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Introduction The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection. Methods After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative. Results Eleven children's hospitals contributed 506 patients. Among 344 prenatally diagnosed lesions, the congenital pulmonary airway malformation volume ratio was measured in 49.1%, and fetal MRI was performed in 34.3%. One hundred thirty-four (26.7%) children had respiratory symptoms at birth. Fifty-eight (11.6%) underwent neonatal resection, 322 (64.1%) had surgery at 1–12 months, and 122 (24.3%) had operations after 12 months. The median age at resection was 6.7 months (interquartile range, 3.6–11.4). Among 230 elective lobectomies performed in asymptomatic patients, thoracoscopy was successfully utilized in 102 (44.3%), but there was substantial variation across centers. The most common lesions were congenital pulmonary airway malformation (n = 234, 47.3%) and intralobar bronchopulmonary sequestration (n = 106, 21.4%). Conclusion This multicenter cohort study on operative CLMs highlights marked disease heterogeneity and substantial practice variation in preoperative evaluation and operative management. Future registry studies are planned to help establish evidence-based guidelines to optimize the care of these patients. Level of evidence Level II.

The purpose of this study was to evaluate the clinical presentation and operative outcomes of patients with congenital lobar emphysema (CLE) within a large multicenter research consortium.After central reliance IRB-approval, a retrospective cohort study was performed on all operatively managed lung malformations at eleven participating children's hospitals (2009-2015).Fifty-three (10.5%) children with pathology-confirmed CLE were identified among 506 lung malformations. A lung mass was detected prenatally in 13 (24.5%) compared to 331 (73.1%) in non-CLE cases (p 0.0001). Thirty-two (60.4%) CLE patients presented with respiratory symptoms at birth compared to 102 (22.7%) in non-CLE (p 0.0001). The most common locations for CLE were the left upper (n = 24, 45.3%), right middle (n = 16, 30.2%), and right upper (n = 10, 18.9%) lobes. Eighteen (34.0%) had resection as neonates, 30 (56.6%) had surgery at 1-12 months of age, and five (9.4%) had resections after 12 months. Six (11.3%) underwent thoracoscopic excision. Median hospital length of stay was 5.0 days (interquartile range, 4.0-13.0).Among lung malformations, CLE is associated with several unique features, including a low prenatal detection rate, a predilection for the upper/middle lobes, and infrequent utilization of thoracoscopy. Although respiratory distress at birth is common, CLE often presents clinically in a delayed and more insidious fashion.Level III.

Amniotic fluid; Neonatal mortality; Exencephaly Líquido amniótico; Mortalidad neonatal; Exencefalia Líquid amniòtic; Mortalitat neonatal; Exencefàlia Exencephaly/anencephaly is one of the leading causes of neonatal mortality and the most extreme open neural tube defect with no current treatments and limited mechanistic understanding. We hypothesized that exencephaly leads to a local neurodegenerative process in the brain exposed to the amniotic fluid as well as diffuse degeneration in other encephalic areas and the spinal cord. To evaluate the consequences of in utero neural tissue exposure, brain and spinal cord samples from E17 exencephalic murine fetuses (maternal intraperitoneal administration of valproic acid at E8) were analyzed and compared to controls and saline-injected shams (n = 11/group). Expression of apoptosis and senescence genes (p53, p21, p16, Rbl2, Casp3, Casp9) was determined by qRT-PCR and protein expression analyzed by western blot. Apoptosis was measured by TUNEL assay and PI/AV flow cytometry. Valproic acid at E8 induced exencephaly in 22% of fetuses. At E17 the fetuses exhibited the characteristic absence of cranial bones. The brain structures from exencephalic fetuses demonstrated a loss of layers in cortical regions and a complete loss of structural organization in the olfactory bulb, hippocampus, dental gyrus and septal cortex. E17 fetuses had reduced expression of NeuN, GFAP and Oligodendrocytes in the brain with primed microglia. Intrinsic apoptotic activation (p53, Caspase9 and 3) was upregulated and active Caspase3 localized to the layer of brain exposed to the amniotic fluid. Senescence via p21-Rbl2 was increased in the brain and in the spinal cord at the lamina I-II of the somatosensory dorsal horn. The current study characterizes CNS alterations in murine exencephaly and demonstrates that degeneration due to intrinsic apoptosis and senescence occurs in the directly exposed brain but also remotely in the spinal cord. This work was supported by Prof. Jose L. Peiro internal Cincinnati Children's Hospital funding.

