Thoracic outlet syndrome in adolescents: a case series. / Síndrome del opérculo torácico en adolescentes: serie de casos.

OBJECTIVE: Thoracic Outlet Syndrome (TOS) is caused by a compression of the brachial plexus and the subclavian vessels in their passage to the upper limb. It mostly occurs in women aged 20-50, so it is infrequent in children. We present our results in the diagnosis and management of pediatric TOS. MATERIAL AND METHODS: Retrospective study of patients diagnosed with TOS between December 2017 and June 2018. Clinical, radiological, surgical, and evolution variables were assessed. RESULTS: Five TOS were diagnosed in 4 patients - one TOS was bilateral. Mean age at diagnosis was 12.5 years (7-15), and there was a delay in diagnosis of 153 days (10-36). TOS was either venous (3) or neurogenic (2). Patients presented with pain (5/5), edema (4/5), hypoesthesia (3/5), decreased strength (3/5), and cervical pain (2/5). One patient presented with sport-related pain. Neurophysiological study was normal in three cases. Two patients presented bone anomalies at CT-scan. Three surgeries were performed in two patients using the supraclavicular approach with resection of the anomalous first rib and scalenectomy. One patient refused surgery, and another patient remained expectant without reappearance of symptoms. Postoperative follow-up was 9 months (6-12), with progressive improvement of symptoms. CONCLUSIONS: TOS may occur in adolescents in the form of upper limb pain and edema. Imaging tests are recommended to detect abnormal anatomical structures. The supraclavicular approach represents a safe and effective technique in decompressing the thoracic outlet.

OBJETIVO: El síndrome del opérculo torácico (SOT) está causado por una compresión del plexo braquial y vasos subclavios en su paso hacia la extremidad superior. Patología típica de mujeres entre 20 y 50 años, que es infrecuente diagnosticar en niños. Presentamos nuestros resultados en el diagnóstico y tratamiento del SOT pediátrico. MATERIAL Y METODOS: Estudio retrospectivo de pacientes diagnosticados de SOT entre diciembre 2017 y junio 2018. Se analizaron variables clínicas, radiológicas, quirúrgicas y de evolución. RESULTADOS: Cinco SOT fueron diagnosticados en cuatro pacientes, uno de ellos bilateral. La edad media al diagnóstico fue de 12,5 años (7-15) y hubo una demora en el diagnóstico de 153 días (10-360). SOT venoso (3) y neurológico (2). Presentaron dolor (5/5), edema (4/5), hipoestesia (3/5), disminución de fuerza (3/5) y dolor cervical (2/5). Una paciente presentaba dolor asociado al deporte. El estudio neurofisiológico fue normal en tres casos. Dos pacientes presentaron anomalías óseas por TAC. Se realizaron tres intervenciones quirúrgicas en dos pacientes por abordaje supraclavicular realizando resección de la primera costilla anómala y escalenectomía. Una paciente rechazo la intervención y en otra se mantuvo en una actitud expectante sin reaparición de los síntomas. Seguimiento posoperatorio de 9 meses (6-12) con mejoría progresivas de los síntomas. CONCLUSION: El SOT puede darse en adolescentes siendo el dolor y edema de la extremidad superior lo más específico. Se recomienda la realización de pruebas de imagen para detectar estructuras anatómicas anómalas. El abordaje supraclavicular se presenta como una técnica segura y eficaz en la descompresión del desfiladero torácico.

Angiotensin II receptor agonist antibodies are associated with microvascular damage in lupus nephritis.

Angiotensin II type 1 receptor agonist antibodies (AT1R-AAs) have been associated with hypertension, atherosclerosis and vascular inflammation in human diseases. The aim of the study was to evaluate the prevalence of AT1R-AAs in active lupus nephritis (LN) patients and their association with vascular damage. One hundred and seven active LN patients underwent a complete clinical examination, measurement of AT1R-AAs, ambulatory blood pressure monitoring, carotid intima-media thickness measurement and morphometric analysis of subintimal fibrosis and medial hyperplasia of the vessels in the kidney tissue. Plasma AT1R-AAs were positive in 58 (54.2%) patients. The Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score, complement C3 and C4 levels and titers of anti-dsDNA antibodies were higher in the group with positive AT1R-AAs compared with those with negative AT1R-AAs. The AT1R-AA titers correlated with anti-dsDNA antibody titers and with complement C3 and C4 serum levels. In the kidney biopsy, the percentage of subintimal fibrosis and the area of medial hyperplasia were greater in the AT1R-AA-positive patients. No differences in arterial pressure, carotid intima-media thickness and response to therapy were detected. In conclusion, AT1R-AAs are prevalent in active LN patients and are associated with histologic features of microvascular damage.

