Current Management of Congenital Pulmonary Airway Malformations: A "European Pediatric Surgeons' Association" Survey.

AIM: To define current management of congenital pulmonary airway malformation (CPAM). METHODS: A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire. MAIN RESULTS: Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%).Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively. CONCLUSION: Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.

Difficulties, guidelines and review of developing an acute rejection model after rat intestinal transplantation.

Experimental small bowel transplantation (SBT) in rats has been proven to be a useful tool for the study of ischemia-reperfusion and immunological aspects related to solid organ transplantation. However, the model is not completely refined, specialized literature is scarce and complex technical details are typically omitted or confusing. Most studies related to acute rejection (AR) use the orthotopic standard, with small sample sizes due to its high mortality, whereas those studying chronic rejection (CR) use the heterotopic standard, which allows longer term survival but does not exactly reflect the human clinical scenario. Various animal strains have been used, and the type of rejection and the timing of its analysis differ among authors. The double purpose of this study was to develop an improved unusual AR model of SBT using the heterotopic technique, and to elaborate a guide useful to implement experimental models for studying AR. We analyzed the model's technical details and expected difficulties in overcoming the learning curve for such a complex microsurgical model, identifying the potential problem areas and providing a step-by-step protocol and reference guide for future surgeons interested in the topic. We also discuss the historic and more recent options in the literature.

Portal Cavernoma in the Era of Mesoportal Shunt (Rex) and Liver Transplant in Children.

OBJECTIVE: The mesoportal shunt (MPS) and liver transplantation (LT) have changed the scenario of extrahepatic portal vein obstruction (EHPVO) since the MPS, the only "curative" technique, can now be offered in asymptomatic patients and also thrombotic complications of LT have increased the incidence of EHPVO. MATERIAL AND METHODS: A retrospective study of patients undergoing surgery for EHPVO was conducted between 1990 and 2015. An analysis was done for the shunt permeability and clinical evolution over time. RESULTS: Of the 73 children with EHPVO, 39 were operated (12 posttransplant and 27 idiopathic). The median age at surgery was 9.36 years (range, 1.60-17.42 years). The MPS was the technique of choice; it was offered in 21 patients but eventually could be performed in only 18 (9 posttransplant and 9 idiopathic). The results of MPS were better in idiopathic (just one thrombosis successfully converted into mesocaval bypass). In the MPS after LT (n = 9), six shunts are permeable, two became thrombotic (one patient requiring retransplantation), and one late thrombosis occurred and the patient died eventually because of gastrointestinal bleeding. In the remaining patients (21 out of the 39), MPS was not performed because of intrahepatic cavernoma, portal vein hypoplasia, or insufficient length of vascular graft (especially in left lateral segment graft LT, since the portal vein is usually located in the right upper quadrant). They underwent 10 distal splenorenal shunts (DSRS) (1 posttransplant and 9 idiopathic), 5 proximal splenorenal shunts (PSRS) (1/4), 6 mesocaval shunts (1/5), and 1 modified Sugiura procedure. The results with DSRS have been favorable (one thrombosis, converted into mesocaval bypass). In the PSRS no thrombosis was identified and in the mesocaval shunt one early thrombosis occurred. Posttransplantation group had higher risk of shunt thrombosis, regardless of the surgical technique (chi-square, 0.021). The total increase of platelets after 6 months was not different in MPS as compared with other surgical techniques (analysis of variance, 0.110). CONCLUSIONS: The MPS is the technique of choice in EHPVO for idiopathic thrombosis as well as secondary to LT. Not all cases are favorable for MPS, so the surgeon must consider the possibility of alternative techniques for EHPVO. The results in terms of shunt patency are much better in idiopathic cavernoma that posttransplant patients.

Congenital Portosystemic Shunts: Clinic Heterogeneity Requires an Individual Management of the Patient.