Background: The factors that contribute to success as a pediatric surgeon–scientist are not well defined. The purpose of this study is to define a group of NIH-funded pediatric surgeons, assess their academic productivity, and elucidate factors that have contributed to their success. Methods: Pediatric surgeons were queried in the NIH report database to determine NIH funding awarded. Academic productivity was then assessed. An online survey was then targeted to NIH-funded pediatric surgeons. Results: Since 1988, 83 pediatric surgeon–investigators have received major NIH funding. Currently, there are 37 pediatric surgeons with 43 NIH-sponsored awards. The mean h -index of this group of pediatric surgeons was 18±1.1, mean number of publications (since 2001) was 21±2.1, and both increase commensurate with academic rank. In response to the survey, 81% engaged in research during their surgical residency, and 48% were mentored by a pediatric surgeon–scientist. More than 60% of respondents had significant protected time and financial support. Factors felt to be most significant for academic success included mentorship, perseverance, and protected time. Conclusions: Mentorship, perseverance, institutional commitment to protected research time, and financial support are considered to be important to facilitate the successes of pediatric surgeon–scientists. These results will be useful to aspiring pediatric surgeon–scientists and departments wishing to develop a robust research program.

Objective The aim of this research was to compare the impact of varying degrees of visceral herniation on the growth rates of the contralateral and ipsilateral fetal lungs in cases of isolated left-sided congenital diaphragmatic hernia (CDH). Methods Data were retrieved from 58 fetuses with isolated left-sided CDH undergoing magnetic resonance imaging studies at both mid-gestation (20–30 weeks) and late-gestation (>30 weeks) time points. The growth of the right and left lungs (ΔLV-R and ΔLV-L) was calculated. The impact of the degree of visceral herniation on the growth disparity between the right and left lungs was then compared. Results Measurable growth occurred in both lungs between the mid-gestation and late-gestation time points in each group. The ΔLV-R exhibited a strong correlation with ΔLV-L. However, the right lung grew significantly faster than the left lung (ΔLV-R = 1.36 vs ΔLV-L = 0.17 mL/week, P

A previously healthy 3-week-old boy presented with 5 hours of marked fussiness, abdominal distention, and poor feeding. He was afebrile and well perfused. His examination was remarkable for localized abdominal tenderness and distention. He was referred to the emergency department in which an abdominal radiograph revealed gaseous distention of the bowel with a paucity of gas in the pelvis. Complete blood cell count and urinalysis were unremarkable. His ongoing fussiness and abnormal physical examination prompted consultation with surgery and radiology. Our combined efforts ultimately established an unexpected diagnosis.

Objective The aim of this study was to evaluate fetal lung growth rate for isolated left-sided congenital diaphragmatic hernia (CDH) using serial magnetic resonance imaging (MRI)-based volumetric measures. Methods Early and late gestational (22–30 and >30 weeks' gestation) lung volumetry was obtained by fetal MRI in 47 cases of isolated left-sided CDH. At both of these time points, lung volume indices, including total lung volume (TLV), observed to expected TLV (o/e TLV), and percentage of predicted lung volume (PPLV) as well as their change rates (Δ) and relative Δ during gestation were calculated and analyzed in regard to their capacity to predict neonatal survival. Results TLV, o/e TLV, and PPLV had various changes during gestation. Late TLV, early and late o/e TLV, and late PPLV were predictive of neonatal survival. Non-survivors had lower ΔTLV and more negative relative ΔPPLV than survivors (1.18 vs 1.85 mL/week, P = 0.004 and −4.15%/week vs −1.95%/week, P = 0.002, respectively). Conclusions The severity of pulmonary hypoplasia is dynamic and can worsen in the third trimester. MRI lung volumetry repeated in late gestation can provide additional information on individual lung growth that may facilitate prenatal counseling and focus perinatal management. © 2014 John Wiley & Sons, Ltd.

