Analysis of a clinical guideline for treatment and early discharge in complicated acute appendicitis. / Análisis de una guía clínica para el tratamiento y alta precoz en apendicitis aguda complicada.

OBJECTIVE: The objective of this study was to assess the results of a clinical guideline for the treatment and early discharge of patients with complicated acute appendicitis in terms of infectious complications and hospital stay. MATERIALS AND METHODS: A guideline for appendicitis treatment according to severity was created. Complicated appendicitis cases were treated with ceftriaxone-metronidazole for 48h, with discharge being approved if certain clinical and blood test criteria were met. A retrospective analytical study comparing the incidence of postoperative intra-abdominal abscess (IAA) and surgical site infection (SSI) in patients under 14 years of age to whom the new guideline was applied (Group A) vs. the historical cohort (Group B, treated with gentamicin-metronidazole for 5 days) was carried out. A prospective cohort study to assess which antibiotic therapy (amoxicillin-clavulanic acid or cefuroxime-metronidazole) proved more effective in patients meeting early discharge criteria was also conducted. RESULTS: 205 patients under 14 years of age were included in Group A, whereas 109 patients were included in Group B. IAA was present in 14.3% of patients from Group A vs. 13.8% from Group B (p= 0.83), while SSI was present in 1.9% of patients from Group A vs. 8.25% from Group B (p= 0.008). Early discharge criteria were met by 62.7% of patients from Group A. Median hospital stay decreased from 6 to 3 days. At discharge, 57% of patients received amoxicillin-clavulanic acid, whereas 43% received cefuroxime-metronidazole, with no differences being found in terms of SSI (p= 0.24) or IAA (p= 0.12). CONCLUSIONS: Early discharge reduces hospital stay without increasing the risk of postoperative infectious complications. Amoxicillin-clavulanic acid is a safe option for at-home oral antibiotic therapy.

OBJETIVO: El objetivo de este estudio es evaluar los resultados en términos de complicaciones infecciosas y estancia hospitalaria de la instauración de una guía clínica para el tratamiento y alta precoz en pacientes con apendicitis aguda complicada. MATERIAL Y METODOS: Se elaboró una guía para el tratamiento de las apendicitis en función de su grado de severidad. Las complicadas se trataron con ceftriaxona-metronidazol durante 48 h, siendo alta si cumplen ciertos criterios clínicos y analíticos. Se realizó un estudio analítico retrospectivo comparando la incidencia de abscesos intraabdominales postquirúrgicos (AIA) e infección del sitio quirúrgico (ISQ) en pacientes menores de 14 años sometidos a la nueva guía (Grupo A), respecto a una cohorte histórica (Grupo B), en la que la pauta de tratamiento era gentamicina-metronidazol 5 días. Además, se realizó un estudio de cohortes prospectivas para evaluar qué antibioterapia (amocilina-clavulánico o cefuroxima-metronidazol) es más eficaz en los pacientes que cumplen criterios de alta precoz. RESULTADOS: Se incluyeron 205 pacientes menores de 14 años en el Grupo A y 109 en el Grupo B. Presentaron AIA un 14,3% en el grupo A, frente al 13,8% en el B (p=  0,83); e ISQ un 1,9% y un 8,25% respectivamente (p=  0,008). Cumplieron criterios de alta precoz el 62,7% de los pacientes del Grupo A. La mediana de estancia disminuyó a de 6 a 3 días. Al alta, el 57% recibieron amoxicilina-clavulánico y el 43% cefuroxima-metronidazol, sin hallarse diferencias en términos de ISQ (p=  0,24) ni de AIA (p=  0,12). CONCLUSIONES: El alta precoz disminuye la estancia hospitalaria sin aumentar el riesgo de complicaciones infecciosas postquirúrgicas. La amoxicilina-clavulánico es una opción segura para la antibioterapia oral domiciliaria.

