Journal of Pediatric Surgery RSS feed: Current Issue. The journal presents original contributions as well as a complete international abstracts section and other special departments to provide the most current source of information and references in pediatric surgery. The journal is based on the need to improve the surgical care of infants and children, not only through advances in physiology, pathology and surgical techniques, but also by attention to the unique emotional and physical needs of the young patienthttp://www.jpedsurg.org/?rss=yesElsevier Inc.en © 2010 Published by Elsevier Inc. Journal of Pediatric Surgery0022-3468452February 2010 © 2010 Published by Elsevier Inc. healthpermissions@elsevier.comhttp://www.jpedsurg.org/article/PIIS0022346809008628/abstract?rss=yesAbstract: This lecture updates pediatric surgeons on the state of the science of tissue engineering and regenerative medicine.Tissue engineering and regenerative medicine: from first principles to state of the artJoseph Vacanti10.1016/j.jpedsurg.2009.10.063Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Journal of Pediatric Surgery Lecture291294http://www.jpedsurg.org/article/PIIS0022346809008616/abstract?rss=yesAbstract: This lecture summarizes the elements of innovation and demonstrates how one can apply the principles every day to be an effective surgical innovator.Endosurgery of the future, today: lessons on how to bean innovatorThom E. Lobe10.1016/j.jpedsurg.2009.10.062Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Karl Storz Lecture295299http://www.jpedsurg.org/article/PIIS0022346809008781/abstract?rss=yesAbstract: Background/Purpose: Ongoing debate surrounds the future provision of general paediatric surgery. The aim of this study was to compare outcomes for childhood appendicitis managed in a district general hospital (DGH) and a regional paediatric surgical unit (RU).Methods: Data collected retrospectively for a 2-year period in a DGH were compared with data collected prospectively for 1 year in an RU, where appendicitis management is guided by a care pathway. Children aged 6 to 15 years were included.Results: Four hundred and two patients were included (DGH ,196; RU, 206). There were more cases of gangrenous/perforated appendicitis in the RU (P < .0001). In the DGH, fewer patients received preoperative antibiotics (P < .0001) or underwent preoperative pain scoring (P < .0001). When adjusted for case mix, the relative risk of complications for a child managed at the DGH was 1.76 (95% confidence interval, 1.44-2.16; P < .0001) and that of readmission was 1.76 (95% confidence interval, 1.43-2.16; P < .0001) when compared with the RU.Conclusions: Patients with appendicitis managed in the DGH had a higher risk of complications and readmission. However, this appears to be related to the use of a care pathway at the RU. Introduction of a care pathway in the DGH may improve outcomes and thus support the ongoing provision of general paediatric surgery.Comparison of childhood appendicitis management in the regional paediatric surgery unit and the district general hospitalHannah L. Collins, Sarah L. Almond, Ben Thompson, David Lacy, Martin Greaney, Colin T. Baillie, Simon E. Kenny10.1016/j.jpedsurg.2009.10.079Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Trainees Prize300302http://www.jpedsurg.org/article/PIIS002234680900863X/abstract?rss=yesAbstract: Introduction: Pediatric general surgeons volunteering internationally often work independently, some without prior assessment of the needs of those they wish to assist. Consequently, care may be inefficient, duplicated, or misdirected. A study was performed to assess whether a network for pediatric surgery volunteer work exists.Methods: A search of the Internet was performed to determine whether a pediatric surgery network exists. Worldwide pediatric surgery societies were identified and grouped by country according to income. Web sites for medical volunteer organizations were examined for links to a network of pediatric surgery volunteer work.Results: A search of the Internet revealed no pediatric surgery volunteer network. Ninety-seven pediatric surgery societies were identified. Fifty-one of the organizations were identified as residing in low- and middle-income countries. Searching 50 Web sites for these societies revealed no existing pediatric surgery network. Of 45 Web sites for volunteer medical work, 1 surgery networking Web site was identified. Only 4 pediatric general surgery international volunteer opportunities were cited on that Web site.Conclusions: This study demonstrated that no pediatric surgery volunteer network exists. By identifying pediatric surgery organizations in low- and middle-income countries, it is speculated that one might link the surgeons in these regions with those wishing to volunteer their services.Fragmented international volunteerism: need for a global pediatric surgery networkMarilyn W. Butler10.1016/j.jpedsurg.2009.10.064Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers303309http://www.jpedsurg.org/article/PIIS0022346809008689/abstract?rss=yesAbstract: Purpose: Seven metrics of metabolic derangement were evaluated as contributors to clinical decision support for operative intervention in infants with suspected necrotizing enterocolitis (NEC).Methods: Records of infants with suspected NEC without radiologic evidence of free air were queried for presence of 7 components of metabolic derangement (CMD), consisting of positive blood culture, acidosis, bandemia, thrombocytopenia, hyponatremia, hypotension, or neutropenia. Cases were stratified by clinical decision after each surgical evaluation as observation (OBS) or intervention (INT). Good outcome was defined as full enteric feeding by discharge and bad outcome as death or ongoing parenteral alimentation. Eleven infants undergoing operative intervention after an initial decision to observe were evaluated as matched pairs. Components of metabolic derangement/case and frequency of each CMD were determined for OBS and INT. Mann-Whitney U test was used to compare proportions of CMD in each group. Outcome was compared using χ2. Observation was then stratified by outcome to determine whether 3 or more metabolic derangements warranting operative intervention would have changed initial clinical decision. The 11 matched cases were similarly analyzed using Wilcoxon-matched pairs.Results: Between March 2005 and July 2008, 35 infants with NEC received 53 surgical evaluations. A median of 1 CMD/case was defined in 32 instances of OBS. Surgical intervention was carried out in 19 infants with a median of 3 CMD/case. Mann-Whitney U test indicated significant difference in the frequencies of each CMD component in OBS vs INT (P = .04). Good outcome was achieved in 75% of OBS and 63% of INT (non-significant, NS). Analysis of OBS by outcome demonstrated a median 1 CMD/case of 25 with good outcome and 3 CMD/case in infants with bad outcome. Frequency of CMD was significantly higher in infants with bad outcome (P = .02). Wilcoxon-matched pair analysis of the 11 infants with paired evaluations demonstrated a similar distribution and frequency of CMD.Conclusion: Progressive metabolic derangement of infants with NEC can be clinically tracked. The appearance of any 3 of these 7 metrics indicates timely operative intervention. Application of CMD trajectory to timing of surgical intervention may improve outcome and define the relationship between specific CMD and operative risk.Timing of surgical intervention in necrotizing enterocolitis can be determined by trajectory of metabolic derangementJ.J. Tepas, Renu Sharma, Cynthia L. Leaphart, Brian G. Celso, Pam Pieper, Veronica Esquivia-Lee10.1016/j.jpedsurg.2009.10.069Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers310314http://www.jpedsurg.org/article/PIIS0022346809008677/abstract?rss=yesAbstract: Aim: The aim of the study was to assess the impact of human immunodeficiency virus (HIV) exposure on survival and extent of disease in necrotizing enterocolitis (NEC).Patients and Methods: All patients with NEC requiring surgery between June 1998 and June 2008 were analyzed. Three groups were identified: those born to HIV-positive (HIV+) mothers, those born to HIV-negative (HIV−) mothers, and those with an unknown HIV status. Primary outcome measure was survival to discharge. Secondary outcome measure was extent of disease.Results: One hundred nine infants (mean gestational age, 31 weeks; birth weight, 1413 g) underwent surgery for NEC. Gestational age, birth weight, and day of presentation were similar in all 3 groups, showing no statistical difference. The HIV+ group consisted of 22 infants, of which 13 (59%) died and 2 (9%) had panintestinal necrosis. The HIV− group consisted of 48 infants, of which 11 (23%) died, with 3(6%) having panintestinal necrosis. The remaining group of HIV (unknown) consisted of 38 infants, of which 14 (37%) died, with 2 (5%) having panintestinal necrosis. The latter group was not included in the analysis; but comparing the HIV+ and HIV− groups, there was a statistically higher chance of death (odds ratio = 4.8, P = .05). There was no difference statistically in the extent