Bladder prolapse through a patent urachus presenting as an umbilical mass in the newborn: characteristic prenatal sonographic findings and the diagnostic benefit of postnatal cystography.

Bladder prolapse through a patent urachus is rare. We present a newborn with an unusual exophytic, erythematous umbilical mass. Voiding cystography readily demonstrated continuity of the bladder dome with the umbilical mass, confirming bladder prolapse through a patent urachus. The diagnosis of bladder prolapse was rapidly made in a second newborn with similar imaging and clinical findings and confirmed by cystography. We discuss the embryology of this condition including the association with a vesico-allantoic cyst in utero. Pre- and postnatal images are presented. The use of cystography in diagnosis is emphasized.

Is necrotizing enterocolitis the same disease in term and preterm infants?

INTRODUCTION: Necrotizing enterocolitis predominantly affects preterm (PT) infants. The paucity of data regarding the clinical course in term infants makes it difficult to predict outcomes and counsel families. To identify predisposing factors and gain a better understanding of the clinical course of NEC in term infants, we reviewed our experience with term infants and compared it to outcomes in PT infants. METHODS: We performed a 10 year retrospective review of all infants admitted to our NICU with Bell stage 2 NEC or greater. Infants < 37 weeks gestation were considered PT. Term and PT infant comorbidities, outcomes and intraoperative findings were compared. RESULTS: Fifteen (12%) of 125 infants were term. Compared to PT infants, term infants were more likely to have congenital heart disease (33% term vs. 10% PT, p = 0.02) and develop NEC sooner (4 days in term vs. 17 days in PT, p < 0.001) but were less likely to require operative intervention (20% term vs. 38% PT; p = 0.17). There was no significant difference in Bell stage, survival and development of intestinal failure. NEC totalis occurred exclusively in PT infants. CONCLUSIONS: NEC in term infants has unique clinical features that distinguishes it from NEC in PT infants.

Outcomes after early splenectomy for hematological disorders.

PURPOSE: Acute splenic sequestration crisis is a devastating complication of sickle cell disease that can require prophylactic splenectomy. Historically, splenectomy before 5 years of age was avoided because of fear of overwhelming postsplenectomy sepsis. Recently, splenectomy has been performed as early as 2 years of age, but the safety of this approach is unknown. This study compared outcomes of splenectomy performed in patients under 5 years of age with those 5 years of age and older. MATERIALS AND METHODS: A retrospective chart review of patients registered in a children's hospital hematology database was performed to examine intraoperative and postoperative outcomes after splenectomy. Statistical data analysis included Fisher's exact tests for categorical variables and the nonparametric median test for continuous variables. RESULTS: From 1997 to 2012, 30 sickle cell patients underwent splenectomy. At surgery, 18 of the 30 patients were under 5 years of age (Group 1), and 12 patients were 5 years of age or older (Group 2). Almost all procedures were laparoscopic. Both group had similar operative times, rates of conversion, and frequencies of complications. Both groups had similar lengths of follow-up (median, 62 months for Group 1 versus 63 months for Group 2). No portal vein thromboses or postsplenectomy sepsis events occurred in either group. CONCLUSIONS: In this study, there was no evidence that the incidence of complications was higher after splenectomy at a younger age. A large, multicenter study is needed to further evaluate the safety of this practice.

Bedside upper gastrointestinal series in critically ill low birth weight infants.

BACKGROUND: The upper gastrointestinal (UGI) series is the preferred method for the diagnosis of malrotation. A bedside UGI technique was developed at our institution for use in low birth weight, critically ill neonates to minimize the risks of transportation from the neonatal intensive care unit (NICU) such as hypothermia and dislodgement of support lines and tubes. OBJECTIVE: To determine the ability of a bedside UGI technique to identify the position of the duodenojejunal junction (DJJ) in low birth weight, critically ill infants in the NICU. MATERIALS AND METHODS: We retrospectively reviewed bedside UGI examinations performed in premature infants weighing less than 1,500 g from 2008 to 2013 and correlated the findings with clinical data, imaging studies and surgical findings. RESULTS: Of 27 patients identified (weight range: 633-1,495 g), 21 (78%) bedside UGI series were diagnostic. Twenty of 27 cases (74%) demonstrated normal intestinal rotation. One case demonstrated malrotation with midgut volvulus, which was confirmed at surgery. In six cases (22%), the position of the DJJ could not be accurately determined. No cases of malrotation with midgut volvulus were missed. None of the patients with normal bedside UGI studies was found to have malrotation based on clinical follow-up (mean: 20 months), surgical findings or further imaging. CONCLUSION: The bedside UGI is a useful technique to exclude malrotation in critically ill neonates and minimizes potential risks of transportation to the radiology suite. Pitfalls that may preclude a diagnostic examination include incorrect timing of radiographs, patient rotation, suboptimal enteric tube position and bowel distention. In cases of diagnostic uncertainty, a follow-up study should be performed.

Interloop fluid in intussusception: what is its significance?