Análisis de una guía clínica para el tratamiento y alta precoz en apendicitis aguda complicada / Analysis of a clinical guideline for treatment and early discharge in complicated acute appendicitis

Objetivo: El objetivo de este estudio es evaluar los resultados entérminos de complicaciones infecciosas y estancia hospitalaria de lainstauración de una guía clínica para el tratamiento y alta precoz enpacientes con apendicitis aguda complicada.Material y métodos: Se elaboró una guía para el tratamiento delas apendicitis en función de su grado de severidad. Las complicadas setrataron con ceftriaxona-metronidazol durante 48h, siendo alta si cumplen ciertos criterios clínicos y analíticos. Se realizó un estudio analíticoretrospectivo comparando la incidencia de abscesos intraabdominalespostquirúrgicos (AIA) e infección del sitio quirúrgico (ISQ) en pacientesmenores de 14 años sometidos a la nueva guía (Grupo A), respecto a unacohorte histórica (Grupo B), en la que la pauta de tratamiento era gentamicina-metronidazol 5 días. Además, se realizó un estudio de cohortesprospectivas para evaluar qué antibioterapia (amocilina-clavulánico ocefuroxima-metronidazol) es más eficaz en los pacientes que cumplencriterios de alta precoz. Resultados: Se incluyeron 205 pacientes menores de 14 años en elGrupo A y 109 en el Grupo B. Presentaron AIA un 14,3% en el grupoA, frente al 13,8% en el B (p= 0,83); e ISQ un 1,9% y un 8,25% respectivamente (p= 0,008). Cumplieron criterios de alta precoz el 62,7%de los pacientes del Grupo A. La mediana de estancia disminuyó a de6 a 3 días. Al alta, el 57% recibieron amoxicilina-clavulánico y el 43%cefuroxima-metronidazol, sin hallarse diferencias en términos de ISQ(p= 0,24) ni de AIA (p= 0,12).Conclusiones: El alta precoz disminuye la estancia hospitalariasin aumentar el riesgo de complicaciones infecciosas postquirúrgicas.La amoxicilina-clavulánico es una opción segura para la antibioterapiaoral domiciliaria.(AU)

Objective: The objective of this study was to assess the results of aclinical guideline for the treatment and early discharge of patients withcomplicated acute appendicitis in terms of infectious complicationsand hospital stay. Materials and methods: A guideline for appendicitis treatmentaccording to severity was created. Complicated appendicitis caseswere treated with ceftriaxone-metronidazole for 48h, with dischargebeing approved if certain clinical and blood test criteria were met. Aretrospective analytical study comparing the incidence of postoperative intra-abdominal abscess (IAA) and surgical site infection (SSI) inpatients under 14 years of age to whom the new guideline was applied(Group A) vs. the historical cohort (Group B, treated with gentamicinmetronidazole for 5 days) was carried out. A prospective cohort study toassess which antibiotic therapy (amoxicillin-clavulanic acid or cefuroxime-metronidazole) proved more effective in patients meeting earlydischarge criteria was also conducted.Results: 205 patients under 14 years of age were included in GroupA, whereas 109 patients were included in Group B. IAA was presentin 14.3% of patients from Group A vs. 13.8% from Group B (p=0.83),while SSI was present in 1.9% of patients from Group A vs. 8.25%from Group B (p=0.008). Early discharge criteria were met by 62.7%of patients from Group A. Median hospital stay decreased from 6 to 3days. At discharge, 57% of patients received amoxicillin-clavulanic acid,whereas 43% received cefuroxime-metronidazole, with no differencesbeing found in terms of SSI (p=0.24) or IAA (p=0.12). Conclusions: Early discharge reduces hospital stay without increas-ing the risk of postoperative infectious complications. Amoxicillin-clavulanic acid is a safe option for at-home oral antibiotic therapy.(AU)

[Evaluation of the anal position in newborns and children with chronic constipation. Incidence of anterior ectopic anus]. / Valoración de la posición del ano en recién nacidos y en niños con estreñimiento crónico. Incidencia del ano anterior ectópico.