of disease.Conclusion: Neonates with NEC born to HIV+ mothers have a higher mortalityEffect of maternal human immunodeficiency virus status on the outcome of neonates with necrotizing enterocolitisJonathan Saul Karpelowsky, Stefanie van Mil, Alp Numanoglu, Ernesto Leva, Alastair J.W. Millar10.1016/j.jpedsurg.2009.10.068Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers315318http://www.jpedsurg.org/article/PIIS0022346809008665/abstract?rss=yesAbstract: Introduction: Isolated intestinal perforation (IIP) and necrotizing enterocolitis (NEC) seem to be separate diseases, though their surgical treatment is similar. We compared the morbidity after surgery for IIP and NEC in preterm infants (birth weight <1500 g).Patients and Methods: Isolated intestinal perforation was defined as intestinal perforation with associated necrosis less than 5% of bowel length and NEC as necrosis of 5% or more of bowel length ± perforation. Outcome measures included time to full enteral feeds, incidence of total parenteral nutrition–associated cholestasis, and nutritional and neurologic status up to 60 months of age. Data are quoted as median (range).Main Results: Sixty-one surviving patients treated between 1986 and 2006 were retrospectively reviewed (IIP: n = 32 versus NEC: n = 29). Median % resection of small and large bowel in IIP and NEC was 2% (1%-22%) versus 12% (6%-32%), and 5% (2%-13%) versus 7% (2%-25%), respectively (P < .05 in both). There was no significant difference in gestational age, birth weight, incidence of cerebral hemorrhage, respiratory distress syndrome, or surgical technique (enterostomy or primary anastomosis). Median time to enteral feeds, incidence of total parenteral nutrition–associated cholestasis, length of ventilation, and ultimate nutritional and neurologic outcome did not differ statistically significantly between the groups.Conclusion: Surgery in NEC resulted in more extensive intestinal resection than in IIP. However, subsequent morbidity was similar in both groups.Morbidity after surgical treatment of isolated intestinal perforation and necrotizing enterocolitis is similar in preterm infants weighing less than 1500 gAntti Koivusalo, Mikko Pakarinen, Risto Rintala10.1016/j.jpedsurg.2009.10.067Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers319323http://www.jpedsurg.org/article/PIIS0022346809008653/abstract?rss=yesAbstract: Introduction: Proponents of peritoneal drainage (PD) hypothesize that it allows stabilization before laparotomy. We examined this hypothesis by comparing clinical status before and after either PD or primary laparotomy (LAP).Methods: In an ethically approved, international, prospective randomized controlled trial (2002-2006), extremely low birth weight (<1000 g) infants with pneumoperitoneum received primary PD (n = 35) or LAP (n = 34). Physiologic data were collected prospectively and organ failure scores calculated and compared between preprocedure and day 1 after procedure. Data, expressed as mean ± SD or median (range), were analyzed using appropriate statistical tests.Results: There was no postprocedure improvement in either PD or LAP group comparing heart rate (PD, P = 1.0; LAP, P = .6), blood pressure (PD, P = .6; LAP, P = .8), inotrope requirement (PD, P = .2; LAP, P = .3), or Arterial partial pressure of oxygen/fraction of inspired oxygen ratio (PD, P = .1; LAP, P = .5). Infants managed with PD had a worsening cardiovascular status (P = .05). There were no differences in total organ failure score in either group (PD, P = .5; LAP, P = 1). Only 4 infants survived with PD alone with no difference between preprocedure and postprocedure organ failure score (P = .4).Conclusions: Peritoneal drainage does not immediately improve clinical status in extremely low birth weight infants with bowel perforation. The use of PD as a stabilizing or temporizing measure is not supported by these results.Peritoneal drainage does not stabilize extremely low birth weight infants with perforated bowel: data from the NET TrialClare M. Rees, Simon Eaton, A. Kate Khoo, Edward M. Kiely, Agostino Pierro, Members of the NET Trial Group10.1016/j.jpedsurg.2009.10.066Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers324329http://www.jpedsurg.org/article/PIIS0022346809008707/abstract?rss=yesAbstract: Purpose: Graft-vs-host disease (GVHD) is a rare complication of transplantation of organs rich in immunocompetent cells. The goal of this study was to report the features of GVHD after small bowel transplantation (SBTx) in children.Methods: The study involved a retrospective review of patients undergoing SBTx between 1999 and 2009 who had GVHD.Results: Of 46 children receiving 52 intestinal grafts (2 liver-intestine and 3 multivisceral), 5 (10%) developed GVHD. Median age at transplant was 42 (19-204) months. Baseline immunosupression consisted of tacrolimus and steroids supplemented with thymoglobulin (n = 2) or basiliximab (n = 3) for induction. Median time between transplantation and GVHD was 47 (16-333) days. All patients had generalized rash, 2 had diarrhea, and 2 had respiratory symptoms. Other symptoms were glomerulonephritis (n = 1) and conjunctivitis (n = 1). Four developed severe hematologic disorders. The diagnosis was confirmed by skin biopsy in 4 patients and supported by chimerism studies in two. Colonoscopy and opthalmoscopic findings were also suggestive in one. Treatment consisted of steroids and decrease of tacrolimus, with partial response in four. Other immunosuppressants were used in refractory or recurrent cases. Three patients died within 4 months after diagnosis.Conclusion: Graft-vs-host disease is a devastating complication of SBTx, with high mortality probably associated with severe immunologic dysregulation.Graft-vs-host disease after small bowel transplantation in childrenAne M. Andres, Manuel Lopez Santamaría, Esther Ramos, Jesus Sarriá, Manuel Molina, Francisco Hernandez, Jose L. Encinas, Javier Larrauri, Gerardo Prieto, Juan Antonio Tovar10.1016/j.jpedsurg.2009.10.071Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers330336http://www.jpedsurg.org/article/PIIS0022346809008690/abstract?rss=yesAbstract: Purpose: This review provides a blueprint to deal with the diagnosis and management of recurrent tracheoesophageal fistulas.Methods: A retrospective review over 27 years found 26 recurrent tracheoesophageal fistulas. Descriptive statistical analyses were performed.Results: In this cohort of 26 patients, 18 had a leak after their primary operation; and 22 had respiratory symptoms leading to the discovery of the recurrent fistula. The diagnosis was made by contrast study in 24. The repairs entailed placing a catheter through the fistula; separating the trachea and esophagus using sharp dissection; and placing tissue, preferably pericardium, between the suture lines. Postoperative complications included 7 anastamotic leaks, 4 strictures, and 3 recurrent fistulas. Long-term follow-up (median of 84 months) showed that 21 took all of their nutrition by mouth, 3 were tube fed, and 2 required a combination. Of the 23 patients with growth chart data, 16 fell in the first quartile of the growth chart, whereas none fell between the 75th and 100th percentile.Conclusion: This series, the largest to date, describes characteristics of recurrent tracheoesophageal fistulas, including techniques to make the diagnosis and provide a secure closure of the fistula, and the long-term outcomes of these patients.The diagnosis and management of recurrent tracheoesophageal fistulasSteven W. Bruch, Ronald B. Hirschl, Arnold G. Coran10.1016/j.jpedsurg.2009.10.070Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers337340http://www.jpedsurg.org/article/PIIS0022346809008641/abstract?rss=yesAbstract: Introduction: Gastric pull-up has become the predominant technique for esophageal replacement because of allegedly deficient results of colon grafts. This retrospective study examines the long-term results in a large series of colonic interpositions.Patients and Method: One hundred six children (median, 2.9 years; range, 0.32-15 years) had their esophagus replaced between 1965 and 2008, of which 96 had colon grafts. Those survivors who were 18 years and older were contacted and, if willing, interviewed, examined, and had their functional ability rated using the Karnofsky index.Results: Ninety-six children had undergone a colon graft, and of these, 9 (9.3%) died. There were 65 long-term survivors whose indications for surgery included caustic injury (n = 32), failed tracheoesophageal fistula repair (n = 16), pure esophageal atresia (n = 14), and others (n = 3). The graft was either retrosternal (n = 49) or mediastinal (n = 16 patients). Twelve patients were unavailable for long-term assessment. After a median follow-up of 33.3 (11-41) years, 23 (43%) of 53 individuals experienced mild symptoms of reflux; scoliosis, 12 (22%) of 53, and/or other complications, 15 (27%) of 53 required further surgery.Thirty-two patients (60%) with Karnofsky indexes of 80% or higher felt healthy, 19 (36%) had mild life-style limitations (index, 40%-80%), and 2 had indexes less than 40%. Most patients live normal lives.Conclusions: Colon conduits restored