BACKGROUND: Sonography has been used to predict pneumatic reduction outcome in children with intussusception. OBJECTIVE: To assess the prognostic significance of fluid between the intussusceptum and intussuscepiens with respect to reduction outcome, lead point or necrosis. MATERIALS AND METHODS: Sonograms of children with a discharge diagnosis of intussusception from four institutions were reviewed for interloop fluid and correlated with results of pneumatic reduction and surgical/pathological findings when available. Maximal dimension of interloop fluid on a transverse image and fluid complexity were evaluated. RESULTS: Of 166 cases, 36 (21.7%) had interloop fluid. Pneumatic reduction was successful in 21 (58.3%) with fluid and 113 (87.6%) without. The average largest fluid dimension was 8.7 mm (range 5 mm-19 mm, median 8 mm) in cases with successful reduction and 12.8 mm (range 4 mm-26 mm, median 12.5 mm) in unsuccessful reduction (p < 0.05). Fluid dimension equal to or greater than 9 mm correlated with failed reduction (p < 0.0001;odds ratio 13:1). In 36 cases with interloop fluid that required surgery, there were four lead points and three necrosis. In cases without fluid with surgical reduction, there was one lead point and one necrosis. Interloop fluid correlated with lead point (p < 0.04) or necrosis (p < 0.03). Its significance increased with larger amounts of fluid (p < 0.0001). Patient age/fluid complexity did not correlate with reduction outcome (p = 0.9). CONCLUSION: Interloop fluid was associated with increased failure of pneumatic reduction and increased likelihood of lead point or necrosis, particularly when the maximum dimension exceeded 9 mm.

Postoperative Hirschsprung's enterocolitis after minimally invasive Swenson's procedure.

BACKGROUND/PURPOSE: Our preferred minimally invasive technique of Swenson's procedure has evolved from laparoscopic (LapSwen) to Swenson's transanal pullthrough (SWAP). We studied the incidence of postoperative Hirschsprung's enterocolitis (HEC) over the past decade. METHODS AND MATERIALS: We retrospectively reviewed the charts of 52 children who had a primary Swenson's pullthrough procedure between 1995 and 2006. Two cohorts (25 LapSwen [1995-2000] and 27 SWAP [1998-2006]) were compared. RESULTS: Median ages of diagnosis were 13 days for LapSwen and 4 days for SWAP. Median age at surgery was 4.1 months for LapSwen and 3.3 months for SWAP. Postoperative HEC occurred in 12% (16% LapSwen, 7.4% SWAP). The incidence of Down's syndrome and preoperative HEC did not differ between the 2 cohorts. Three children with HEC were Clostridium difficile-positive. Long-term function in the 36 children older than 4 years was excellent in 22%, good in 50%, fair in 11% and poor in 17%. CONCLUSION: Our incidence of postoperative HEC is low, and we have seen a trend toward fewer cases after the SWAP procedure. Early diagnosis, preoperative rectal irrigations, and routine postoperative anal dilatation may be contributing to the elimination of HEC as a significant risk after surgery for Hirschsprung's disease.

Heterotaxia syndromes and their abdominal manifestations.

PURPOSE OF REVIEW: Children with heterotaxia often have abnormalities of intestinal rotation, some of which may predispose to midgut volvulus. This review summarizes the literature and proposes an individualized approach to the patient based on symptoms and findings on gastrointestinal imaging. RECENT FINDINGS: This report reviews the types of heterotaxia syndromes and the debate surrounding the optimal management of the associated intestinal rotational abnormalities. Recent publications suggest that not all rotational abnormalities predispose to volvulus and that the natural history of rotational abnormalities in asymptomatic patients with heterotaxia is relatively benign. On the basis of these findings, an algorithm for the management of intestinal rotational abnormalities is proposed. SUMMARY: Malrotation and midgut volvulus is a life-threatening complication. Children with heterotaxia and symptoms suggestive of a proximal intestinal obstruction require urgent investigation and surgical treatment. Asymptomatic children require close clinical follow-up and urgent investigation of gastrointestinal symptoms.

Effect of subspecialty training and volume on outcome after pediatric inguinal hernia repair.

BACKGROUND/PURPOSE: Inguinal hernia repair is the most common operation performed in children. The aim of this study was to determine if there are any differences in outcome when this procedure is performed by subspecialist pediatric surgeons when compared with general surgeons. METHODS: All pediatric inguinal hernias repaired in the province of Ontario between 1993 and 2000 were reviewed using a population-based database. Children with complex medical conditions or prematurity were excluded. Cases done by general surgeons were compared with those done by pediatric surgeons. The chi2 test was used for nominal data and the Student's t test was used for continuous variables. Probabilities were calculated based on a logistic regression model. RESULTS: Of 20,545 eligible hernia repairs, 50.3% were performed by pediatric surgeons and 49.7% were performed by general surgeons. Pediatric surgeons operated on 62.4% of children younger than 2 years, 51.8% of children aged 26 years, and 37% of children older than 7 years. Duration of operation, length of hospital stay, and incidence of early postoperative complications were similar among pediatric and general surgeons. The rate of recurrent inguinal hernia was higher in the general surgeon group compared with pediatric surgeons (1.10% vs 0.45%, P < .001). Among pediatric surgeons, the estimated risk of hernia recurrence was independent of surgical volume. There was a significant inverse correlation between surgeon volume and recurrence risk among general surgeons, with the highest volume general surgeons achieving recurrence rates similar to pediatric surgeons. CONCLUSIONS: Pediatric surgeons have a lower rate of recurrence after inguinal hernia repair in children. General surgeons with high volumes have similar outcomes to pediatric surgeons.