INTRODUCTION: Anterior ectopic anus (AEA), continues to be a controversial issue. The objective is to determine the normal position of the anus or anal ano-genital index (AGI) in newborns (NB) and in a group of infants with chronic constipation, know the incidence of AEA in both groups, and its relationship with constipation. METHODS: A prospective aleatory study is performed in where the AGI is determined in 529 NB and 64 older patients with chronic constipation. The AGI is obtained by dividing the distances (cm): vulvar fornix-anus/fornix-coccyx in females and scrotum-anus/scrotum-coccyx in males. The NB group is further studied for gestational age, weight, number of meconium discharges and anal calibration. The newborns with AEA are then controlled to exclude constipation. In patients with chronic constipation, their age, sex and AGI are evaluated. The statistical comparisons are made by a Student's t test and Welch's t test for independent samples. RESULTS: 49.5% of NB are male. The average gestational age is 38.95 +/- 2.08/w in NB females and 38.54 +/- 2.26/w in NB males. The average weight is 3101.91 +/- 511.41/g in newborn females and 3145.14 +/- 573.36/g in newborn males. The average AGI of NB females is 0.40 +/- 0.05 and NB males 0.53 +/- 0.06. AEA is considered with AGI < 0.30 in females and < 0.41 in males. 2.2% of NB females (6/267) and 1.1% of NB males (3/262) have AEA. There is a significant difference between the AGI of NB females and NB males (p < 0.0001). 98% of NB discharge meconium in the first 24 hours. 2 NB females with AGI 0,21 and 0,26 had severe constipation in the first months of life. In group with chronic constipation (64 pacients), 59.4% are female. The AGI is 0.36 +/- 0.1 in females and 0.47 +/- 0.1 in males (p < 0.0001). Concerning of AEA in the NB group (AGI < 0.30 in females and 0.41 in males) incidence of AEA in group with chronic constipation is 35% in males and 47% in females. CONCLUSIONS: The position of the anus in the perineal floor is anterior in females, with significant differences (p < 0.0001) to the AGI in males. AEA has more incidence in females (2.2%). In the constipated group 47% of females had AEA and it is considered the probable reason for this disorder in these patients. To conclude, each NB evaluation protocol should include determination of the AGI in the neonatal period to exclude AEA and to prevent clinical consequences.

[Gastroesophageal reflux disease. Evaluation and indication for surgical treatment by esophageal phmetry]. / Enfermedad por reflujo gastroesofágico, Valoración e indicación de tratamiento quirúrgico mediante pHmetría esofágica.

INTRODUCTION: Oesophageal pH monitoring is considered the best procedure to assess the severity of gastroesophageal reflux disease (GERD), conditioning its treatment. The indication for surgery is becoming less common. We intend to meet this test to what extent it has influenced. MATERIAL AND METHODS: We recruit patients treated in our Centre since the introduction of pH monitoring. We compare the number of times before and after its use. We value clinical manifestations, usual pH metric values versus non-operated patients with pathological test, associated pathology. SAMPLE: 91 patients, 151 pH metric studies (98 preoperative). Number of interventions prior to introduction of pH monitoring: 20/year, post: 3.79/year. SYMPTOMS: gastrointestinal 60.2%, respiratory 10.2%, mixed 24.5%. 495 non-operated patients, 692 pathological studies. PH metric values operated/not operated: No. reflux 116.91 +/- 125.46/101.69 +/- 83.39 (p < 0.001), No. reflux >5 minutes 8.49 +/- 8.28/4.43 +/- 4.85 (p = 0.001), longest reflux 60.21 +/- 95.93/31.16 +/- 80.09 (p < 0.001), clearance 1.27 +/- 1.44/0.86 +/- 1.05 (p = 0.04), DeMeester 52.74 +/- 56.21/29.49 +/- 23.57 (p < 0.001). 14 of 98 preoperative pH monitoring were normal. Associated pathology operated/not operated: 13.26%/ 7.47%. CONCLUSIONS: The pH metric control of patients with GERD and the progress of medical treatment have led to a decrease in surgical procedures. In our experience, the intensity of GERD is a valuable parameter in deciding on the surgery. We attach particular value parameters measuring oesophageal clearance and indices that integrate several of them. However, in some patients without pathological pH monitoring, we have indicated the operation. The prevalence of associated pathology in the operated makes us value them more demanding.