Objective The aim of this study was to determine whether fetal lung volume and visceral herniation are associated with changes in intrathoracic space in congenital diaphragmatic hernia(CDH). Methods We retrospectively examined the relationship between magnetic resonance imaging-derived measurements of intrathoracic space [predicted lung volume (PLV)] and residual lung volume or visceral herniation among isolated left-sided CDH fetuses. Results Data from fetal magnetic resonance imaging studies of 60 isolated left-sided CDH cases were analyzed. The median PLV of the CDH fetuses was found to be much greater than the expected total lung volume (eTLV) of a normal fetus at the same gestational age. Surprisingly, liver herniation and observed TLV(oTLV) were positively correlated with the PLV. Although the PPLV was consistently less than the o/eTLV, both indices were greater in survivors than in non-survivors, whereas no significant difference was seen in the PLV/eTLV ratio in regard to survivorship. Conclusion The intrathoracic domain available for lungs and viscera is expanded in CDH fetuses and positively affected by the lung volume and the presence of liver herniation, leading to the difference in the PPLV and o/eTLV. Future study of intrathoracic space as it relates to the growth of the lung and herniated viscera is needed to better characterize the relationship between these parameters. © 2014 John Wiley & Sons, Ltd.

Background Chronic wounds are characterized by a wound healing and neovascularization deficit. Strategies to increase neovascularization can significantly improve chronic wound healing. Insulin-like growth factor (IGF)-1 is reported to be a keratinocyte mitogen and is believed to induce angiogenesis via a vascular endothelial growth factor (VEGF)-dependent pathway. Using a novel ex vivo human dermal wound model and a diabetic-impaired wound healing murine model, we hypothesized that adenoviral overexpression of IGF-1 (Ad-IGF-1) will enhance wound healing and induce angiogenesis through a VEGF-dependent pathway. Methods Ex vivo: 6-mm full-thickness punch biopsies were obtained from normal human skin, and 3-mm full-thickness wounds were created at the center. Skin explants were maintained at air liquid interface. Db/db murine model: 8-mm full-thickness dorsal wounds in diabetic (db/db) mice were created. Treatment groups in both human ex vivo and in vivo db/db wound models include 1 × 108 particle forming units of Ad-IGF-1 or Ad-LacZ, and phosphate buffered saline (n = 4–5/group). Cytotoxicity (lactate dehydrogenase) was quantified at days 3, 5, and 7 for the human ex vivo wound model. Epithelial gap closure (hematoxylin and eosin; Trichrome), VEGF expression (enzyme-linked immunosorbent assay), and capillary density (CD 31 + CAPS/HPF) were analyzed at day 7. Results In the human ex vivo organ culture, the adenoviral vectors did not demonstrate any significant difference in cytotoxicity compared with phosphate buffered saline. Ad-IGF-1 overexpression significantly increases basal keratinocyte migration, with no significant effect on epithelial gap closure. There was a significant increase in capillary density in the Ad-IGF-1 wounds. However, there was no effect on VEGF levels in Ad-IGF-1 samples compared with controls. In db/db wounds, Ad-IGF-1 overexpression significantly improves epithelial gap closure and granulation tissue with a dense cellular infiltrate compared with controls. Ad-IGF-1 also increases capillary density, again with no significant difference in VEGF levels in the wounds compared with control treatments. Conclusions In two different models, our data demonstrate that adenoviral-mediated gene transfer of IGF-1 results in enhanced wound healing and induces angiogenesis via a VEGF-independent pathway. Understanding the underlying mechanisms of IGF-1 effects on angiogenesis may help produce novel therapeutics for chronic wounds or diseases characterized by a deficit in neovascularization.