INTRODUCTION: Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition. MATERIAL AND METHODS: A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted. Description of the morphology, clinical impact, and treatment is given. RESULTS: According to the imaging techniques (IT), the shunt was apparently intrahepatic in 14 patients, extrahepatic in 10 patients, and mixed in 1 patient. In 14 children, IT showed hepatic portal circulation. In total shunts in which radiological examination was performed, invasive radiological techniques were able to demonstrate intrahepatic portal vein. In other patients, it was not investigated as they are asymptomatic. A child presented multiorgan failure with fulminant hepatic failure at birth. The shunt was radiologically closed and clinical impairment reversed rapidly. He is now asymptomatic with no longer images of CPSS in ultrasound scan controls. Also, seven children are asymptomatic at this time and are monitored periodically. Seven children had prenatal diagnosis, in five the shunt closed spontaneously. Nine children were symptomatic in their evolution (hyperammonemia, regenerative nodules, cholestasis, gastrointestinal bleeding). Of these, in five we performed balloon test occlusion, tolerated in all patients, followed by radiological closure. In our experience, the advancement of interventional radiology techniques avoided surgery to close the shunt. CONCLUSIONS: Morphologically, the CPSS is extremely heterogeneous, with multiple possible connections established. CPSS has multiple clinical presentations, from asymptomatic patients to acute liver failure. The therapeutic approach should be individualized and therefore held in overspecialized centers.

Severe tracheobronchial injuries: our experience.

INTRODUCTION: Severe tracheobronchial injuries (TBI) in children are usually traumatic or iatrogenic. However, they can also be caused by mediastinal infections that lead to critical situations. We herein report our experience in the treatment of these lesions. METHODS: A retrospective study was conducted for patients treated at our center from 2008 to 2014. TBI was diagnosed by imaging studies and bronchoscopy. Treatment was initially conservative (drainage of air and secretions, mechanical ventilation with minimal pressures, and an early extubation) with a limited use of surgical procedures whenever necessary. RESULTS: A total of 10 patients (7 males and 3 females) with a median age of 7.5 years (range, 3-17 years) suffered TBI. The mechanism was traumatic in six (three accidental and three iatrogenic) and mediastinal infection in four (three mycotic and one bacterial abscesses). All traumatic cases responded to conservative measures, except one iatrogenic lesion, which was surgically repaired. There were no complications or residual damages. Two patients with mediastinal infection presented with sudden cardiorespiratory arrest, one with hemoptysis caused by an arteriotracheal fistula and the other because of carinal rupture. Both died before any therapeutic measures could be taken. The other two patients were treated, one with previous extracorporeal membrane oxygenation support, underwent arterial embolization, but ultimately died, and the other one survived, but required esophagectomy and creation of a thoracostome for secondary wound closure of the bronchocutaneous fistula. CONCLUSION: Conservative treatment with gentle respiratory support suffices in most traumatic cases of TBI. Infectious abscesses with involvement of adjacent structures sometimes require complex surgery and are life-threatening.

Tertiary surgery for complicated repair of esophageal atresia.