Síndrome del colon izquierdo pequeño: Revisión de nuestra experiencia / No disponible

Objetivo. El objeto es presentar nuestra experiencia con el síndrome de colon izquierdo pequeño (SCIP) e investigar la utilidad de lamanometría anorrectal en el estudio de estos pacientes. Material y métodos. Se realiza una revisión de las historias clínicas de 10 pacientes tratados en nuestro Hospital por SCIP. Se valoran: sexo, antecedentes maternos, tipo de parto, edad gestacional, peso al nacimiento, manifestaciones clínicas, expulsión de meconio, enema con gastrografín, manometría anorrectal y tipo de tratamiento (Nursing, días de reposo digestivo, duración nutrición parenteral) y tiempo de hospitalización. Resultados. De los 10 pacientes, 5 son hombres; 2 son gemelos afectos y otro gemelo afecto presenta un hermano gemelo sano; 6 son hijos de madre diabética. Edad gestacional: 36,6 ± 2,11 semanas. Peso:3.001 ± 689,72 g. Anomalías asociadas en 2: fetopatía diabética, hemivértebrasD11-L2. Todos presentan distensión abdominal y vómitos a las48,73 ± 45,39 horas de vida. Sólo 2 expulsan meconio en las primeras 24horas. Los enemas con gastrografín muestran hallazgos típicos de este síndrome en todos los casos. La manometría anorrectal se realiza en 8 (..) (AU)

Objective. The object is to present our experience with the small lleft colon syndrome (SLCS) and to investigate the usefulness of the anorrectal manometry in the study of these patients. Material and methods. This was a retrospective review of the clinical histories of 10 patients treated in our Hospital for SLCS. The following variables are valued: sex, mother precedents, type of childbirth, gestational age, birth weight, clinical presentation (low colonic obstruction), delayed meconium passage, contrast enema with gastrografín, anorrectal manometry and treatment (Nursing, days of digestive rest, time of parenteral nutrition).Results. During the study period, 10 patient were identifi ed (5are men). SLCS is reported in 2 sets of twins; in one set both twins are affected and in the other set 1 twin is affected. Maternal diabetes (..) (AU)

Valoración de la posición del ano en recién nacidos y en niños con estreñimiento crónico. Incidenciadel ano anterior ectópico / Evaluation of the anal position in newborns and children with chronic constipation. incidence of anterior ectopic anus

Introducción. El ano anterior ectópico (AAE) continúa siendo una entidad controvertida. El objeto es determinar la posición normal del ano o índice anogenital (IAG) en recién nacidos (RN) y en un grupo de niños con estreñimiento crónico, conocer la incidencia del AAE en ambos grupos, y la relación de éste con el estreñimiento. Material y métodos. Se realiza un estudio prospectivo y aleatorio en el que se determina el IAG en 529 RN y en 64 pacientes de más edad con estreñimiento crónico. El IAG se obtiene dividiendo las distancias(cm): horquilla vulvar-ano/horquilla-coxis en niñas y escrotoano/escroto-coxis en niños. En el grupo de RN se estudian, además, la edad gestacional, peso, evacuación meconial y calibre anal. Los RN con AAE son controlados para el despistaje de estreñimiento. En los paciente scon constipación crónica se valora la edad, sexo e IAG. Las comparaciones estadísticas se efectúan mediante los tests de t-Student y elde Welch para muestras independientes .Resultados. De los 529 RN, el 49,5% son hombres. La edad gestacional media en RN niñas es 38,95 ± 2,08/s y 38,54 ± 2,26/s en RN niños. El peso es 3.101,91 ± 511,41/g en RN niñas y 3.145,14 ± 573,36/gen RN niños. El IAG medio en RN niñas es 0,40 ± 0,05 y en niños 0,53± 0,06. Se considera AAE los IAG de menos 2 DS por debajo de la (..) (AU)