Background The current research environment for academic surgeons demands that extramural funding be obtained. Financial support from the National Institutes of Health (NIH) is historically the gold standard for funding in the biomedical research community, with the R01 funding mechanism viewed as indicator of research independence. The NIH also supports a mentor-based career development mechanism (K-series awards) in order to support early-stage investigators. The goal of this study was to investigate the grants successfully awarded to pediatric surgeon-scientists and then determine the success of the K-series award recipients at achieving research independence. Methods In July 2012, all current members of the American Pediatric Surgery Association (APSA) were queried in the NIH database from 1988–2012 through the NIH Research Portfolio Online Reporting Tools. The following factors were analyzed: type of grant, institution, amount of funding, and funding institute or center. Results Among current APSA members, there have been 83 independent investigators receiving grants, representing 13% of the current APSA membership, with 171 independent grants funded through various mechanisms. Six percent currently have active NIH funding, with $7.2 million distributed in 2012. There have been 28 K-series grants awarded. Of the recipients of expired K08 awards, 39% recipients were subsequently awarded an R01 grant. A total of 63% of these K-awarded investigators transitioned to an independent NIH award mechanism. Conclusions Pediatric surgeon-scientists successfully compete for NIH funding. Our data suggest that although the K-series funding mechanism is not the only path to research independence, over half of the pediatric surgeons who receive a K-award are successful in the transition to independent investigator.

Gastroschisis is a congenital abdominal wall defect in which the intestines develop outside the abdomen and are exposed to amniotic fluid. When the defect is small, lymphatic, venous, and intestinal obstruction may occur and contribute to the formation of intestinal edema, atresia, ischemia, and a thick inflammatory peel. Treatment requires early coverage of abdominal contents either by primary closure or by the placement of temporary Silastic silo followed by abdominal wall closure. Currently, both traditional suture closure and the sutureless plastic closure are being employed to repair the gastroschisis defect. The goal of the current study is to evaluate plastic closure. We predict no difference will be found in clinical outcomes between plastic closure and traditional suture closure.A retrospective review of 80 patients treated between 2000 and 2009 was performed. Plastic closure was used in 52 (65%) and traditional suture closure in 28 (35%) babies. The surgical procedure was determined by surgeon preference. Of the 31(39%) babies who required silos, 15 (19%) were treated with plastic closure and 16 (20%) underwent traditional closure. We collected the following demographic data and clinical progression data. Using SAS 9.2 (SAS Institute Inc, Cary, NC), we conducted linear regression, logistic regression, and time to event models to compare the following outcomes: days on ventilator, days to start enteral feeds, days to reach goal enteral feeds, days on total parenteral nutrition, hospital charges, duration of stay, mortality, and complications.The mean duration of follow-up was 11.4 months. Patients spent an average of 6 days on the ventilator. There were 2 mortalities. A multivariate analysis demonstrated that no differences were found between the 2 closures with most of the outcomes; however, when compared with traditional suture closure, those babies treated with plastic closure spent 4 days fewer days on the ventilator (P.01). Those babies who underwent suture closure were more likely to have an infection or sepsis (odds ratio, 5.15; P.001). When the entire cohort was considered, no significant difference was found between plastic and suture closure in time to start feeds, time to reach goal feeds, time on parenteral nutrition, hospital charges, duration of stay, or complications. Ventral hernias were noted in 46 (58%) patients, 32 (62%) after plastic closure and 14 (50%) after suture closure (P = .32). Hernia repair was required in 16 (20%) patients, 11 (21%) after plastic closure, and 5 (18%) after traditional repair (P = .32). In the silo cohort, children treated with plastic closure required 7.5(P.01) fewer days to start enteral feeds than those treated with suture closure.Plastic closure of abdominal wall defects in gastroschisis is effective both as a primary procedure and after silo placement. A multivariate analysis shows plastic closure to be associated with fewer days of mechanical ventilation and less likelihood of developing infection or sepsis.

Background Clerkship directors (CDs) are key educators and active clinicians. In 2003, the Alliance for Clinical Education published standards for CD resources and responsibilities, but how reality compares is unknown. Methods Representatives from each core clinical disciplines' CD organizations created an electronic survey that CDs received in 2006–2007. Results More than 500 CDs responded, including 71 surgeons. Surgeons reported spending approximately 27% of professional time on education. Most have codirectors, so total CD effort approximates the greater than 50% Alliance for Clinical Education guidelines. No disciplines' CDs have more than one support staff as recommended. Surgeons have the least clinic time, but the most inpatient weeks and many publications. Surgery CD concerns are curricula and simulation; few believe being a CD impairs academic advancement and more than 95% believe it enhances work satisfaction. Conclusions Surgery CDs are clinically active and academically productive. Although few surgery CDs have the recommended support staff, more than 95% report being a CD enhances work satisfaction.