gastrointestinal continuity with limited mortality and considerable morbidity but good functional outcome and health perception in the long-term. Our study suggests that colon grafts are no worse than gastric pull-ups in the long-term.Colonic interposition for esophageal replacement in children remains a good choice: 33-year median follow-up of 65 patientsLaura Burgos, Saturnino Barrena, Ane M. Andrés, Leopoldo Martínez, Francisco Hernández, Pedro Olivares, Luis Lassaletta, Juan A. Tovar10.1016/j.jpedsurg.2009.10.065Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers341345http://www.jpedsurg.org/article/PIIS0022346809008720/abstract?rss=yesAbstract: Introduction: The aim of this study was to compare outcomes after laparoscopic and open techniques for Nissen fundoplication and gastrostomy placement in the neonatal intensive care unit (NICU) population.Methods: The medical records for NICU inpatients who underwent laparoscopic and open Nissen fundoplication and gastrostomy placement from August 2002 to August 2008 were reviewed after Institutional Review Board approval. Each technique was compared with regard to operative time, estimated blood loss, postoperative 24-hour narcotic requirements, time to goal feeds, and complication rates. Analysis of variance was used to determine statistical significance. Data are quoted as mean ± SEM.Results: Fifty-seven NICU patients underwent fundoplication and gastrostomy placement (25 laparoscopic and 32 open). The time to goal feeds was significantly shorter for the laparoscopic group (4.3 ± 0.4 vs 6.1 ± 0.6 days, P = .04). The 24-hour postoperative narcotic requirement was significantly lower in the laparoscopic group (0.24 ± 0.05 vs 0.55 ± 0.08 mg/kg, P = .007). Operation times (111 ± 5 [open] vs 113 ± 5 minutes, P = .76) and estimated blood loss (13 ± 2 [open] vs 11 ± 1 mL, P = .33) were comparable for both groups.Conclusion: Laparoscopic and open techniques for Nissen fundoplication with gastrostomy placement are safe and appropriate treatment methods with equivalent operating times for the treatment of gastroesophageal reflux in the NICU population.A comparison of laparoscopic and open Nissen fundoplication and gastrostomy placement in the neonatal intensive care unit populationKeith A. Thatch, Edward Y. Yoo, L. Grier Arthur, Christine Finck, Douglas Katz, Matthew Moront, Rajeev Prasad, Charles Vinocur, Marshall Z. Schwartz10.1016/j.jpedsurg.2009.10.073Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers346349http://www.jpedsurg.org/article/PIIS0022346809008768/abstract?rss=yesAbstract: Purpose: Delayed gastric emptying (DGE) as indicated by preoperative gastric emptying scan (GES) is one rationale for performing a gastric emptying procedure (GEP) at time of fundoplication for gastroesophageal reflux disease (GERD). However, the role of GES and GEP in the surgical management of GERD remains unclear. We examined the use of preoperative GES in fundoplication patients.Methods: Retrospective chart review of patients undergoing fundoplication from 2000 to 2005 in a single institution including patient demographics, operative procedure, and postoperative outcomes at 1-year follow-up was analyzed using χ2 test.Results: Of 76 fundoplication patients, 39 (51%) had preoperative GES with 11 patients (28%) having DGE and 16 GEP performed. Developmentally delayed children were more likely to have GES. There were no significant differences in postoperative complications, length of hospital stay, or use of anti-GERD medications at 1 year between patients who had preoperative GES and those who did not. In the developmentally delayed group, there were no differences in outcomes between those with preoperative GES and those who did not.Conclusions: There were no differences in outcomes for GERD patients with or without preoperative GES or GEP postfundoplication. The use of GES in the management of GERD requires further evaluation.Gastric emptying scans: unnecessary preoperative testing for fundoplications?Marie-Chantal Struijs, David Lasko, Stig Somme, Priscilla Chiu10.1016/j.jpedsurg.2009.10.077Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers350354http://www.jpedsurg.org/article/PIIS0022346809008719/abstract?rss=yesAbstract: Introduction: Thoracoscopic repair of congenital diaphragmatic hernia (CDH) has been described, but its efficacy and safety have not been validated. The aim was to compare our experience of thoracoscopy with laparotomy repair.Methods: After ethics approval, we reviewed the notes of neonates with CDH operated in our institution between 2003 and 2008. Two historical groups were compared: infants who underwent laparotomy (2003-2008) or thoracoscopy (2007-2008). Data were compared by t test or Mann-Whitney tests.Results: Thirty-five children had open repair of CDH, and 13 had thoracoscopic repair. Groups were homogeneous for age and weight. Five (38%) neonates who had thoracoscopy were converted to open for surgical difficulties (n = 4) and O2 desaturation (n = 1). Patch repair was used in 12 (34%) open and 6 (46%) thoracoscopic repairs. End-tidal CO2 was significantly elevated during thoracoscopy, but this was not reflected in arterial CO2 or pH. There were 3 (8%) recurrences after open repair and 2 (25%) after thoracoscopy (P = .19).Conclusion: Thoracoscopic repair of CDH is feasible. Arterial blood gases should be closely monitored. Despite higher EtCO2, conversion to open was mainly because of difficult repair. A randomized trial is necessary to assess the effect of thoracoscopy on ventilation and recurrences.Thoracoscopic repair of congenital diaphragmatic hernia: intraoperative ventilation and recurrenceMerrill McHoney, Luca Giacomello, Shireen A. Nah, Paolo De Coppi, Edward M. Kiely, Joe I. Curry, David P. Drake, Simon Eaton, Agostino Pierro10.1016/j.jpedsurg.2009.10.072Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers355359http://www.jpedsurg.org/article/PIIS0022346809008756/abstract?rss=yesAbstract: Introduction: Pulmonary morbidity in survivors of congenital diaphragmatic hernia (CDH) is caused by hypoplasia, barotraumas, or other reasons. We have previously shown deficient tracheal innervation in rats with CDH. Now we examine whether bronchopulmonary innervation is also abnormal in both infants and rats with CDH.Material and Methods: Sections of E15, E18, and E21 rat lungs were immunostained for Protein gene product 9.5 and S100 antibodies. Similar immunostaining was performed on tissue from infants dying from CDH (n = 6) and other causes (n = 6) with Neurofilament, S100, and Rearranged during transfection antibodies. Nerve trunks/bronchus were counted, and the proportion of glial and RET-positive cells/bronchial surface was calculated. Glial cell-line derived neurotrophic factor protein and mRNA were measured in rat lungs.Results: Nerve trunks/bronchus were decreased in infants and rat fetuses with CDH. In contrast, glial and RET-positive cells/bronchial surface were increased in infants and rats with CDH. Both lungs were equally affected. GDNF protein was high, whereas GDNF mRNA was decreased in preterm animals with CDH.Conclusions: The lungs of infants and rats with CDH have decreased neural components compensated by increased supporting glial cells and persistence high expression of RET and GDNF protein. Because bronchopulmonary innervation controls airway smooth muscle, vessels, and glandular secretions, it is tempting to hypothesize that these deficiencies might play a role in respiratory morbidity in CDH.Bronchopulmonary innervation defects in infants and rats with congenital diaphragmatic herniaFederica Pederiva, Rosa Aras Lopez, Jose I. Rodriguez, Leopoldo Martinez, Juan A. Tovar10.1016/j.jpedsurg.2009.10.076Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers360365http://www.jpedsurg.org/article/PIIS0022346809008744/abstract?rss=yesAbstract: Purpose: The pathogenesis of pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) is not fully understood. The serine/threonine protein kinase B (AKT) plays important roles for lung morphogenesis through epithelial-mesenchymal interaction in phosphatidylinositide 3-kinase (PI3K)-dependent manner. It has been reported that the lung explant morphogenesis in mice is interfered by inhibitors of the PI3K-AKT pathway. We hypothesized that PI3K and AKT gene and protein expression/distribution are altered during epithelial morphogenesis in the nitrofen-induced hypoplastic lung.Methods: Pregnant rats were exposed to either olive oil or nitrofen on day 9 of gestation (D9). Fetal lungs were harvested on D15, D18, and D21 and divided into 3 groups as follows: control, nitrofen with CDH (CDH[−]), and nitrofen without CDH (CDH[+]) (n = 8 at each time-point, respectively). Reverse transcription polymerase chain reaction and immunohistochemistry were performed.Results: Messenger RNA expression levels of PI3K at D21 was significantly decreased in CDH(−) and CDH(+) group (5.71 ± 0.85 and 6.80 ± 0.88, respectively) compared to controls (8.95 ± 3.22; P < .05). Messenger RNA levels of AKT were also significantly decreased at D18 in CDH(−) and CDH(+) lungs (1.21 ± 0.16 and 1.20 ± 0.32, respectively) compared to controls (1.62 ± 0.14; P < .01). The PI3K immunoreactivity was diminished in the distal epithelium at D18 and decreased in the overall intensity at D21 in hypoplastic lungs compared to controls. The AKT immunoreactivity was decreased in mesenchyme at D18 and decreased overall intensity at D21 in CDH lungs compared to controls.Conclusion: Spatiotemporal alteration of pulmonary PI3K and AKT gene and protein expression during epithelial morphogenesis may interfere with epithelial-mesenchymal interaction, causing pulmonary hypoplasia in CDH by disrupting PI3K-AKT signaling pathway.Spatiotemporal alteration in phosphatidylinositide 3-kinase–serine/threonine protein kinase B signaling in the nitrofen-induced hypoplastic lungTakashi Doi, Elke Ruttenstock, Jens Dingemann, Prem Puri10.1016/j.jpedsurg.2009.10.075Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers366371http://www.jpedsurg.org/article/PIIS0022346809008732/abstract?rss=yesAbstract: Introduction: Isolated splenic abscesses (SAs) are rare in children. We report a single-center experience with emphasis on their diagnosis, etiology, treatment, and outcome.Methods: This is a retrospective review.Results: Eighteen children (age, 3-16 years; male-female ratio, 5:1) were managed over a period of 8 years in a tertiary-care institution. Presenting symptoms included fever, abdominal pain, and anorexia. Splenomegaly was present in 12 (67%), leukocytosis in 9 (50%), and thrombocytosis in 12 (67%) patients. Associated diseases were thalassemia (1), tuberculosis (1), and typhoid fever (9). Solitary and multiple SAs were seen in equal numbers. Blood culture grew Salmonella paratyphi A in 1 case. Splenic aspirate culture was positive in 3 (Escherichia coli [1], S paratyphi A [1], Acinetobacter [1]). Widal serology was positive in 9 (50%) patients. Management consisted of intravenous broad-spectrum antibiotic therapy in all patients, together with percutaneous aspiration in 10 (56%) cases where the abscess size was greater than 3 cm. All patients responded, and complete resolution was observed.Conclusion: Isolated SA in children responds favorably to conservative treatment with intravenous broad-spectrum antibiotics and percutaneous drainage without the need for splenectomy.Conservative management of isolated splenic abscess in childrenSubhasis Roy Choudhury, Pinaki Ranjan Debnath, Prashant Jain, Anand Singh Kushwaha, Archana Puri, Rajiv Chadha, Pitamber Sonker10.1016/j.jpedsurg.2009.10.074Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers372375http://www.jpedsurg.org/article/PIIS002234680900877X/abstract?rss=yesAbstract: Introduction: Choledochal cyst (CC) is closely associated with anomalous arrangement of the pancreaticobiliary duct, which is considered a high-risk factor for biliary tract malignancy. Early diagnosis and early treatment for CC could lead to a good prognosis. This study investigated late complications and long-term outcomes after surgery for CC.Patients and Methods: Fifty-six patients with CC and over 10 years of postoperative follow-up were analyzed retrospectively. All patients had undergone total resection of the extrahepatic bile duct and hepaticojejunostomy.Results: Six patients showed liver dysfunction manifested in the first 10 years after surgery, but all returned to normal thereafter. Dilatation of intrahepatic bile ducts persisted in 6 postoperatively, and in 3, this was still apparent more than 10 years after. Recurrent abdominal pain was encountered in 3, 1 had pancreas divisum with a pancreatic stone, and 1 had adhesive small bowel obstruction. Two patients developed biliary tract malignancy. A 14-year-old girl died of recurrent common bile duct cancer 2 years after the initial resection of CC with adenocarcinoma. A 26-year-old man with repeated cholangitis owing to multiple intrahepatic bile stones developed cholangiocarcinoma 26 years after the initial resection of CC. Event-free survival rate and overall survival rate were 89% (50/56) and 96% (54/56), respectively.Conclusions: Choledochal cyst generally has an excellent prognosis with early total resection and reconstruction. Long-term surveillance for the development of malignancy is still essential, especially if there is ongoing dilatation of the intrahepatic bile duct or biliary stones.Long-term outcomes after hepaticojejunostomy for choledochal cyst: a 10- to 27-year follow-upShigeru Ono, Shigehisa Fumino, Shinichi Shimadera, Naomi Iwai10.1016/j.jpedsurg.2009.10.078Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers376378http://www.jpedsurg.org/article/PIIS0022346809008823/abstract?rss=yesAbstract: Introduction: We routinely perform intraoperative endoscopy (IOE) of the intrahepatic bile duct (IHBD) and intrapancreatic bile duct (IPBD) with a pediatric cystoscope during excision of choledochal cyst (CC). Here we compare extended long-term follow-up patients with a series of recent cases.Methods: We compared 25 CC patients treated over the past 5 years (group 1) with 69 CC patients treated between 1986 and 1998 and followed up for more than 10 years (group 2), focusing on stone formation.Results: In group 1 (mean follow-up, 2.6 years) IOE identified IHBD debris in 7 (28%) of 25 and IPBD protein plugs in 9 (36%) of 25. In group 2 (mean follow-up, 14.6 years) IOE identified IHBD debris in 11 (16%) of 69 and IPBD protein plugs in 17 (25%) of 69. There was no relation between type of CC and incidence of IHBD debris or IPBD protein plugs. The incidence of postoperative stones to date is 0% in group 1 and 2.9% in group 2, rates far lower than those reported in the literature.Conclusions: We attribute our lower incidence of stones directly to IOE and recommend that it be performed routinely during cyst excision. It is simple, is effective, and improves outcome.Intraoperative endoscopy during choledochal cyst excision: extended long-term follow-up compared with recent casesTsubasa Takahashi, Akihiro Shimotakahara, Tadaharu Okazaki, Hiroyuki Koga, Go Miyano, Geoffrey J. Lane, Yoshifumi Kato, Atsuyuki Yamataka10.1016/j.jpedsurg.2009.10.083Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers379382http://www.jpedsurg.org/article/PIIS0022346809008811/abstract?rss=yesAbstract: Introduction: The aim is to identify the incidence of genetic mutations and outcome of children presenting with phaeochromocytoma/paraganglioma (PGL) to a single paediatric surgical service to determine the need for genetic counselling in associated kindreds.Methods: A retrospective case note review was undertaken of all cases treated between 1998 and 2008 with particular reference to presentation, management, and predisposing genetic conditions.Results: Seven cases (4 male, 3 female) were identified (median age, 13 years; interquartile range, 9-16). Three cases had a family history of phaeochromocytoma/PGL. All presented with neurologic symptoms related to hypertension, including headaches (n = 5), hemiparesis (n = 2), facial palsy, and hemianopia. All underwent surgical resection. Five patients had meta-iodobenzylguanidine (MIBG) therapy for apparently malignant features. All cases were found to have a predisposing genetic mutation: von Hippel-Lindau (n = 3), succinate dehydrogenase mutations (n = 3), and multiple endocrine neoplasia (n = 1). All patients are alive after a median follow-up of 5 (interquartile range, 2-7) years.Conclusions: All 7 cases had a familial genetic mutation identified, and none arose de novo. We advocate genetic counselling for all families of children diagnosed with phaeochromocytoma/PGL with lifelong surveillance tailored to the underlying syndrome because of the increased risk of synchronous and metachronous tumours associated with these genetic syndromes.Childhood phaeochromocytoma and paraganglioma: 100% incidence of genetic mutations and 100% survivalPhilip J. Hammond, Dermot Murphy, Robert Carachi, D. Fraser Davidson, Diana McIntosh10.1016/j.jpedsurg.2009.10.082Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers383386http://www.jpedsurg.org/article/PIIS002234680900880X/abstract?rss=yesAbstract: Purpose: The activation of the hedgehog (Hh) signaling is involved in the progression of various cancers. However, the correlation between the Hh signaling and tumorigenesis of pediatric malignancies has not been well documented. The present study was undertaken to examine the expression of the Hh signaling pathway in various pediatric tumors to elucidate the role of Hh signaling in pediatric malignancies.Methods: Surgical specimens were obtained from 68 patients with pediatric malignancies (neuroblastoma, 25; rhabdomyosarcoma, 18; hepatic tumor, 12; and renal tumor, 13). The expression of sonic hedgehog (Shh), its receptor Patched (Ptch), and downstream transcription factor Gli1 was evaluated using immunohistochemical staining.Results: In neuroblastoma, 96%, 100%, and 68%; in rhabdomyosarcoma, 78%, 100%, and 78%; in Wilms' tumor, 71%, 100%, and 43%; and in hepatoblastoma, 100%, 100%, and 73% of the specimens stained positive for Shh, Ptch, and Gli1, respectively. Differentiated neuroblastoma cells showed more intense Gli1 expression than in immature neuroblastoma cells. In rhabdomyosarcoma, the expression of Gli1 was higher in alveolar type