Introduction. Anterior ectopic anus (AEA), continues to be a controversial issue. The objective is to determine the normal position of theanus or anal ano-genital index (AGI) in newborns (NB) and in a group of infants with chronic constipation, know the incidence of AEA in both groups, and its relationship with constipation. Methods. A prospective aleatory study is performed in where the AGI is determined in 529 NB and 64 older patients with chronic constipation. The AGI is obtained by dividing the distances (cm): vulvarfornix-anus/fornix-coccyx in females and scrotum-anus/scrotum-coccyx in males. The NB group is further studied for gestational age, weight, number of meconium discharges and anal calibration. The newborns with AEA are then controlled to exclude constipation. In patients with chronic constipation, their age, sex and AGI are evaluated. The statistical comparisons are made by a Student’s t test and Welch’s t test for independent samples. Results. 49.5% of NB are male. The average gestational age is38.95 ± 2.08/w in NB females and 38.54 ± 2.26/w in NB males. The average weight is 3101.91 ± 511.41/g in newborn females and 3145.14± 573.36/g in newborn males. The average AGI of NB females is 0.40± 0.05 and NB males 0.53 ± 0.06. AEA is considered with AGI < 0.30in females and < 0.41 in males. 2.2% of NB females (6/267) and 1.1%of NB males (3/262) have AEA. There is a significant difference between (..) (AU)

Enfermedad por reflujo gastroesofágico. Valoración e indicación de tratamiento quirúrgicomediante pHmetría esofágica / Gastroesophageal reflux disease. Evaluation and indication for surgical treatment by esophageal phmetry

Introducción. La pHmetría esofágica es considerada el mejor procedimiento para valorar la intensidad del reflujo gastroesofágico (RGE),condicionando su tratamiento. La indicación quirúrgica es cada vez menos frecuente. Nos proponemos conocer en qué medida esta prueba ha influido en ello. Material y métodos. Reclutamos los pacientes intervenidos en nuestro Centro desde la introducción de la pHmetría. Comparamos número de intervenciones antes y después de su utilización. Valoramos manifestaciones clínicas, valores pHmétricos habituales frente a no intervenidos patológicos, patología asociada. Resultados. Muestra: 91 pacientes, 151 estudios pHmétricos (98prequirúrgicos). Número de intervenciones previas a introducción de pHmetría: 20/año, posteriores: 3,79/año. Sintomatología: digestiva (..) (AU)

Introduction. Oesophageal pH monitoring is considered the best procedure to assess the severity of gastroesophageal reflux disease(GERD), conditioning its treatment. The indication for surgery is be comingless common. We intend to meet this test to what extent it has influenced. Material and methods. We recruit patients treated in our Centre since the introduction of pH monitoring. We compare the number oftimes before and after its use. We value clinical manifestations, usual (..) (AU)

[Small left colon syndrome: a review of our experience]. / Síndrome del colon izquierdo pequeño: revisión de nuestra experiencia.

OBJECTIVE: The object is to present our experience with the small left colon syndrome (SLCS) and to investigate the usefulness of the anorrectal manometry in the study of these patients. MATERIAL AND METHODS: This was a retrospective review of the clinical histories of 10 patients treated in our Hospital for SLCS. The following variables are valued: sex, mother precedents, type of childbirth, gestational age, birth weight, clinical presentation (low colonic obstruction), delayed meconium passage, contrast enema with gastrografin, anorrectal manometry and treatment (Nursing, days of digestive rest, time of parenteral nutrition). RESULTS: During the study period, 10 patient were identified (5 are men). SLCS is reported in 2 sets of twins; in one set both twins are affected and in the other set 1 twin is affected. Maternal diabetes was identified in 6 patients. The mean gestational age was 36.6 + 2.11 weeks, and the weight 3001 +/- 689.72 g. 2 patient presented anomalies associated: fetopatía diabetic, hemivértebras D11-L2. All 10 infants presented abdominal distention and vomiting at 48.73 +/- 45.39 hours of life. Only 2 presented meconium passage in the first 24 hours. The contrast enema with gastrografin performed show typical findings of this syndrome in all cases. The anorrectal manometry carries out in 8 patients and demonstrates presence of the recto-anal inhibitory reflex (RAIR) in 5 and absence in 3. In these 3 patients with no RAIR, the study was repeated weekly until the presence being observed (normally between 2a and 3a week of life). In 1 newborn was necessary colostomy; the suction rectal biopsy showed immaturity of ganglionars cells and the manometric control previously at the close of the colostomy (9 months) showed presence of the RAIR. Conservative treatment (nursing and digestive rest) was successful in all cases with a mean of duration the 6.40 +/- 5.10 days. 6 patient needed parenteral nutrition during 7.30 +/- 8.49 days. The mean of discharge was 17.78 +/- 13.28 days. The clinical development was favourable in all the cases. CONCLUSIONS: The contrast enema with gastrografin is diagnostic and therapeutic and together with the nursing, has allowed solve the distal intestinal obstruction in 9 cases (90%). In the newborns with absence of RIAR, the anomanometric controls have allowed to know the moment of the maturation of the anorectal function.