Background/Purpose Spinal muscular atrophy (SMA) in children leads to progressive muscle weakness, dysphagia, aspiration, and death. We hypothesized that early laparoscopic fundoplication and gastrostomy in infants with SMA type I could be performed safely perhaps leading to fewer aspiration events and improved nutritional status. Methods Children diagnosed with SMA type I from 2002 through 2005 were included (n = 12). All children underwent laparoscopic Nissen fundoplication with gastrostomy shortly after diagnosis. Postoperative respiratory management and discharge criteria were standardized. Results All patients were extubated immediately postoperatively. There were no significant complications. Average time to full feeding and inpatient length of stay were 42 ± 4.9 hours (range, 30-48 hours) and 78 ± 22.5 hours (range, 44-120 hours), respectively. Mean weight-for-length percentile was doubled at 1 year postoperatively ( P = .03). The number of respiratory-related hospitalizations in the cohort decreased by almost 50% in the ensuing 12 months after surgery, although this did not reach statistical significance in this small cohort ( P = .34). Conclusions Early laparoscopic fundoplication and gastrostomy is safe and is associated with improved nutritional status. A trend toward fewer significant long-term aspiration-related events was seen after fundoplication. To better assess the long-term benefits of performing an antireflux procedure in these high-risk patients, a larger prospective trial comparing current nutritional support practices is needed.

The development of hydrops fetalis and maternal mirror syndrome in the setting of fetal sacrococcygeal teratoma (SCT) carries a grave prognosis. Fetal surgery is not typically offered for hydropic fetuses beyond 26 weeks of gestational age. Few options exist for these families, and they are typically counseled to continue pregnancy until delivery is necessary for maternal indications or the fetus is 30 weeks old. Although a number of series report the survival of hydropic SCT infants delivered after 30 weeks of gestation, a paucity of information is available regarding the outcome of hydropic SCT infants delivered between 26 and 30 weeks of gestation. We now report successful postpartum resection and survival of a hydropic fetus delivered at 27 weeks for maternal mirror syndrome that may assist in counseling families in similar situations.

Fetal teratomas are the most common tumors diagnosed prenatally. The majority of these tumors are benign and cured by complete resection of the mass during the neonatal period. Prenatal diagnosis has improved the perinatal management of these lesions and especially for the teratomas that might benefit from fetal intervention. A comprehensive prenatal evaluation including conventional ultrasounds, Doppler, echocardiography and fetal MRI, is essential for an effective counseling and perinatal management. Antenatal counseling helps the parents to better understand the natural history, fetal intervention, and perinatal management of these tumors, which differ dramatically depending on their size and location. Fetal surgical debulking improves survival in cases of sacrococcygeal teratoma with cardiac decompensation. Additionally, the use of an EXIT procedure reduces the morbidity and mortality if a complicated delivery in cases of cervical and mediastinal teratomas. Here, we offer an overview of all fetal teratomas and their recommended management, with emphasis on in utero treatment options.

The creation of mixed hematopoietic chimerism has become an important clinical strategy for tolerance induction for cellular and organ transplantation, and for the treatment of numerous hematopoietic diseases. Clinical success has been limited however, by host immune response and by competition from host hematopoiesis. Recent data suggests that limited donor stem cell engraftment after minimally myeloablative hematopoietic stem cell (HSC) transplantation may in part be due to MHC associated microenvironmental mismatch resulting in a competitive disadvantage for donor HSC. A strategy to overcome this barrier to stable mixed hematopoietic chimerism would involve concurrent transplantation of a donor bone marrow microenvironment. To test this possibility, we set out to develop a method to tissue engineer a bone marrow microenvironment. One to two murine femurs were mechanically crushed to a fine suspension and were combined in vitro with various delivery vehicles. These constructs were transplanted into syngeneic animals in locations that are known to support transplantation of other tissues. Although bone formation was observed with several conditions, bone marrow formation was noted only within the small bowel mesentery when type I collagen was used as the delivery vehicle. No bone marrow formed when the vehicle was changed to polyglycolic acid or type IV collagen. We have demonstrated that the small bowel mesentery can support bone marrow formation under specific in vivo conditions. Future work will focus on strategies for transplantation of an engineered donor bone marrow environment to facilitate creation of allogeneic mixed hematopoietic chimerism.