Errors and adverse outcomes on a surgical service: what is the role of residents?

PURPOSE: The extensive changeover in residents that occurs every July in university-affiliated hospitals has been postulated to result in impaired delivery of patient care as new house staff are less experienced and unfamiliar with hospital-specific systems (the "July phenomenon"). To assess the impact of this process on patient safety, we examined the incidence and sources of medical error and adverse outcomes on a pediatric general surgery service during the final month of an academic year and the first month of the subsequent academic year. MATERIALS AND METHODS: All admissions to two pediatric surgeons during June and July 2002 were prospectively followed. The attending surgeon, a surgical fellow, and a medical student reviewed in-patient care daily. Errors committed by doctors, nurses, and allied health workers were identified through daily patient encounters, nursing rounds, medical rounds, and chart audit. Adverse outcomes were evaluated based on type and contributing factors, including involvement of residents. To correct for variations in patient volume, the incidence of errors and adverse outcomes were expressed as a percentage of total patient days. RESULTS: The error rate was 46/643 patient days (7.1%) in June, and 58/776 patient days (7.5%) in July (P = 0.9). Resident error accounted for 52.2% of errors in June and 39.7% of errors in July (P = 0.28). There was no significant difference in the adverse outcome rates (5% versus 6.7%, P = 0.21) or incidence of error-related adverse outcomes (10.8% versus 22.4%, P = 0.2) between June and July. Most errors were made by the on-call resident. CONCLUSION: Resident changeover at the completion of an academic year did not result in an increased number of medical errors or adverse outcomes, indicating that effective systems are in place to prevent the "July phenomenon."

Induction of xenogeneic neonatal tolerance to transgenic human leukocyte antigen class I grafts.

BACKGROUND: The immune response against xenografts is vigorous and poorly controlled with conventional immunosuppressants. Therefore, success in xenotransplantation will depend on developing additional approaches such as induction of immunologic unresponsiveness or tolerance. Although classic protocols of neonatal tolerance induction in mice are very tolerogenic in many allogeneic models, they have generally failed in xenogeneic models. The purpose of these studies was to determine whether failure results from an intrinsic property of xenogenic major histocompatibility complex (MHC) molecules themselves or, instead, is caused by some limitation in species-specific molecular interactions distinct from the polymorphic domains of xenogenic MHC molecules. METHODS: Our approach was to test the ability of lymphoid cells from a transgenic (Tg) mouse donor expressing a xeno-MHC class I molecule encoding the polymorphic alpha1/alpha2 for human leukocyte antigen (HLA)-B7 to induce neonatal tolerance in non-Tg syngeneic C57BL/6 recipients. Because the donor and recipient strains are genetically identical (C57BL/6, H-2b) except for Tg human MHC HLA-B7, any species-specific molecular incompatibility in this mouse anti-human class I xeno-combination that could potentially interfere with induction of tolerance has been eliminated. RESULTS: Our results show that HLA-B7 Tg-, but not C57BL/6 syngeneic-, injected neonates were unresponsive as adults to HLA-B7-expressing target cells in vitro and specifically accepted HLA-B7-expressing Tg skin grafts. In addition, neonatal injection of donor cells resulted in peripheral chimerism. CONCLUSIONS: These experiments demonstrate that, as long as species-specific molecular interactions are maintained, recognition of the polymorphic domains of xenogeneic MHC does not represent a barrier to neonatal tolerance induction.

Transgenic expression in mouse lung reveals distinct biological roles for the adenovirus type 5 E1A 243- and 289-amino-acid proteins.

Little is known about the biological significance of human adenovirus type 5 (Ad5) E1A in vivo. However, Ad5 E1A is well defined in vitro and can be detected frequently in the lungs of patients with pulmonary disease. Transgenic expression of the Ad5 E1A gene targeted to the mouse lung reveals distinct biological effects caused by two Ad5 E1A products. Either of two Ad5 E1A proteins was preferentially expressed in vivo in the transgenic lungs. The preferential expression of the Ad5 E1A 243-amino-acid (aa) protein at a moderate level was associated with cellular hyperplasia, nodular lesions of proliferating lymphocyte-like cells, and a low level of p53-dependent apoptosis in the lungs of transgenic mice. In contrast, the preferential expression of the Ad5 E1A 289-aa protein at a moderate level resulted in a proapoptotic injury and an acute pulmonary proinflammation in the lungs of transgenic mice, mediated by multiple apoptotic pathways, as well as an enhancement of the host immune cell response. Expression of the Ad5 E1A 243-aa protein resulted in proliferation-stimulated p53 upregulation, while expression of the Ad5 E1A 289-aa protein led to DNA damage-induced p53 activation. These data suggest that the Ad5 E1A 243- and 289-aa proteins lead to distinct biological roles in vivo.