Cierre quirúrgico del conducto arterioso en el pretérmino extremo o muy extremo / Surgical closure of ductus arteriosus in the extreme or very extreme preterm infant

Objetivos. El cierre quirúrgico del conducto arterioso en pretérminos extremos o muy extremos presenta dificultades, tanto por su anestesia e intervención como por las circunstancias clínicas que suelen acompañarle. Revisamos nuestra experiencia con el objeto de conocerlos factores que más han contribuido a los resultados obtenidos. Métodos. En todos los pacientes revisados, valoramos: edad gestacional; peso al nacimiento; tratamiento farmacológico; cierre quirúrgico; incidencia de displasia broncopulmonar, insuficiencia renal y enterocolitis necrosante; tiempo de ingreso; mortalidad y sus causas. Resultados. Constituyen la muestra 26 prematuros de edad gestacional media de 26,24 semanas y peso medio de 885 g. Se trataron con Indometacina 22, a una edad media de 7 días y mediante ligadura (..) (AU)

Objectives. The surgical close of patent ductus arteriosus in the extreme or very extreme premature presents difficulties, because of its anaesthesia, surgical intervention and the clinical circumstances that usually accompany this pathology. We reviewed our experience in order to understand the factors that have contributed to the obtained results. Methods. In all the reviewed patients we evalue: gestational age, birth weight, drug treatment, surgical close, incidence of broncho pulmonary dysplasia, necrotizing enterocolitis and renal failure, time of (..) (AU)

La ecografía fetal en el segundo trimestre: interés para el cirujano pediatra / The fetal ultrasonography in the second quarter: Interest for the pediatric surgeon

Introducción. El cirujano pediatra debe conocer los detalles del diagnóstico ecográfico prenatal para participar en el asesoramiento a los padres a efectos de continuar o no el embarazo, y planificar el tratamiento pre y postnatal. Los objetivos de nuestro estudio son: conocer la incidencia de las malformaciones congénitas detectadas con la ecografía del2º trimestre, cuántos de estos diagnósticos se confirman al nacimiento y cuál es la utilidad de esta prueba a la hora de informar a los padres. Pacientes y métodos. Se revisan los informes ecográficos prenatales del 2º trimestre realizados entre enero de 2005 y julio de 2009,recogiendo los hallazgos ecográficos y la edad materna y gestacional. A continuación, se anotan la evolución de las alteraciones de los fetos y el número de abortos espontáneos y voluntarios. Por último, se comprueba el diagnóstico en (..) (AU)

Introduction. The paediatric surgeon should know the details of prenatal ultrasound diagnosis to participate in advising parents about the continuation of the pregnancy, and to plan the prenatal and postnatal treatment. Our objectives are: to determine the incidence of congenital anomalies detected with ultrasound in the 2 nd trimester, the number of these diagnoses which is confirmed at birth and what is the usefulness of this test when advising parents. Patients and methods. We reviewed the prenatal ultrasound in 2ndtrimester reports made from January 2005 to July 2009. We note the ultrasound findings, the maternal and gestational age. The evolution of anomalies of the fetuses and the number of spontaneous and volunteers abortions are noted. Finally, the diagnoses are checked in the newborns. Results. 10,256 ultrasonographies are made in this period. 209 stories of pregnant women (2%), which present fetal pathology amenable (..) (AU)

[Familial adenomatous polyposis: a follow-up of the extracolonic manifestations]. / Poliposis adenomatosa familiar: seguimiento de las manifestaciones extracolónicas.