Mesenchymal stem cells are multipotent cells that can be isolated from adult bone marrow and can be induced in vitro and in vivo to differentiate into a variety of mesenchymal tissues, including bone, cartilage, tendon, fat, bone marrow stroma, and muscle1,2. Despite their potential clinical utility for cellular and gene therapy, the fate of mesenchymal stem cells after systemic administration is mostly unknown. To address this, we transplanted a well-characterized human mesenchymal stem cell population3 into fetal sheep early in gestation, before and after the expected development of immunologic competence. In this xenogeneic system, human mesenchymal stem cells engrafted and persisted in multiple tissues for as long as 13 months after transplantation. Transplanted human cells underwent site-specific differentiation into chondrocytes, adipocytes, myocytes and cardiomyocytes, bone marrow stromal cells and thymic stroma. Unexpectedly, there was long-term engraftment even when cells were transplanted after the expected development of immunocompetence. Thus, mesenchymal stem cells maintain their multipotential capacity after transplantation, and seem to have unique immunologic characteristics that allow persistence in a xenogeneic environment. Our data support the possibility of the transplantability of mesenchymal stem cells and their potential utility in tissue engineering, and cellular and gene therapy applications.

Background/Purpose: Immune responses to both vector and transgene antigens have limited the efficacy of postnatal gene therapy. We hypothesize that the fetal period may offer immunologic and developmental advantages for successful gene therapy. In this study we examined the efficacy, persistence, and immunologic effects of recombinant adenovirus after intramuscular delivery into fetal mice. Methods: E1-deleted adenovirus (AdCMVlacZ) containing the β-galactosidase marker gene was used for injection. Fetal Balb/c mice (14 to 15 days' gestation) were injected with AdCMVlacZ in 10-μL volume in either the shoulder or hindlimb musculature. Animals were killed at 18 to 20 days' gestation and up to 4 months postnatally for analysis of transgene expression and adenoviral genome persistence. Results: Fetuses were injected with doses of AdCMVlacZ from 1 × 10 8 to 2 × 10 10 viral particles (n = 80). Optimal survival rate was 83% at 18 to 20 days' gestation and 55% at 4 weeks of age using a dose of 1 × 10 9 particles. Expression of β-galactosidasae at 18 to 20 days localized to multiple muscle groups surrounding the site of injection, as well as bone marrow stroma, liver, lung, and dorsal root ganglia. Persistent muscle and liver transgene expression was observed for as long as 16 and 8 weeks, respectively, after injection. The pattern of liver expression was confined to discrete foci of hepatocytes, which appeared to increase in size in older animals. No histological evidence of muscle or liver inflammation was observed at any time after injection. No neutralizing antibodies were observed postnatally. Conclusions: Our results confirm that gene therapy in the fetus may be advantageous. Distribution of vector in the fetus at the site of injection is clearly broader than in the adult setting. Furthermore, the absence of immune response and persistence of transgene expression suggests that fetal exposure to foreign transgene and vector antigens may induce tolerance. Although we have not proven genomic integration, the histological appearance of transgene expression in the liver supports this conclusion. By understanding the mechanisms that underlie persistent transgene expression, fetal gene therapy may become a feasible strategy for the treatment of fatal genetic diseases.

Aortopexy is the therapeutic modality of choice for severe primary tracheomalacia. The thoracoscopic approach has been used with good results in infants and toddlers, but little information exists on the use of aortopexy in older children. We present the case of a boy with a lifelong history of refractory, a steroid-dependent reactive airway disease, and who was found to have severe primary tracheomalacia. He subsequently underwent thoracoscopic aortopexy with immediate resolution of the tracheomalacia as demonstrated by serial bronchoscopy and long-term resolution of his clinical respiratory symptoms at 1 year.