AIM: The ideal repair of esophageal atresia (EA) is primary anastomosis with closure of the fistula if present. Long gap or local circumstances prompt other procedures that occasionally lead to disastrous complications. The aim of this study was to analyze the management of these complications in a tertiary referral center. PATIENTS AND METHODS: A retrospective review of patients treated for EA between 1993 and 2013 was conducted. Both the patients were primarily treated by us, and referrals from elsewhere after two or more failed operations were included. RESULTS: In total, 23 patients were included (3/176 cases of EA treated primarily by us and 20 referrals). Of the 23 patients, 6 had type I EA, 15 type III (four long gaps), 1 type IV, and 1 type V. Cardiac anomalies were associated in seven cases, duodenal atresia in three, and Down syndrome in two patients. Primary anastomosis was initially achieved in 12 patients. Primary or secondary Foker lengthening was used in seven cases. The causes of the failure were anastomotic leaks in nine, unmanageable strictures in seven, and refistulization in five patients. These patients required 66 reoperations (median of 3 [2-7]) before inclusion in the study. Radical tertiary treatment consisted of 15 esophageal replacements (11 colonic grafts and 4 gastric pull-ups), and 1 esophageal-gastric disconnection. Five patients previously treated with esophageal replacement and referred for graft problems required 13 interventions. Two families did not give consent for one replacement and one disconnection. Complications appeared in 12 patients, and 9 additional operations were required in 7 patients. With a follow-up of 31 months (range, 4-139 months) 15 patients take all their meals per os, 5 occasionally use the gastrostomy, and 2 and 1 are fed exclusively via gastrostomy or jejunostomy. All tracheoesophageal fistulas were closed, but 15 cases are below p3 for weight and 12 for height. Three patients (13%) ultimately died 32 months (range, 9-56 months) after the first operation (due to aspiration in one, and for causes unrelated to it in the other two [tracheostomy obstruction and Guillain-Barré syndrome]). CONCLUSIONS: When repeated complications appear after EA repair, radical surgical attitudes may be justified. If esophageal continuity cannot be reestablished, the native esophagus may have to be discarded and replaced. Many complications should be expected, but the end result can be good. These patients should be referred to centers with large experience in the management of this complex condition.

Early endoscopic dilation and mitomycin application in the treatment of acquired tracheal stenosis.

INTRODUCTION: Acquired airway stenosis is a common complication in children after periods of tracheal intubation. We reviewed our experience in the endoscopic treatment of these lesions. PATIENTS AND METHODS: We performed a retrospective review of patients who presented acquired tracheal-subglottic stenosis (SGS) treated at our center from 2005 to 2012. We reviewed the etiology, age, clinical presentation, methods of diagnosis, number of bronchoscopies, angioplasty balloon dilations performed, and long-term results. RESULTS: A total of 18 patients (13 M, 5 F) were treated at our institution between 2005 and 2012. Median age at treatment was 3.5 months (range, 1-96 months). Of the 18 children, 16 children had SGS (all cases were postintubation), and 2 children presented tracheal stenosis (1 postintubation, 1 after tracheal surgery). Median intubation time was 30 days (range, 3-120 days). Extubation failure and stridor were the main clinical features. SGS were diagnosed as grade I in three patients, grade II in nine patients, and grade III in six patients. Bronchoscopy allowed diagnostic in all cases, and was followed by angioplasty balloon dilation, with a median of 2.5 (range, 1-5) sessions. In SGS grade I, the relation patient/number of dilations was 1; in SGS grade II 2.6, and in SGS grade III 3.5. Mitomycin was applied in 15 patients. No patients presented intraoperative complications or required reoperation. Median follow-up time was 36 months (range, 5-72 months) and no recurrence was noticed. CONCLUSIONS: Early endoscopic dilation with balloon shows as an effective and safe treatment in acquired tracheal and SGS.

Failed Nissen fundoplication in children: causes and management.