[Slipping rib syndrome in paediatrics: report of 4 cases]. / Síndrome de la costilla deslizante. Serie de 4 casos clínicos.

AIM OF THE STUDY: The slipping rib syndrome (SRS) is an unknown pathology for the pediatric surgeon due to its low incidence in children. The weakness of the costal ligaments allowing an area of rib hypermobility has been postulated recently as the main etiology. It produces an intermittent pain in the lower thorax or upper abdomen that can affect to the daily activities and can be the origin of unspecific chronic pain. METHODS: A retrospective review of patients diagnosed with SRS between october 2012 and march 2017 was performed. Data of demographics, symptoms, imaging studies, surgical findings and long-term follow-up were collected. RESULTS: During this period, 4 patients were diagnosed with SRS. Median age at diagnosis was 13 years (12-15 years) with a mean duration of symptoms of 13 months (12-36 months). In 2 patients the SRS was associated with Costal Dysmorphia (CD). The initial diagnosis was clinical with posterior ultrasound confirmation. Resection of the affected cartilages was performed in 3 patients and after a follow-up of 6 months (3-30 months), they all are painless and refer a good cosmetic result. One patient refused the intervention. CONCLUSIONS: The SRS is an infrequent cause of thoracic pain with an etiology not well understood. The awareness of this disease and its typical presentation can avoid unnecessary studies. The resection of the affected cartilages is a safe and effective treatment.

INTRODUCCION: El síndrome de costilla deslizante (SCD) es una entidad poco frecuente en niños. Se cree que su causa es una debilidad en los ligamentos costales que permite una hipermovilidad de las costillas. Genera un dolor intermitente en la región baja del tórax o alta del abdomen que puede afectar a las actividades de la vida diaria o generar un dolor crónico. MATERIAL Y METODOS: Revisión retrospectiva de SCD entre octubre de 2012 y diciembre de 2017. Se recogió información acerca de los datos demográficos, síntomas, estudios de imagen, hallazgos intraoperatorios, material fotográfico y seguimiento a largo plazo. RESULTADOS: Durante este periodo, 4 pacientes fueron diagnosticados de SCD. La mediana de edad al diagnóstico fue de 13 años (12-15 años) con una duración previa de los síntomas de 13 meses (12-36 meses). En 2 pacientes se asoció una dismorfia costal (DC). El diagnóstico fue clínico con confirmación ecográfica. Se realizó resección de los cartílagos afectos en 3 pacientes con un seguimiento posterior de 6 meses (3-30 meses). Actualmente se encuentran sin dolor y con un resultado estético satisfactorio. Un paciente rechazó la intervención. CONCLUSIONES: El SCD aparece en pacientes preadolescentes que en algunos casos asocian DC. Una exploración física y ecografía enfocada son las claves para un diagnóstico certero. La resección de cartílagos es efectiva a largo plazo.

[Long-term results of the early endoscopic treatment of acquired tracheal-subglottic stenosis: 10 years of experience]. / Resultados a largo plazo del tratamiento endoscópico precoz de las estenosis subglóticas y traqueales adquiridas: 10 años de experiencia.

INTRODUCTION: Acquired stenosis of the airway is a common complication after endotracheal intubation. Endoscopic dilation has been accepted as the treatment of choice in cases detected precociously. Our goal is to know the current status of the patients treated in our hospital with endoscopic dilation in the last 10 years. MATERIAL AND METHODS: Retrospective cohort study of patients with subglottic and tracheal acquired stenosis (STAS) early treated endoscopically with balloon dilation at our center in the last 10 years. Bronchoscopy control at 2 weeks, a month, 3 and 6 months post-dilation were performed and later on depending on the symptoms. RESULTS: 32 patient were treated in the period considered. The median age was 4.5 (3-120) months. There were necessary 2.5 (1-5) dilations per patient. All cases were extubated in the operating room or in the following 24 hours. There were no complications during the procedure. Follow-up time was 6 (1-10) years. Only 1 of the 32 patients have had recurrence of stenosis 2 years after, it was secondary to reintubations due to new surgical interventions; which it was dilated successfully. CONCLUSIONS: Early endoscopic dilation in the acquired airway stenosis is a safe and effective long-term procedure. The results support the use of this technique as a treatment of choice in these patients.