Purpose The purpose of this study was to characterize the growth rate of sacrococcygeal teratomas (SCTs) and determine its relationship to adverse outcomes. Methods A retrospective review of all pathology-confirmed isolated SCT patients evaluated with at least two documented ultrasounds and followed through hospital discharge between 2005 and 2012 was conducted. SCT growth rate was calculated as the difference between tumor volumes on a late- and early-gestation ultrasound divided by the difference in time. Outcomes were death, high-output cardiac failure (HOCF), hydrops, and preterm delivery. Student's t -test, receiver operator characteristics, Fisher's Exact test, and Pearson's correlation were performed. Results Of the 28 study subjects, there were 3 in utero demises and 2 neonatal deaths. Significantly faster SCT growth rates were seen in all adverse outcomes, including death ( p p =0.005), and preterm delivery ( p =0.009). There was a significant association with adverse outcomes at >61cm 3 /week (AUC=0.87, p =0.001, LR=4.52). Furthermore, there was an even greater association with death at >165cm 3 /week (AUC=0.93, p =0.003, LR=18.42). Growth rate was directly correlated with the percent of solid tumor ( r =0.60, p =0.0008). Conclusion Faster SCT growth is associated with adverse outcomes. SCT growth rate determined by ultrasound is an effective prognostic indicator for adverse outcomes and easily applied to patient management.

In extreme cases of breast hypertrophy, amputation of the nipple-areolar complex and transplantation during reduction mammaplasty has been advocated to avoid nipple necrosis. We report our experience with 172 patients having inferior breast pedicle reduction without amputation of the nipple-areolar complex. Mean total weight of resected tissue was 1,946 g (548 to 5,100 g), with a mean nipple-areolar transposition of 10 cm (0.5 to 23 cm). Dividing patients into four groups by weight of resection, we compared complication rates. In this series, where nipple-areola amputation was avoided, there was a 99.6% survival rate of the nipple-areolar complex with 97.1% retention of nipple sensibility. Patients with extreme breast hypertrophy (3,000 g resected tissue) experienced no increase in complications when compared to smaller reductions. In most cases of gigantomastia, amputation of the nipple can be avoided using the inferior breast pedicle technique. Size of breast resection alone should not determine the fate of the nipple.

We present the case of an 8-year-old girl with acute onset of intermittent lower abdominal pain. The gray-scale US examination showed an enlarged right ovary with peripheral cysts, reflecting ovarian congestion and strongly suggesting the diagnosis of torsion. Normal arterial and venous flow, however, was found on Doppler US.To demonstrate the importance of gray-scale US findings despite the presence of blood flow found on Doppler US in salvaging a viable, torsed ovary.Despite the Doppler findings, a presumptive diagnosis of ovarian torsion was made.Surgery confirmed the presence of a torsed ovary, which was viable and appeared normal after detorsion.This case illustrates that the gray-scale US appearance of the ovary can be more reliable than Doppler US for the diagnosis of ovarian torsion.

Children represent a unique group of patients who are likely to greatly benefit from minimally invasive surgery (MIS). The promise of less postoperative pain, smaller scars, shorter hospital stays, and a faster return to school continues to drive growth in this area. The development of pediatric-specific techniques and documentation of improved outcomes form a critical gateway to widespread application of pediatric MIS. A brief perspective on current approaches to MIS for pediatric congenital and acquired disease is provided in this report. Technical departures from standardized adult MIS and the rationale for their modification are highlighted.

To the best of the authors' knowledge, this report represents the first description of a ventriculoperitoneal (VP) shunt that migrated into the chest cavity where it caused recurrent pneumonias. This 15-year-old boy with a history of hydrocephalus treated with VP shunt therapy as an infant presented with a 2-year history of chronic coughing and recurrent pneumonia. A high-resolution chest computed tomography scan revealed a right lower lobe infiltration and evidence of migration of the peritoneal shunt tubing through the diaphragm into the lung parenchyma. The catheter was pulled back into the peritoneal cavity via a simple abdominal incision. The patient's long-term outcome was excellent, and there was complete cessation of the pneumonia.

