficient affinity to mediate recognition of the physiologic levels of Ag expressed by tumor cells in the absence of CD8 expression.