INTRODUCTION: Total colectomy is the only effective treatment for prophylaxis against colon cancer in patients with familial adenomatous polyposis (FAP). We present our experience with 4 children colectomized for FAP, with a particular focus on the long-term surveillance to detect extracolonic manifestations. PATIENTS AND METHODS: We review the clinical histories of 4 patients from 10 to 16-years-old, with family histories of FAP. Performed for each patient were: genetic testing, colonoscopy, double contrast enema, gastro-oesophageal duodenoscopy, thyroid and abdominal ultrasound, fundus oculi, and tumour markers (CEA, CA 19.9). They underwent total colectomy with ileoanal anastomosis and anorectal mucosectomy with an ileal J-pouch. During follow-up, they were monitored regularly with imaging techniques (including a video capsule in one patient) and endoscopy. Also evaluated were faecal continence, food intake, and height-weight development. RESULTS: All had multiple polyps in the colon, and mutation of the APC gene. Hypertrophy of the retinal pigment epithelium was observed in three. Immediately postoperative, there were abundant diarrhoeic stools, two presented an episode of "pouchitis", and one moderate undernourishment. One patient had an intraperitoneal haemorrhage that was resolved by blood transfusion. All 4 have normal faecal continence. During the first months postoperative, two patients showed considerable weight loss. In the follow-up (> 3 years), moderate undernourishment was observed in one patient. Duodenal polyps were found in two patients--in one by duodenoscopy, and in the other with the video capsule. CONCLUSIONS: After colectomy, FAP patients may develop extracolonic clinical manifestations, some of which may be malignant such as thyroid and periampullary cancer. Careful surveillance of these patients is therefore very important, with annual checks using gastroduodenoscopy and thyroid and abdominal ultrasound.

[Anorectal manometry in the neonatal diagnosis of Hirschsprung's disease]. / La manometría anorrectal en el diagnóstico neonatal de la enfermedad de Hirschsprung.

OBJECTIVE: To determine the usefulness of anorectal manometry (AM) in neonatal screening for Hirschsprung's disease (HD). MATERIAL AND METHODS: We review the anomanometric studies of 98 newborn (63 males and 35 females) with clinical suspicion of HD, noting the indications of AM, gestational age, weight, age in days at the test, and whether or not other diagnostic methods were performed. Studied at rest were the pressures (mmHg) in the rectal ampulla (RA), proximal anal canal (PAC), and distal anal canal (DAC), and, in the stimulation phase, the presence or absence of a recto-anal inhibitory reflex (RAIR). In patients with no RAIR, the study was repeated weekly until the end of the first month, and if there was still no RAIR a suction rectal biopsy (SRB) was performed. In these patients and those with signs of intestinal obstruction, a contrast enema was carried out. RESULTS: The indications of AM are: delayed meconium passage, abdominal distension, and/or vomiting in 61.5% of the patients, intestinal obstruction in 16%, constipation in 15.1%, and other causes in 7.4%. The mean gestational age was 35.59 +/- 4.59 weeks, and the weight 2518 +/- 912.91 g. The mean age at the first test was 15.08 +/- 11.33 days. A RAIR was observed at the first study in 65 patients (healthy 54, meconium plug 2, meconium ileus 2, intestinal neuronal dysplasia 1, false negative 1, and other diagnoses 6), and was absent in 27 (HD 20, small left colon syndrome 4, hypoganglionism 1, and false positives 2). In 6 patients the first study was considered invalid. Histological studies confirmed HD in 21 newborn, in 11 of whom the contrast enema showed a transition zone. There were no differences between healthy and HD newborn in the RA, PAC, or DAC resting pressures. The sensitivity of AM for the diagnosis of HD was 95% and its specificity 90.24%. The sensitivity and specificity of rectal biopsy were 100%. The sensitivity of contrast enema was 52.3%, and its specificity 78.6%. CONCLUSIONS: AM is a simple and safe method with high sensitivity and specificity for the neonatal diagnosis of HD. We consider it indicated prior to SRB in every newborn with clinical suspicion of this disease.

[Digestive malformations and their associations to syndrome condition and genetic defects]. / Malformaciones digestivas y su asociación a patología sindrómica y defectos genéticos.