INTRODUCCION: La estenosis adquirida de la vía aérea es una complicación frecuente tras la intubación endotraqueal. La dilatación endoscópica ha sido aceptada como tratamiento de elección en los casos detectados de forma precoz. Nuestro objetivo es conocer el estado actual de los pacientes tratados en nuestro centro mediante dilatación endoscópica en los últimos 10 años. MATERIAL Y METODOS: Estudio de cohorte retrospectivo de pacientes con estenosis subglóticas y traqueales adquiridas (ESTA) tratadas endoscópicamente mediante dilatación con balón en nuestro centro en los últimos 10 años. Se realizaron broncoscopias de control a las 2 semanas, al mes, a los 3 y 6 meses postdilatación y posteriormente en función de la clínica. RESULTADOS: Se trataron 32 pacientes de ESTA de reciente aparición en dicho periodo. La mediana de edad fue de 4,5 (3-120) meses. Fueron necesarias 2,5 (1-5) dilataciones por paciente. Todos los pacientes fueron extubados en quirófano o en las 24 horas siguientes al procedimiento. No hubo complicaciones durante los procedimientos ni durante el postoperatorio. El tiempo de seguimiento fue de 6 (1-10) años. Solo 1 de los 32 pacientes presentó recidiva de la estenosis 2 años después que fue secundaria a reintubaciones por nuevas intervenciones quirúrgicas; la cual se dilató nuevamente. CONCLUSIONES: La dilatación endoscópica precoz en las estenosis adquiridas de la vía aérea es un procedimiento seguro y eficaz a largo plazo. Los resultados avalan el uso de esta técnica como tratamiento de elección en estos pacientes.

Mesohepatectomy for Centrally Located Tumors in Children.

INTRODUCTION: Central hepatectomy or mesohepatectomy (MH) is a complex surgical technique rarely used in children. It is indicated in central tumors to preserve functioning liver mass avoiding an extended right hepatectomy. The purpose of this article is to analyze our experience with this technique. METHODS: We reviewed five patients who underwent MH in the period from 2008 to 2014. Diagnoses were hepatoblastoma PRETEXT III (two cases), hepatic embryonal sarcoma (one case), focal nodular hyperplasia (one case), and vascular tumor with rapid growth in a newborn causing an acute liver failure, compartment syndrome, and multiple organ failure (one case). In all cases, the tumor was centrally located, including the segment IVb, with large displacement of the hepatic pedicle in two cases. RESULTS: MH was standard in three cases and under total vascular exclusion in two cases. All children are alive with a mean follow-up of 38 (6-70) months. None of the children required reoperation because of bleeding. One child developed a biliary fistula in the cutting area that closed spontaneously. The newborn with the vascular tumor required the placement of a Gore-Tex patch (W. L. Gore & Associates, Inc, Flagstaff, Arizona, United States) to relieve the compartment syndrome. He subsequently underwent partial embolization of the tumor and MH under vascular exclusion. CONCLUSIONS: In selected patients, MH is an alternative to trisegmentectomy and should be available in advanced pediatric hepatobiliary units.

[Nissen fundoplication in children under 1 year of age: is age important?] / Funduplicatura de Nissen en niños menores de 1 año: ¿la edad importa?

AIM: Nissen fundoplication (NF) is a procedure with technical difficulties and variable functional prognosis the lower the patient's age is. Our objective is to analyze the peculiarities of this procedure when performed in children under 1 year. MATERIALS AND METHODS: Retrospective study of the NF in our center from 1999 to 2014. We review the differences between children under 1 year of age and the leftover of the series: history, indications, surgical approach and postoperative outcomes. RESULTS: A total of 233 patients (57.1% male) were operated at a median age of 2.3years (1 month-17.31years), of which 82 (35.2%) were younger than 1 year. It Open surgery was performed in 118 patients (86.6% of children under 1 year and 31.1% over 1 year, p <0.05) and laparoscopic in 115. The median follow-up was 3.92 ± 3.24 years. Patients under 1 year had a higher number of comorbidities (91.5% vs 81.5%), respiratory symptoms (76.8% vs 49.7%) and postoperative complications (20.7% vs 9.9% OR = 2.4), with statistically significant differences (p <0.05). There were not differences in the Nissen's failure rate (15.9% vs 8.6%) or the need of reoperation (15.9% vs 7.9%). CONCLUSIONS: Patients under 1 year operated by NF form a group with particular indications and comorbidities. Although the outcomes among these patients are favourable, surgical complications are more frequent than in older children.