than in embryonal type.Conclusions: These findings suggest that the Shh-Ptch1-Gli1 signaling pathways are frequently activated in most pediatric malignant tumors. The Hh signaling pathway may therefore play an important role in the differentiation and malignant potential of pediatric malignancies.Increased expression of the hedgehog signaling pathway in pediatric solid malignanciesTakaharu Oue, Akihiro Yoneda, Shuichiro Uehara, Hiroaki Yamanaka, Masahiro Fukuzawa10.1016/j.jpedsurg.2009.10.081Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers387392http://www.jpedsurg.org/article/PIIS0022346809008793/abstract?rss=yesAbstract: Introduction: Hirschsprung disease (HSCR) is associated with the later development of multiple endocrine neoplasia (MEN2), because RET gene variations are associated with both conditions. Specifically, HSCR-MEN2 cosegregation mostly relates to the cysteine-rich area at the RET-620 (the “Janus gene”).Aim: The aim of this study was to explore the clinical and genetic associations of HSCR-MEN2 in a cohort of HSCR patients.Methods: RET gene variation was evaluated by heteroduplex single-strand conformational polymorphism analysis and validated with automated sequencing techniques in HSCR patients (including 18 kindreds). Those with RET C620 variations were subjected to familial evaluation for coexisting HSCR-MEN2.Results: A cohort of 118 patients with HSCR (n = 89) or medullary thyroid carcinoma (n = 29) were studied, including 3 families where a RET-620 point mutation was identified. No C618, C609, or C611 variations were detected. In 1 remarkable 6-generational family (family 3), HSCR in early generations seemed to be later replaced by MEN2A. In the other 2 families with total colonic aganglionosis, a relative with a medullary thyroid carcinoma was identified.Conclusion: Gene mutation in the RET-620 position carries significant risk and may be part of a targeted investigation of high-risk areas in HSCR. We propose an alternative hypothesis of endoplasmic reticulum control to explain the changing phenotypic expression.Familial associations in medullary thyroid carcinoma with Hirschsprung disease: the role of the RET-C620 “Janus” genetic variationSam W. Moore, Monique Zaahl10.1016/j.jpedsurg.2009.10.080Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers393396http://www.jpedsurg.org/article/PIIS0022346809008847/abstract?rss=yesAbstract: Introduction: We report a novel technique to measure the length of the rectourethral fistula (RUF) in male patients with high-/intermediate-type imperforate anus during laparoscopically assisted anorectal pull-through (LAARP) to prevent incomplete excision.Method: During LAARP for RUF in 5 male patients (mean age, 4.6 months; prostatic in 3, bulbar in 2), the RUF was dissected carefully close to the urethra and opened; and a fine catheter with 10-mm calibrations was inserted by the laparoscopic surgeon until it was seen to emerge at or near the verumontanum by another surgeon performing cystoscopy. The laparoscopic surgeon then measured the distance from the point where dissection was ceased at the rectal end to the urethral orifice. The RUF was dissected free from the prostate for exactly this length, tied, and excised; and colon pull-through was performed to finish LAARP.Result: Rectourethral fistulae ranged from 5 to 15 mm and were much longer than expected. All dissections were uncomplicated without any injury to the urethra, and postoperative courses were unremarkable. At mean follow-up of 11 months, urination is normal in all without evidence of residual fistula.Conclusion: Knowing the exact length of the RUF facilitates safe and complete excision in an otherwise blind situation.Intraoperative measurement of rectourethral fistula: prevention of incomplete excision in male patients with high-/intermediate-type imperforate anusHiroyuki Koga, Yoshifumi Kato, Akihiro Shimotakahara, Go Miyano, Geoffrey J. Lane, Tadaharu Okazaki, Atsuyuki Yamataka10.1016/j.jpedsurg.2009.10.085Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers397400http://www.jpedsurg.org/article/PIIS0022346809009257/abstract?rss=yesAbstract: Purpose: The aim of this study was to evaluate the outcome of nonoperative vs operative management of blunt pancreatic trauma in children.Methods: Retrospective review of pancreatic injuries from 1995 to 2006 at an urban level I regional pediatric trauma center.Results: Forty-three children with pancreatic injury were included in the analysis. Injuries included grade I (n = 18), grade II (n = 6), grade III (n = 17), and grade IV (n = 2). For grade II to IV injuries, patients managed operatively (n = 14) and nonoperatively (n = 11) had similar lengths of stay and rates of readmission, despite increased pancreatic complications (PCs) in the nonoperative cohort (21% vs 73%; P = .02). There was a trend toward increased non-PCs in patients managed with resection (P = .07). Twelve patients underwent successful diagnostic endoscopic retrograde cholangiopancreatography in which duct injury was identified. In this group, nonoperative management was pursued in 6 patients but was associated with increased rates of PC (86% nonoperative vs 29% operative; P = .02).Conclusions: Operative management of children with grades II to IV pancreatic injury results in significantly decreased rates of PCs but fails to decrease length of stay in the hospital, possibly as a result of non-PCs. Endoscopic retrograde cholangiopancreatography may serve as a useful diagnostic modality for guiding operative vs nonoperative management decisions.Operative vs nonoperative management of blunt pancreatic trauma in childrenJames H. Wood, David A. Partrick, Jennifer L. Bruny, Angela Sauaia, Steven L. Moulton10.1016/j.jpedsurg.2009.10.095Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers401406http://www.jpedsurg.org/article/PIIS0022346809008835/abstract?rss=yesAbstract: Background/Purpose: Urinomas have been thought to protect renal function in boys with posterior urethral valves (PUVs), although recent reports have disputed this. This study tested the hypothesis that urinomas protect global renal function in boys with PUV.Methods: A retrospective analysis of all boys with PUV presenting to a tertiary unit derived from a region with an estimated population of 5.5 million was performed. Comparisons of the initial nadir creatinine, current creatinine, and renal status score (RSS) were made between those with and without urinomas. The RSS was derived from nephrology assessment of current renal status (0 = normal to 4 = end-stage renal failure or transplantation). Results were given as median (range), except for RSS, which was given as mean ± SEM. P ≤ .05 was regarded as significant.Results: During 1989-2009, 9 of 89 PUV boys were diagnosed with urinomas. Initial nadir creatinine was statistically lower in boys with urinomas (31 [18-44] vs 45 [20-574] μmol/L, P < .01). Length of follow-up was similar (5.1 [2.2-17.3] vs 5.9 [1.8-19.7] years, P = .59). Follow-up creatinine was significantly lower in urinoma boys (44 [25-77] vs 61 [29-1227] μmol/L, P < .05), as was the RSS (0.14 ± 0.14 vs 0.91 ± 0.14, P < .01). No urinoma boys progressed to end-stage renal failure or required transplant.Conclusion: This population-based study of PUV boys demonstrates that urinomas reduce nadir creatinine and significantly protect long-term global renal function.Urinomas protect renal function in posterior urethral valves—a population based studyJ.M. Wells, S. Mukerji, H. Chandran, K. Parashar, Liam McCarthy10.1016/j.jpedsurg.2009.10.084Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers407410http://www.jpedsurg.org/article/PIIS0022346809008859/abstract?rss=yesAbstract: Introduction: Testicular torsion may be an important cause of male infertility. We aimed to investigate the late hormonal function in patients with testicular ischemia/reperfusion injury of the testis after orchidectomy or detorsion.Methods: Twenty patients (mean age, 13.6 years) were prospectively evaluated at a mean of 5 years after testicular torsion. The serum follicle-stimulating hormone, luteinizing hormone (before and after gonadotropin-releasing hormone stimulation), testosterone, and inhibin B were measured. Fifteen age-matched adolescents without evidence of endocrine disease were used as controls for inhibin B values. Data are quoted as mean ± SEM.Results: Twelve patients were treated with detorsion and orchidopexy, and 8 underwent orchidectomy. Serum follicle-stimulating hormone, luteinizing hormone, and testosterone were all within the reference range. Inhibin B levels were significantly reduced in the 2 groups compared with the controls (34.5 ± 5.2 vs 63.9 ± 12.8 pg/mL, P = .02), but were not significantly different between the orchidectomy group and the group that underwent detorsion (41.3 ± 9.7 vs 30.4 ± 5.9 pg/mL, P = .41).Conclusion: Hormonal testicular function can be compromised after testicular torsion, although the type of surgery (orchidectomy or orchidopexy) does not seem to change the effect of this ischemia/reperfusion injury.Late hormonal function after testicular torsionCarmelo Romeo, Pietro Impellizzeri, Teresa Arrigo, Pietro Antonuccio, Mariella