Background Intestinal malrotation in adulthood may present with a variety of chronic symptoms. Surgical intervention frequently leads to other complications in these patients. We hypothesized that the chronic nature of malrotation in adults could cause a delay in diagnosis and increased perioperative complications. Study Design All patients diagnosed with intestinal malrotation from July 2002 through July 2006 were included. IRB approval was obtained. Outcomes in patients less than 16 years of age were compared with outcomes from those older than 16. Presenting symptoms, initial diagnosis, results of imaging data, and time to diagnosis were evaluated. Surgical management, resulting complications, and rate of reoperation were analyzed. Results Twenty-four patients with intestinal malrotation were identified (age range, 10 days to 89 years old; 10 adults, 14 children). Seventy percent of adults experienced chronic symptoms for 6 months or more before the diagnosis of malrotation was made (children, 14%, p=0.017). No patients in the adult group were initially diagnosed with malrotation, although 57% of children were correctly diagnosed at the time of presentation of symptoms (p=0.006). Postoperative complications occurred in 60% of adults, but in only 29% of children, though this did not reach significance (p=0.211). Forty percent of adult patients required reoperation (p=0.020). Conclusions Intestinal malrotation in adults is often associated with a delay in diagnosis and increased morbidity. Enhanced awareness of this entity in adults may enhance patient counseling and improve therapeutic outcomes in these patients.

RESULTS: There were 76 piglets, of which 35 received supplemental PN with enteral feeding and 39 received supplemental oxygen. Incidence of NEC was higher in piglets receiving supplemental PN (91.4% vs 24.4% ; p

The treatment of congenital hematologic disorders before birth by in utero hematopoietic stem cell transplantation remains a challenging goal. Although success has been achieved in X-linked severe combined immunodeficiency, the approach has failed in all other disorders attempted thus far. In this review, we examine relevant experimental data from the perspective of an analysis of why failure has occurred. We will also attempt to pose the important questions that will need to be answered prior to further clinical attempts to treat most target disorders by this approach.

Background/Purpose: In utero hematopoietic stem cell transplantation (IUHSCTx) has been experimentally or clinically effective only in circumstances in which there is a survival advantage for donor cells. A survival advantage exists for normal muscle cells in muscular dystrophy. Because hematopoietic and mesenchymal stem cells may have the capacity to differentiate into muscle cells, the authors hypothesized that in utero bone marrow (BM) or fetal liver (FL) stem cell transplantation may be used to treat muscular dystrophy. Methods: Time-dated 14-day-gestation fetal muscular dystrophy mice ( mdx ) were injected intraperitoneally with 1 to 5 × 10 6 BM or FL cells per fetus from Rosa26 donor mice (transgenic for lacZ). Four weeks after birth, peripheral blood from the pups was analyzed for hematopoietic chimerism by using fluorescence-activated cell sorting (FACS) for the Ly-9.1 marker. Chimeric mice (6 BM and 2 FL recipients) were sacrificed at 12 to 14 months of age, muscles were stained with X-gal, and analyzed by 1- to 2-μm plastic sections. Polymerase chain reaction (PCR) for lacZ was performed in other organs to determine systemic engraftment. Results: At the time of death, all animals that were chimeric at 4 weeks continued to show hematopoietic chimerism of 0.2% to 9% by FACS. Engrafted donor cells were found in multiple sections from hindlimb skeletal muscles, diaphragms, and hearts from both BM and FL recipients. These cells had incorporated into the host muscles, and their morphology was consistent with myogenic differentiation. PCR of BM, liver, spleen, thymus, kidney, and lung for lacZ was positive in multiple animals. Conclusions: IUHSCTx leads to widespread engraftment of donor cells in multiple muscle compartments of hematopoietic chimeras. The advantage for normal myocytes offered in the mdx model allows engraftment and myogenic differentiation of transplanted BM or FL cells by morphology at a relatively higher frequency in muscle relative to other tissues, without the need for host conditioning. Because muscular dystrophy now can be detected early in gestation, such a strategy may offer a future alternative in the clinical treatment of this disease. J Pediatr Surg 37:1058-1064. Copyright 2002, Elsevier Science (USA). All rights reserved.

Purpose Posterior sagittal anorectoplasty is a well accepted procedure utilized for repair of congenital imperforate anus. This procedure allows acceptable function and quality of life in these unfortunate children. However, for obvious reasons it is seldom performed in the adult patient.