INTRODUCCION Y OBJETIVOS: La funduplicatura de Nissen (FN) es un procedimiento que plantea dificultades técnicas y un pronóstico funcional variable cuanto menor es la edad del paciente. Nuestro objetivo es analizar las peculiaridades de esta intervención cuando se realiza a niños menores de 1 año. MATERIAL Y METODOS: Estudio retrospectivo de las FN en nuestro centro de 1999 a 2014. Se evaluaron las diferencias entre menores de 1 año de vida y el resto de la serie en cuanto a antecedentes, indicaciones, abordaje quirúrgico y evolución postoperatoria. RESULTADOS: Un total de 233 pacientes (57,1% varones) fueron intervenidos a una mediana de edad de 2,3 a (1 mes-17,31 a), de los que 82 (35,2%) eran menores de 1 año. La cirugía fue abierta en 118 pacientes (86,6% de los menores de 1 año y 31,1% de los mayores de 1 año, p <0,05) y laparoscópica en 115. La mediana de seguimiento fue de 3,92 años. Los pacientes menores de 1 año presentaron mayor número de comorbilidades (91,5% vs 81,5%), sintomatología respiratoria (76,8% vs 49,7%) y complicaciones postoperatorias (20,7% vs 9,9%, OR=2,4), siendo estas diferencias estadísticamente significativas (p <0,05). No hubo diferencias en cuanto al fallo del Nissen (15,9% vs 8,6%) ni a la necesidad de reintervención (15,9% vs 7,9%). CONCLUSIONES: Los pacientes menores de 1 año operados mediante FN constituyen un grupo con indicaciones y comorbilidades particulares. Aunque los resultados son favorables, las complicaciones quirúrgicas son más frecuentes.

[Noninvasive positive pressure ventilation in postoperative period of tracheal surgery]. / Ventilación con presión positiva no invasiva en el postoperatorio de cirugía traqueal.

OBJECTIVES: Reconstructive surgery of the airway often means prolonged periods of intubation during the post-operatory period, increasing the needs for drugs and favoring the appearance of infectious complications. We present an original system of ventilatory support with non-invasive positive pressure ventilation (NIPPV) using in patients subjected to reconstructive surgery of the airway. PATIENTS AND METHODS: A retrospective study in patients undergoing reconstructive procedures of the airway in the year 2009 was carried out. We exclude those treated endoscopically and those who had vascular rings. The positive pressure mechanism used in the Surgery Critical Care Unit was a design made by the unit based on the circuit devised by Mapleson that provides optimum levels of ventilation without need for connection to a respiratory. We analyze the results, postoperatory intubation time, time dependent on NIPPV and medical treatment received. RESULTS: A total of 7 patients (1 Female and 6 Males) with median age of 1.6 (0.1-7.5) years were included. The diagnoses were: 4 subglottic stenosis, 2 had tracheal stenosis and 1 subcarinal stenosis with involvement of both principal bronchioles. The techniques used were: laryngotracheoplasty with costal cartilage graft (4), tracheoplasty with costal cartilage (1) and sliding tracheoplasty (2) with bilateral bronchoplasty in one of them. The mean time of nasotracheal intubation was 3 days, and mean time of NIPPV was 2.3. No patient required reintubation and none had infectious complications. CONCLUSIONS: Ventilatory support by VPPNI allows effective extubation in these patients, it being possible to maintain a safe airway. Infectious complications, frequent in prolonged intubations, were not observed in any of the cases.

Late surgery for spinal deformities in children previously treated for neural tumors.