Valenzise, Silvio Mirabelli, Francesca Astra Borruto, Gianfranco Scalfari, Francesco Arena, Filippo De Luca10.1016/j.jpedsurg.2009.10.086Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers411413http://www.jpedsurg.org/article/PIIS0022346809008872/abstract?rss=yesAbstract: Background/Purpose: Inguinoscrotal testicular descent is controlled by androgens and the genitofemoral nerve, but the trigger for what makes the gubernaculum become a migratory organ like a limb bud remains unknown. Recent observations in the flutamide-treated rat suggested a link with the mammary line. We aimed, therefore, to reassess histologic anatomy in 2 different rodent models of androgen blockade, the testicular feminisation mouse (TFM) and the flutamide-treated rat.Methods: Neonatal TFM mice and fetal and neonatal rats after pretreatment of dams with an antiandrogen, flutamide (75 mg/kg; sunflower oil; days 16-19), were prepared for histologic analysis of the inguinal region and compared with fetal and neonatal controls.Results: Fetal control rats (E15.5 days) showed a mammary bud just outside the future inguinal canal adjacent to the gubernaculum. Neonatal TFM mice showed persistence of the inguinal breast bud supplied by the genitofemoral nerve. Flutamide-treated rats (D2) showed the gubernaculum surrounded by a persisting breast bud.Conclusions: The inguinal mammary line is adjacent to the gubernaculum in fetal rodents, and after androgen blockade, the gubernaculum becomes connected to the breast. The male mammary line, which is hidden in plain sight outside the inguinal canal, is made visible by androgen blockade. It may be the missing link in testicular descent, regulating gubernacular migration.Hidden in plain sight: the mammary line in males may be the missing link regulating inguinoscrotal testicular descentAdam Balic, Tamara Nation, Silverton Buraundi, Pamela Farmer, Don Newgreen, Bridget Southwell, John Hutson10.1016/j.jpedsurg.2009.10.088Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers414418http://www.jpedsurg.org/article/PIIS0022346809008884/abstract?rss=yesAbstract: Purpose: The insertion of a permanent central venous access device (PCVAD i.e. Broviac or Hickman lines and vascuports) is often considered a low priority and performed as an emergency. In 2004, a vascular access team (VAT), responsible for all PCVAD insertions, was established in our institution to address this.Methods: Data were collected retrospectively on all PCVAD insertions in 2 periods (January 2002-December 2003 and January 2005-December 2006). This included procedure, list type, surgeon grade, and operative time.Results: During 2002 to 2003 and 2005 to 2006, 465 and 569 PCVADs were inserted, respectively (22% increase). After introduction of the VAT, the percentage of lines inserted during emergency lists fell from 24% (n = 112) to 13% (n = 72), and out-of-hours fell from 6% (n = 29) to 3% (n = 17; P < .05), respectively. Median time taken for PCVAD insertion in 2005 to 2006 was significantly less if using an elective list compared with insertions performed on an emergency list (elective, 67 [56-82] minutes vs emergency, 85 (65-110) minutes; P < .05).Discussion: Introduction of a VAT has led to a significant decrease in emergency and out-of-hours PCVAD insertions, despite an increase in overall activity for the period. The median time taken for elective insertions is significantly less than emergency insertions, which has increased the efficiency of vascular access provision at our institution.A dedicated consultant-led vascular access team significantly reduces out-of-hours and emergency permanent central venous access insertionsJohn M. Wells, Wajid B. Jawaid, Peter Bromley, James Bennett, G. Surren Arul10.1016/j.jpedsurg.2009.10.089Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers419421http://www.jpedsurg.org/article/PIIS0022346809008860/abstract?rss=yesAbstract: This is a summary of the annual summer meeting that was a combination of two of the largest European paediatric surgical associations.Summary of the 56th BAPS and 10th EUPSA Congress: Graz, Austria 17th-20th June 2009Mark Davenport10.1016/j.jpedsurg.2009.10.087Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1BAPS Papers422423http://www.jpedsurg.org/article/PIIS0022346809009403/abstract?rss=yesAbstract: Herniation of the liver into the fluid-filled pericardial sac resulting in a thoracic mass is a particularly rare form of Morgagni hernia (congenital diaphragmatic hernia of Morgagni). We report an early antenatal diagnosis of congenital diaphragmatic hernia of Morgagni with pericardium effussion at 21 weeks' gestation. Two pericardiocentesis were performed at 21 and 22 4/7 weeks' gestation because of recurrence of pericardial effussion. Regular ultrasound assessments showed progressive herniation of the liver to practically fill the right hemithorax. An ex utero intrapartum treatment procedure was performed at 37 weeks' gestation to rescue maximum intrathoracic space for ventilation of the remaining functional lung tissue and to establish an airway for postnatal support. After birth, the patient successfully underwent early correction of the hernia. Postoperative course was uneventful, and the newborn girl was discharged 18 days later without complications and is currently doing well.Diagnosis and management of fetal intrapericardial Morgagni diaphragmatic hernia with massive pericardial effussionGuillermo Antiñolo, Juan Carlos De Agustin, Antonio Losada, Maria Luisa Marenco, Lutgardo Garcia-Diaz, Juan Morcillo10.1016/j.jpedsurg.2009.11.009Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Independent Case Reports424426http://www.jpedsurg.org/article/PIIS0022346809010458/abstract?rss=yesAbstract: Thoracoscopic total pneumonectomy has recently been described in the pediatric literature for the first time (Anselmo D, Perez I, Shaul D. Thoracoscopic Pneumonectomy for Severe Bronchiectasis in a 9-Year-Old Female. J Laparoendosc Adv Surg Tech 2008;18:775-777). In this article, we describe the use of thoracoscopic pneumonectomy in a 4-month-old boy with a complete (entire left lung) communicating bronchopulmonary foregut malformation.The patient is a 4-month-old boy who presented with persistent cough and low-grade fever. Chest radiograph was performed and showed a diffuse infiltrative pattern in the left lung. Computed tomography was then performed and revealed a poorly aerated abnormal left lung with absence of the left main stem bronchus. Subsequently, the patient underwent an upper gastrointestinal (UGI) series, which demonstrated that the left main stem bronchus arose from the distal esophagus and had no communication with the trachea. The patient tolerated a left thoracoscopic pneumonectomy very well. On postoperative day 5, a UGI demonstrated a question of a small contained leak. Therefore, a repeat study was performed 1 week later and did not demonstrate a leak. He was discharged in good health.Communicating bronchopulmonary foregut malformations are rare anomalies that require resection of the anomalous lung segment. This can be safely performed with a thoracoscopic technique even if it requires a pneumonectomy.Thoracoscopic pneumonectomy for communicating bronchopulmonary foregut malformation in a 4-month-old childRyan Katz, Roger Pitt, Daniel Kim, Brian Wingrove10.1016/j.jpedsurg.2009.11.045Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Independent Case Reports427429http://www.jpedsurg.org/article/PIIS0022346809010604/abstract?rss=yesAbstract: Sanjad-Sakati syndrome is a rare autosomal recessive disorder mainly occurring in the Arab Peninsula. This condition is associated with metabolic and septic complications starting in the neonatal period. Chronic intestinal pseudoobstruction owing to visceral myopathy is a rare disabling condition. We report a rare concurrence of Sanjad-Sakati syndrome and chronic intestinal pseudoobstruction in a Saudi child complicated by intestinal failure, sepsis, and early mortality.Visceral myopathy causing chronic intestinal pseudoobstruction and intestinal failure in a child with Sanjad-Sakati syndromeKamalesh Pal, Hissa Moammar, Dilip K. Mitra10.1016/j.jpedsurg.2009.12.004Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Independent Case Reports430434http://www.jpedsurg.org/article/PIIS0022346809010641/abstract?rss=yesAbstract: Congenital hernias arising in the subcostal region are rare. We describe a case of a former preterm infant, born with congenitally fused right 11th and 12th ribs and a protuberant mass in the right subcostal region. This mass was associated with a small fascial defect and herniation of abdominal contents. At operation, the mass was determined to be a hernia with an incarcerated ovarian remnant and fallopian tube.Congenital subcostal hernia with unusual contentsJulie Monteagudo, Kimberly A. Ruscher, Elizabeth Margolis, Fabiola Balarezo, Christine M. Finck10.1016/j.jpedsurg.2009.12.008Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Independent Case Reports435437http://www.jpedsurg.org/article/PIIS0022346809010628/abstract?rss=yesAbstract: Objective: The objective was to present a case series of pediatric patients presenting