INTRODUCTION: Nissen fundoplication (NF) is the gold standard procedure for the treatment of gastroesophageal reflux (GER) in children. However, it fails in a significant proportion of patients. The purpose of this study was to identify preoperative predictors of failure and to examine the results of reoperations. PATIENTS AND METHODS: The charts of patients who underwent NF at our institution between 1992 and 2011 were retrospectively reviewed. Surgery was indicated in patients with symptomatic GER in whom medical treatment failed, particularly, in cases of esophageal atresia (EA), congenital diaphragmatic hernia (CDH), and neurologic impairment (NI). Chi-square comparisons and logistic regression were used to test comorbidities, previous abdominal surgery, surgical technique, gastrostomy, pyloromyotomy or pyloroplasty, age, weight, and surgical complications as possible predictors of NF failure. RESULTS: A total of 360 children (217 male and 143 female) underwent NF. Comorbidities were NI (n = 100, 27.8%), EA (n = 50, 13.9%), CDH (n = 22, 6.1%), and abdominal wall defects (AWD) (n = 6, 1.7%). A total of 35 patients (9.7%) had esophageal stenosis. Age at surgery was 3.06 years (0.04-20.7 years) and weight was 12 kg (2-77 kg). NF was open in 196 patients (54.4%) and laparoscopic in 164 patients (45.6%) (with 9 conversions). Follow-up was 6.7 years (0.01-18.7 years). A total of 42 patients (11, 7%) had postoperative complications (10 wound infection, 9 dumping syndrome, 8 gastrostomy related complications, 7 intestinal obstruction, 5 evisceration, 2 chylothorax, and 1 pneumothorax). Reflux recurred in 42 patients (11.7%) and 35 patients (9.7%) underwent redo NF 1.01 years (0.02-8.4 years) after the initial surgery. A total of nine patients (2.5%) required further interventions (five another redo NF, three esophageal replacements, and one esophago-gastric disconnection). A total of 29 patients (8.1%) died during the follow-up (25 because of their baseline disease, 3 in the postoperative period, and 1 because of pulmonary aspiration 3 years after surgery). EA (31.6% failure) and CDH (46.7% failure) were the only comorbidities predictive of NF failure (p < 0.05). CONCLUSIONS: Failure of NF is particularly frequent in patients previously operated upon for EA or CDH and can be predicted preoperatively. However, the benefits of the operation may outweigh this risk. Redo NF is indicated if symptoms of GER recur, but the proportion of failure is even higher. In subsequent failures, other options like esophageal replacement or esophagogastric dissociation should also be considered.

Cephalic pancreaticoduodenectomy for bleeding duodenal arteriovenous malformation.

Introduction Treatment of recurrent severe gastrointestinal bleeding due to arteriovenous malformations may require complex resections. In some particular locations, extensive surgery is the only way out, as shown in this report. Case Report A 2.5-year-old child suffered repeated episodes of upper gastrointestinal bleeding since the first month of life. After an extensive diagnostic workout, the diagnosis of duodenal arteriovenous malformation was established. Cephalic pancreaticoduodenectomy with pyloric preservation was performed and no further episodes of bleeding occurred in the ensuing 2 years. Conclusion Bleeding malformations located in the pancreaticoduodenal area can be effectively treated in children by pylorus-preserving cephalic pancreaticoduodenectomy.

Abnormal development of the enteric nervous system in rat embryos and fetuses with congenital diaphragmatic hernia.

BACKGROUND/AIM: Esophageal dilatation, gastroesophageal reflux, and intestinal obstruction have been demonstrated in CDH survivors. Abnormal esophageal and intestinal innervations were recently found in rats and babies with this disease. Our aim was to further characterize these malformations in embryos and fetal rats exposed to nitrofen. METHODS: Pregnant rats received either 100 mg nitrofen or vehicle on E9.5. Fetuses were recovered at E15, E18, and E21. Sections of esophagus and small bowel were histochemically stained with acetylcholinesterase (AChE) and immunostained for PGP9.5. PGP9.5 gen protein were measured on E21 and PGP9.5 mRNA on E15, E18 and E21. Comparisons between groups were made with non-parametrics tests. RESULTS: Histochemistry and immunohistochemistry showed deficient innervation in all anatomical areas studied at E15, E18, and E21, and WB confirmed this decrease in E21 fetuses. PGP9.5 messenger was decreased in nitrofen-exposed animals on E18 (esophagus) or E15 (small bowel), and increased on E21 in the esophagus and E18 in small bowel. CONCLUSIONS: Development of the enteric nervous system of the esophagus, stomach, and small bowel is deficient in rat embryos and fetuses exposed to nitrofen. These anomalies could account in part for the long-term gastrointestinal morbidity observed in CDH survivors.

Graft-vs-host disease after small bowel transplantation in children.

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.