BACKGROUND/AIM: Surgery for neural tumors occasionally involves opening of the neural canal and/or costovertebral resection that may be followed by radiotherapy. Spinal deformities (SD) may occur in these cases. This study investigated the incidence, surgical indications and results of repair of late SD in a large series of patients with neural tumors. METHODS: A retrospective review of neural tumors treated between 1991 and 2009 at our institution identified children with SD who subsequently required spinal surgery. SD was meaÂ-sured using Cobb's angle. Instrumentation of the spine was indicated in patients with a Cobb's angle of more than 60° or rapidly progressing SD. The operation was adapted to the location, type and degree of SD. Patients were followed up and the remaining deviations were measured annually. RESULTS: Out of a series of 188 patients with neural tumors, 5 of 173 patients with neuroblastomas/ganglioneuromas (NB/GN) and 3 of 15 patients with PNET/Askin tumors developed SD over time. NB/GN children had intraspinal tumors requiring laminotomy (n=3) or laminectomy (n=2). PNET/Askin tumor patients had undergone largescale rib and partial vertebral body resection and radiotherapy. Scoliosis or kyphosis were located in the thoracic spine in 4, in the thoracolumbar spine in 3 and in the cervicothoracic spine in 1. The median preoperative Cobb's angle was 65° (50°-90°) prior to spinal surgery performed 2.9 (0.4-11) years after tumor removal. 3 growth bars, 2 vertical expandable prosthetic titanium ribs, 1 pediatric ISOLA device and anterior instrumentation were used. Arthrodesis was necessary in 3 patients. Median post-instrumentation deformity after 3.1 (0.2-12.2) years was 40° (20°-50°), giving an improvement of 38.4%. CONCLUSIONS: With increasing numbers of children surviving cancer, attention must focus on sequelae. A sizeable number of survivors of NB/GN and PNET/Askin tumors develop serious SD requiring surgical correction. Pediatric surgeons, patients and relatives must be aware of these possible sequelae.

High-risk hepatoblastoma: results in a pediatric liver transplantation center.

AIM: Aim of the study was assess the results of the treatment of High-Risk Hepatoblastoma (HRH) in a tertiary center where all liver surgery facilities, including pediatric transplantation (LT), are available. METHODS: 91 primary liver tumors treated between 1991 and 2009 were retrospectively reviewed. HRHs as defined by the SIOP criteria (PRETEXT IV or any stage with venous involvement, extrahepatic disease, tumor rupture and <100 ng/ml serum AFP) were identified and imaging and biopsies were reviewed. The treatment consisted of total removal of the tumor, involving extended hepatectomies and LT if necessary, together with SIOPEL-guided chemotherapy. RESULTS: 23/57 hepatoblastomas were HRH (11F/12M). 17 were considered unresectable by standard techniques, 3 had extrahepatic disease, and 3 fulfilled both criteria. Mean age at diagnosis was 2.3 ± 2.4 years. 3 children (referred after chemotherapy) died without surgery. 4 had resections (2 left and 2 right trisegmentectomies). Primary LT was required in 15 children (7 cadaveric donors and 8 living related donor transplantations (LRDT), 2 of them with retrohepatic vena cava replacement), and 1 patient had rescue LT after recurrence. Mean follow-up was 4.8 ± 2.9 years. 2 children who had undergone liver resection developed pulmonary metastases at 1.7 and 1.6 years postoperatively and survived after surgical treatment. 2 children with LT developed EBV-related lymphoma and leukemia respectively but survived. Event-free survival (EFS) at 1, 5, and 10 years was 78.3 ± 8.6%, 63.1 ± 10.5%, and 63.1 ± 10.5%, respectively. 6 children died (3 without surgery, 1 after liver resection, 1 after primary LT and 1 after rescue LT). Overall survival at 1, 5 and 10 years was 78.3 ± 21.7%, 73.2 ± 26.8% and 73.2 ± 26.8%. Of those with primary LT, survival at 1, 5 and 10 years was 93.3 ± 6.4%, 93.3 ± 6.4% and 93.3 ± 6.4%. CONCLUSIONS: Outstanding results in the treatment of HRH are possible in tertiary centers when referral is early (preferably at diagnosis) and specialized liver surgery and transplantation facilities are available.

[Traumatic diaphragmatic hernia: a vital need for an early diagnosis]. / Hernia diafragmática traumática: necesidad vital de un diagnóstico precoz.