with small bowel obstruction secondary to both congenital and acquired internal mesocolic hernias, and the use of imaging technology in the management of this condition.Methods: A retrospective review of patients treated at the Yale–New Haven Children's Hospital for small bowel obstruction from 1998 to 2008 (n = 6) who presented with acute small bowel obstruction secondary to internal mesocolic hernias was performed.Results: We present 6 patients with small bowel obstruction caused by congenital (n = 4) and acquired (n = 2) mesocolic hernias after previous surgery. The median age at presentation was 13 years. Small bowel obstruction with a mesocolic hernia was identified by preoperative abdominal computerized tomography in 3 patients (50%) and at operation in the others. The mean length of stay was 6 days, with no recurrent episodes in the follow-up period.Conclusion: Small bowel obstruction secondary to mesocolic hernias, although rare, may be considered in the differential diagnosis of patients with history of malrotation or abdominal wall defects owing to their association with congenital mesenteric anomalies. This condition requires special attention from the clinician because of its catastrophic consequences. Imaging studies are an important asset because of the difficulty in making an accurate clinical diagnosis and the rarity of internal hernias.Congenital and acquired mesocolic hernias presenting with small bowel obstruction in childhood and adolescenceGustavo A. Villalona, Karen A. Diefenbach, Robert J. Touloukian10.1016/j.jpedsurg.2009.12.006Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Independent Case Reports438442http://www.jpedsurg.org/article/PIIS0022346809010719/abstract?rss=yesAbstract: Duplicate gallbladder is a rare congenital anomaly resulting from abnormalities in embryogenesis during the fifth and sixth weeks of gestation. Approximately 210 cases have been described. Variations include duplicate, triplicate, and septated gallbladder. We encountered a 15-year-old girl with both a duplicated gallbladder and a duplicated cystic duct who underwent successful laparoscopic cholecystectomy. This combination is extremely unusual, and based upon our findings in this case and a review of the literature, we propose the Unified Classification of Multiple Gallbladders.Gallbladder duplication: evaluation, treatment, and classificationMarlin Wayne Causey, Seth Miller, Colby A. Fernelius, Jeanette R. Burgess, Tommy A. Brown, Christopher Newton10.1016/j.jpedsurg.2009.12.015Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Independent Case Reports443446http://www.jpedsurg.org/article/PIIS0022346809007842/abstract?rss=yesAbstract: Angiogenesis has a key role for embryonic development and is crucial in several major diseases. Molecular basis of angiogenesis has been widely investigated (J Biochem Mol Biol. 2006;39:469-478, Oncogene. 2000;19:5598-5605). In this review, vascular endothelial growth factor (VEGF) and related receptors and their key roles in embryonic bladder development are discussed. The normal VEGF expression and related angiogenesis pattern of embryonic bladder are highlighted. The VEGF family especially VEGF-A is the major player in angiogenesis as well as many other angiogenic factors and activates 2 tyrosine kinase receptors, VEGFR-1 and VEGFR-2 (J Biochem Mol Biol. 2006;39:469-478). Besides its worthy role in angiogenesis, VEGF-A also seems to participate in normal bladder development (J Urol. 2007;177:1552-1557, Br J Urol Int. 2006;98:217-225). In previous studies, we have shown that exogenous VEGF or hypoxia-induced endogenous upregulation of this protein accelerates the growth of the bladder by detrusor and urothelium hypertrophy and hyperplasia (J Urol. 2007;177:1552-1557, Br J Urol Int. 2006;98:217-225, Dev Biol. 1997;183:139-149, Neurourol Urodyn. 2004;23:342-348). This abrupt role of VEGF on detrusor muscle through a hypoxic pathway may potentially be a part of the solution for many urologic conditions such as remodeling of detrusor muscle in antenatal bladder outlet obstruction.Vascular endothelial growth factor and bladder from a different perspective: not only an angiogenic factorBerk Burgu, Ozgu Aydogdu10.1016/j.jpedsurg.2009.10.002Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Review447452http://www.jpedsurg.org/article/PIIS0022346809008276/abstract?rss=yesAbstract: Proboscis lateralis is a rare craniofacial malformation characterized by absence of nasal cavity on one side with a trunk-like nasal appendage protruding from superomedial portion of the ipsilateral orbit. High-resolution computed tomography and magnetic resonance imaging are extremely useful in evaluating this congenital condition and the wide spectrum of associated anomalies occurring in the surrounding anatomical regions and brain. We present a case of proboscis lateralis in a 2-year-old girl with associated ipsilateral sinonasal aplasia, orbital cyst, absent olfactory bulb and olfactory tract. Absence of ipsilateral olfactory pathway in this rare disorder has been documented on high-resolution computed tomography and magnetic resonance imaging by us for the first time in English medical literature.Proboscis lateralis with ipsilateral sinonasal and olfactory pathway aplasiaSanjay Vaid, Darshan Shah, Sudarshan Rawat, Rahul Shukla10.1016/j.jpedsurg.2009.10.044Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Pediatric Surgical Image453456http://www.jpedsurg.org/article/PIIS002234680900712X/abstract?rss=yesAbstract: Tapping and aspirating abscesses or fluid collections in children can be difficult and even hazardous. A simple technique is described based on limiting the depth of penetration of the needle. This is achieved by removing the tubular plastic needle cover (or cap), cutting it to a predetermined length and replacing it. Thus, the cap acts as a “stopper.” In addition, this approach allows the operator to perform the task with only 1 hand on the syringe-needle unit.A simple technique to enhance the safety of abscess aspirationsMichael W.L. Gauderer10.1016/j.jpedsurg.2009.09.001Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Operative Technique457458http://www.jpedsurg.org/article/PIIS0022346809009452/abstract?rss=yes Gunnar Grotte, Emeritus Professor in Pediatric Surgery at Uppsala University, died on September 16, 2009, at the age of 89 years.Gunnar Grotte, MD, 1920-2009Leif Olsen10.1016/j.jpedsurg.2009.11.014Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Obituary459460http://www.jpedsurg.org/article/PIIS0022346809009221/abstract?rss=yesInfantile fibrosarcoma is a rare childhood mesenchymal malignant tumor usually presenting at birth or in the first year of life and affecting the extremities or axial region . Morphologically, it closely resembles its adult counterpart but differs by rarely metastasizing and usually having a good prognosis . Only 3 previous cases of gastrointestinal infantile fibrosarcoma have been documented; 2 associated with bowel perforation and meconium peritonitis . We report the first case of infantile fibrosarcoma occurring in the terminal ileum, diagnosed and operated on before bowel perforation or other complications.Infantile fibrosarcoma of the ileum presenting with congenital bowel obstructionMartin Louis van Niekerk, Willem A. Nel, Tomas Slavik10.1016/j.jpedsurg.2009.10.092Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Correspondence461462http://www.jpedsurg.org/article/PIIS0022346809009294/abstract?rss=yesWe read with interest the letter written by Drs Patel and Feins (J Pediatr Surg. 2008;43:1765) and also further publications regarding single-site laparoscopic gastrostomy insertion in children. The technique was first described by Drs Ponsky and Lukish in the Journal of Pediatric Surgery. This method of gastrostomy insertion has been used at our center in 12 children between 1 and 18 years of age, 6 of whom were neurologically impaired, with a weight range of 4.3 to 41 kg. One patient with adhesions, who had previously undergone Ladd procedure and duodenoduodenostomy, required an additional port to ensure safe gastrostomy placement. There were no intraperitoneal leaks or visceral injuries. Two cases developed mild leakage on to the skin around the button, which settled with a period of enteral rest or continuous feeds. One child developed postoperative pneumonia.Letter to the editor regarding single-site laparoscopic gastrostomy in childrenDylan Wanaguru, Philip Hammond, James Hamill10.1016/j.jpedsurg.2009.10.097Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Correspondence462463http://www.jpedsurg.org/article/PIIS0022346809009270/abstract?rss=yesIn response to the letter from Dr Wanaguru and his associates, I agree with each of their points. When we first described this procedure, we used an operative bronchoscope. This instrument did have poor visibility mostly due to the fact that half of the screen was obstructed by the grasper. Furthermore, because the bronchoscope was not perfectly round, it was difficult to maintain a tight seal in the trocar, which made it difficult to maintain pneumoperitoneum. The poor insufflation along with poor optics of the scope made it