Diseño y validación de una vía clínica para la apendicitis aguda infantil / Design and validation of a clinical pathway for acute appendicitis in children

Objetivo: Elaboración, implantación y evaluación de una vía clínica (VC) para la apendicitis aguda en el Hospital Infantil La Paz de Madrid. Material y método: Coordinación del equipo de trabajo por la unidad de calidad del hospital. Revisión bibliográfica buscando la mejor evidencia científica. Diseño de los documentos de la VC. Implantación de la vía en junio de 2003. Realización del estudio piloto descriptivo. Resultados: Diseño de los documentos de la VC mediante el consenso de todos los profesionales implicados en el proceso. Para ello se realizaron 5 reuniones durante los meses de abril y mayo de 2003. Los documentos son los siguientes: matriz temporal, hoja de información para el paciente durante su estancia hospitalaria, recomendaciones al alta, hoja de registro para tratamientos y cuidados de enfermería, hoja de variaciones, encuesta de satisfacción, indicadores para la evaluación. Realización del estudio piloto descriptivo previo a la implantación definitiva: la estancia media se ajusta a la estipulada en la VC (6,8 días en apendicitis complicadas y 3,0 en no complicadas), las variaciones se produjeron por causa del paciente (13,5%) y por los profesionales (29,7%), ninguna por la institución. El tipo de antibioterapia fue el estipulado en la mayoría de los casos (94,6%). Conclusiones: La implantación de la vía clínica ha facilitado el consenso entre los profesionales. La estandarización del tratamiento antimicrobiano ha facilitado el control de la variabilidad clínica no justificada. El cumplimiento de las estancias establecidas se produjo en la mayoría de los pacientes

Objective: To design, introduce and evaluate a clinical pathway (CP) for acute appendicitis in children at La Paz Children's Hospital in Madrid (Spain). Material and method: A multidisciplinary team was coordinated by the quality assurance unit at the hospital. A search was conducted for the best scientific evidence published and the CP documents were designed. The CP was implemented in June 2003, the starting point for the descriptive pilot study. Results: Following five meetings held in April and May 2003, the professionals involved agreed on the design of the CP documents. The documents are as follows: framework, patient information sheet, postoperative recommendations, records of medical care and treatment, record of changes, patient evaluation sheet, evaluation instructions. A descriptive pilot study was performed prior to definitive implantation of the CP. The mean length of stay matched that stipulated by the CP (6.8 days for complicated appendicitis and 3.0 for uncomplicated appendicitis). Variations were related to the patient's condition (13.5%) and to the medical staff (29.7%). No variations were due to the center. In most patients (94.6%) the type of antibiotic therapy stipulated was administered. Conclusions: The implantation of the CP has made consensus among professionals easier. The standardization of antibiotic treatment has helped to control unjustified clinical variations. In most patients, the length of stay coincided with that established in the CP

Compound heterozygous mutations in the SRD5A2 gene exon 4 in a male pseudohermaphrodite patient of Chinese origin.

The goal of this study was to perform 5-alpha-reductase type 2 gene (SRD5A2) analysis in a male pseudohermaphrodite (MPH) patient with normal testosterone (T) production and normal androgen receptor (AR) gene coding sequences. A patient of Chinese origin with ambiguous genitalia at 14 months, a 46,XY karyotype, and normal T secretion under human chorionic gonadotropin (hCG) stimulation underwent a gonadectomy at 20 months. Exons 1-8 of the AR gene and exons 1-5 of the SRD5A2 gene were sequenced from peripheral blood DNA. AR gene coding sequences were normal. SRD5A2 gene analysis revealed 2 consecutive mutations in exon 4, each located in a different allele: 1) a T nucleotide deletion, which predicts a frameshift mutation from codon 219, and 2) a missense mutation at codon 227, where the substitution of guanine (CGA) by adenine (CAA) predicts a glutamine replacement of arginine (R227Q). Testes located in the inguinal canal showed a normal morphology for age. The patient was a compound heterozygote for SRD5A2 mutations, carrying 2 mutations in exon 4. The patient showed an R227Q mutation that has been described in an Asian population and MPH patients, along with a novel frameshift mutation, Tdel219. Testis morphology showed that, during early infancy, the 5-alpha-reductase enzyme deficiency may not have affected interstitial or tubular development.