INTRODUCTION: Traumatic diaphragmatic hernia (HDT) is exceptional in children. Its diagnosis is usually delayed for appearing in the context of severe polytrauma. The aim is to analyze our series in relation to the severity scores. PATIENTS AND METHODS: Five patients with HDT are presented. Age, biomechanics, clinical, ISS (injury severity score), ITP (pediatric trauma index), GCS (Glasgow coma scale), RTS (Revised Trauma Score) and treatment were studied. The probability of survival (Ps) was calculated with the TRISS method, Ps = 1/(1 + eb), b = b0 + bl (RTS) + b2 (ISS) + B3 (age). RESULTS: There were two males and three females, mean age was six years old (range = 3-10). Traffic accident was the mechanism of the thoracoabdominal trauma in 80% and one was crushed by garage door. The affected side was right in two cases and left in three. Mean ISS was 41 (range = 32-57), ITP 6 (range = 2-9) and RTS 6.1 (range = 5.1 to 6.9). The main symptom was respiratory. Plain chest radiography was diagnostic in three patients, one by CT scann, and another was a finding at laparotomy. Two had liver herniation, one had tension gastrothorax and two had gastric perforations. All patients underwent surgery through the abdomen. Estimated survival by the TRISS method was respectively 86.6%, 78.2%, 57.2%, 84.7% and 57.1%, while the actual was 100%. One has a severe disability. DISCUSSION: To study the biomechanics and semiology is essential in the early diagnosis of TDH in pediatric polytrauma and suspect it is mandatory prior to realize percutaneous chest procedures. The TRISS method has a great importance to assess the adult polytrauma, but specific scores are needed for children.

[Correction of the complicated pectus excavatum with extracorporeal circulation (ECC) support]. / Corrección del pectus excavatum complicado con apoyo de circulación extracorpórea (CEC).

INTRODUCTION: The most serious complication in surgery of the pectus excavatum is the possible cardiac perforation. After its resolution, it is very likely that the relapse of the excavatum will be more severe due to the intense retrosternal fibrosis generated, involving cardiac structures and fixing them to the sternum. Reintervention requires modifications of the usual techniques and special monitoring due to the increased risk of cardiac and large vessel lesions. We present the case of 2 patients with severe pectus excavatum with a previous attempt for correction with cardiac perforation. CLINICAL CASES: Case 1. A 16 year old male with Haller index 9.9, diagnosed of Ehlers-Danlos syndrome and operated on in neonatal period of cardiac disease, with previous attempt for correction using the Ravitch technique that relapsed and cardiac perforation during it. Case 2. An 11 year old girl, with background of diaphragmatic hernia and cardiac perforation on introducing the Nuss bar at 5 years of age, with Haller index 5.5. Both were operated on under ECC with the collaboration of the Cardiac Surgery Service and cardiac adherences were released after median sternotomy. After it, the Nuss bar was introduced and then sternal closure was performed with reinforced steel wires with titanium plates in the anterior and posterior part. DISCUSSION: The backgrounds of the cardiac approach complicate the intervention for the correction of the pectus excavatum, with a high risk of cardiac trauma. Counting on the support of the cardiac surgery and the ECC, we achieve an adequate control and prediction of the complications

[The risk of necrotizing enterocolitis in newborns with congenital heart disease. a single institution-cohort study]. / El riesgo de enterocolitis necrosante en recién nacidos con cardiopatía congénita.

BACKGROUND/AIM: Necrotizing enterocolitis (NEC) is the most common gastrointestinal surgical disease among neonates. Congenital heart disease (CHD) is also a significant cause of infant morbidity and mortality and it is usually associated with prematurity, low birth weight, pulmonary and gastrointestinal disease, conditions that also account for the development of NEC. Our aim is to analyze the incidence and the risk factors for NEC in neonates with CDH admitted to the Neonatal Intensive Care Unit (NICU) in a 6 years period. METHODS: All patients admitted in the NICU for any type of CDH from January 2003 to December 2008 comprised the cohort of this study. The primary outcome measure was the development of NEC in any stage. Sixteen independent variables were selected to include those factors previously correlated with NEC. Uni and multivariate statistical analysis were performed with SPSS 15.0 for Windows. Relative Risks (RR) are presented with 95% confidence intervals. RESULTS: 935 out of the 7803 neonates admitted during this period, 935 (11.9%) presented a CHD. Among them, 70 also had NEC (RR 8.4-17.8), 31 requiring surgical treatment (Bell stages III and IV). The risk for NEC was specially increased if CHD needed surgical correction (2.1-5.1) and among babies with very low birth weight (VLBW, <1,500 g) (2-4.8). After multivariate analysis the only independent variables associated with an increased risk of NEC were birth weight less than 1,500 g (2.2-7.4), the need of surgical correction of CHD (2.2-7.1) and a clinical picture of septicaemia (1.1-3.9). CONCLUSIONS: The incidence of NEC is greater in neonates with CDH than in the normal newborn population. Early recognition of NEC should be warranted among CHD patients, specially in those with VLBW, surgical treatment of CHD and sepsis. In this sub-cohort of patients an increased risk of NEC should be suspected.