difficult to identify the optimal location on the stomach where the gastrostomy should be placed.Reply to Letter to the EditorTodd A. Ponsky10.1016/j.jpedsurg.2009.10.096Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Correspondence463463http://www.jpedsurg.org/article/PIIS0022346809009282/abstract?rss=yesThank you very much for your comments regarding our letter to Drs Ponsky and Lukish dealing with their article in the Journal of Pediatric Surgery (2008) 43, 412-414. The article described single-site laparoscopic gastrostomy.ReplyNeil Feins10.1016/j.jpedsurg.2009.11.001Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Correspondence463464http://www.jpedsurg.org/article/PIIS0022346809010240/abstract?rss=yesThe recent historical review by Dr Raffensperger about Pierre Frédet and pyloromyotomy speculates about the naming of the procedure . It is suspected that in retrospect, Frédet (of France) may not have obtained the deserved credit that was received by Ramstedt (of Germany) due to Franco-German antagonisms at the time. Also, it is possible that Americans may have adopted the name Ramstedt for the procedure because of a temporary preference for the German medical system in that era.Letter to the EditorFelix Schier10.1016/j.jpedsurg.2009.11.024Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Correspondence464464http://www.jpedsurg.org/article/PIIS0022346809010239/abstract?rss=yesWe are indebted to Professor Schier for his additional information concerning pyloromyotomy. I, too, was puzzled by the ambivalence of Fredet about his procedure and continued use of gastroenterostomy in cases of large tumors. From 1913 until 1920, he wrote mostly about traumatic surgery and did not address pyloric stenosis until 1921. By 1927, he had adopted the Ramstedt operation. His own French colleagues chided him for neglect of his original operation. He, like many other surgeons, was reluctant to leave the mucosa exposed when they could not suture the pyloric muscle transversely. Ramstedt certainly deserves tremendous recognition for his operation. Perhaps Professor Schier could organize an international meeting to celebrate the 100 years since the operation by Ramstedt on August 23, 2011. The meeting could be held in Munster.Reply to Letter to the EditorJohn Raffensperger10.1016/j.jpedsurg.2009.11.023Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Correspondence464464http://www.jpedsurg.org/article/PIIS0022346809007040/abstract?rss=yesAcknowledgment10.1016/j.jpedsurg.2009.08.025Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Acknowledgment of Reviewers465471http://www.jpedsurg.org/article/PIIS0022346810000515/abstract?rss=yesAnnouncements of Future Meetings10.1016/S0022-3468(10)00051-5Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Acknowledgment of Reviewers472473http://www.jpedsurg.org/article/PIIS0022346809009269/abstract?rss=yesAbstract: Colonic agenesis is a rare cause of congenital bowel obstruction and is usually associated with other malformations such as abdominal wall defects or gastrointestinal, cardiac, urogenital, and musculoskeletal lesions. Although many uncommon types have been reported, ileovesical fistula (IVF) associated with total colonic agenesis (TCA) has not been reported in a newborn with anorectal malformation (ARM). This is the first report of a 1-day-old newborn with ARM, IVF, and TCA.Colon agenesis with ileovesical fistula and anorectal malformationMüslim Yurtçu, H. Hasan Esen, Engin Günel10.1016/j.jpedsurg.2009.09.056Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Journal of Pediatric Surgery Electronic Pages (Available only online at www.jpedsurg.org)e1e3http://www.jpedsurg.org/article/PIIS0022346809009786/abstract?rss=yesAbstract: Fanconi anemia (FA) is an autosomal recessive inherited syndrome characterized by congenital abnormalities, aplastic anemia, and a high likelihood of developing cancer. We describe a child who presented with 2 synchronous solid tumors (Wilms tumor and neuroblastoma), later found to have FA, who developed severe toxicity and died after a first cycle of chemotherapy. Our experience emphasizes that a predisposing genetic condition should be sought in cases of multiple tumors and that managing FA patients with cancer can be particularly difficult.Multiple synchronous tumors in a child with Fanconi anemiaAlessia Compostella, Tiziana Toffolutti, Pietro Soloni, Patrizia Dall'Igna, Modesto Carli, Gianni Bisogno10.1016/j.jpedsurg.2009.11.015Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Journal of Pediatric Surgery Electronic Pages (Available only online at www.jpedsurg.org)e5e8http://www.jpedsurg.org/article/PIIS0022346809009439/abstract?rss=yesAbstract: Hypertrophic pyloric stenosis (HPS) is the most common cause of gastric outlet obstruction in infants, with an incidence of 1.5 to 3 per 1000 live births, and classically presents at 3 to 4 weeks of age. Delayed presentation of HPS is an extremely rare occurrence after early infancy. With the exclusion of congenital HPS, gastric outlet obstruction in childhood is a rare condition (1:100,000). We report a case of delayed presentation of HPS in a 4 1/2–year-old child in whom the pylorus was hypertrophied and appeared like an “olive,” and for which pyloromyotomy was curative.Delayed presentation of hypertrophic pyloric stenosis: a rare caseSantosh Mahalik, Alpana Prasad, Aparna Sinha, Rajeev Kulshrestha10.1016/j.jpedsurg.2009.11.012Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Journal of Pediatric Surgery Electronic Pages (Available only online at www.jpedsurg.org)e9e11http://www.jpedsurg.org/article/PIIS0022346809009816/abstract?rss=yesAbstract: Hepatic alveolar echinococcosis (AE) is an endemic disease in certain parts of the world and relatively rare in children. This report describes a 9-year-old girl with hepatic AE invading the diaphragm and directly spreading to the chest and abdominal wall. She was treated by surgical extirpation and albendazole therapy. In advanced hepatic AE, combined therapy of surgical and medical intervention is thought to improve the prognosis.Pediatric alveolar echinococcosis invading the diaphragm and spreading to the chest and abdominal wallShohei Honda, Tadao Okada, Fumiaki Sasaki, Satsuki Naito, Naoki Sato, Toshiya Kamiyama, Tomoo Itoh, Kanako Chikae Kubota, Satoru Todo10.1016/j.jpedsurg.2009.11.018Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Journal of Pediatric Surgery Electronic Pages (Available only online at www.jpedsurg.org)e13e16http://www.jpedsurg.org/article/PIIS002234680901063X/abstract?rss=yesAbstract: Phyllodes tumor is uncommon in children, and the malignant form is rare. There have only been a few cases of benign phyllodes tumor reported in peripubertal children and only one malignant case. We report the second case of malignant phyllodes tumor in a premenarchal 11-year-old girl.Malignant phyllodes tumor in an 11-year-old premenarchal girlPaolo G. Sorelli, Daniel Thomas, Ashley Moore, Mihir Khan, Happy Hoque10.1016/j.jpedsurg.2009.12.007Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Journal of Pediatric Surgery Electronic Pages (Available only online at www.jpedsurg.org)e17e20http://www.jpedsurg.org/article/PIIS0022346809010689/abstract?rss=yesAbstract: Polyorchidism is a rare congenital anomaly. A review of literature has yielded about 100 cases. We describe a rarer case of polyorchidism consisting of 3 testes on left hemiscrotum and one normal testis in right hemiscrotum. Diagnosis and management are discussed.Three small testes in left hemiscrotum: a rarer caseof polyorchidismPaolo Repetto, Pierluca Ceccarelli, Anastasia Bianchini, Viviana Durante, Diego Biondini, Alfredo Cacciari10.1016/j.jpedsurg.2009.12.012Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Journal of Pediatric Surgery Electronic Pages (Available only online at www.jpedsurg.org)e21e23http://www.jpedsurg.org/article/PIIS0022346809009865/abstract?rss=yesAbstract: Congenital cystic adenomatoid malformation, a rare developmental, hamartomatous abnormality of the lung, usually is unilateral, is localized, and presents in early infancy. Delayed occurrence in older children and multilobar involvement are rare. We describe a case of congenital cystic adenomatoid malformation type 1 with multilobar involvement, associated emphysema, and coexistent tracheobronchopathia osteochondroplastica in an adolescent girl for whom the correct diagnosis was achieved only on histologic examination. The importance of an accurate diagnosis of this entity enables proper subtyping, management to minimize the risk of infections and malignancy, and exclusion of associated malformations.Congenital cystic adenomatoid malformation of lung type 1Alka Mary Mathai, Hema Kini, Muktha R. Pai, Ashok B. Shetty, Nirupama Murali10.1016/j.jpedsurg.2009.11.022Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1Journal of Pediatric Surgery Electronic Pages (Available only online at www.jpedsurg.org)e25e28http://www.jpedsurg.org/article/PIIS0022346810000308/abstract?rss=yesContents10.1016/S0022-3468(10)00030-8Journal of Pediatric Surgery 45, 2 (2010)2010-02-01Journal of Pediatric Surgery2010-02-01452S0022-3468(10)X0002-1FrontmatterA7A10