INTRODUCTION: The incidence of disease syndromes and genetic defects in patients with malformations is much higher than in the general population. We reviewed our experience in infants with gastrointestinal malformations to know the incidence of genetic defects and syndromic presentation, for purposes of carrying out the most complete assessment and treatment. MATERIAL AND METHODS: We recruited 161 patients with one or more malformations or congenital gastrointestinal pathology. We evaluated: type of malformation (isolated or syndromic), association with polimalformative complex, genetic testing and its results. RESULTS: The main diagnosis, from highest to lowest incidence, is intestinal malrotation (17.18%), Hirschsprung's disease (13.64%), Meckel's diverticulum (13.14%), oesophageal atresia (12.13%), anorectal malformation (11.12%), diaphragmatic hernia (6.57%), duodenal atresia (5.56%), small bowel atresia (4.55%), omphalocele (4.04), and other (12.07%). In 45.3% the malformation is not isolated. 27.4% appear as syndrome, 4.1% as development defect and 2.7% as association. Genetic study was performed in 30.43%, detecting defects in 36.7% of the studies. Down syndrome (n = 8) is the most frequent. DISCUSSION AND CONCLUSIONS: Our experience with these patients is similar to that provided in the literature. We consider that it is important to identify any associated anomalies, especially heart disease, craniofacial anomalies and other gastrointestinal malformations, because they condition the patient's management. Pathologies that are often associated with other defects require more effort for their detection. The knowledge of these patients is essential for correct treatment.

[Esophageal atresia in the Goldenhar syndrome]. / Atresia de esófago en el síndrome de Goldenhar.

Among the multiple congenital defects associated to esophagueal atresia, the characteristic ones of the Goldenhar syndrome usually are not included. The high incidence has been reported, about 5% of esophagueal atresia in patients with Goldenhar syndrome. Our experience includes two patients with this association who presented anesthetic problems and surgical complications associated with gastroesophageal reflux and esophageal anastomosis.

Poliposis adenomatosa familiar: seguimiento de las manifestaciones extracolónicas / Familial adenomatous polyposis: a follow-up of the extracolonic manifestations

Introducción. La colectomía total es el único tratamiento eficaz para prevenir el cáncer de colon en pacientes con Poliposis Adenomatosa Familiar (PAF). El objetivo es exponer nuestra experiencia con 4niños colectomizados por PAF con especial atención al seguimiento a largo plazo para la detección de manifestaciones extracolónicas. Pacientes y métodos. Revisamos las historias clínicas de 4 pacientes de entre 10 y 16 años, con antecedentes familiares de PAF. En cada paciente se realiza: estudio genético, colonoscopia, enema de doble contraste, esófago-gastro-duodenoscopia, ecografías abdominal y tiroidea, fondo de ojo y marcadores tumorales (CEA, Ca 19.9). Se les practica (..) (AU)

Introduction. Total colectomy is the only effective treatment for prophylaxis against colon cancer in patients with familial adenomatous polyposis (FAP). We present our experience with 4 children colectomized for FAP, with a particular focus on the long-term surveillance to detect extracolonic manifestations. Patients and methods. We review the clinical histories of 4 patients from 10 to 16 years old, with family histories of FAP. Performed for each patient were: genetic testing, colonoscopy, double contrast enema, gastro-œsophageal duodenoscopy, thyroid and abdominal ultrasound, fundus oculi, and tumour markers (CEA, CA 19.9). They underwent total colectomy with ileoanal anastomosis and anorectal mucosectomy with an ileal J-pouch. During follow-up, they were monitored regularly with imaging techniques (including a video capsule in one patient)and endoscopy. Also evaluated were (..) (AU)

La manometría anorrectal en el diagnóstico neonatal de la enfermedad de Hirschsprung / Anorectal manometry in the neonatal diagnosis of hirschsprung's disease

Objetivo. Es conocer la utilidad de la manometría anorrectal (MA)para el screening neonatal de la enfermedad de Hirschsprung (EH). Material y métodos. Se revisan los estudios anomanométricos de 98 neonatos (63 hombres y 35 mujeres) con sospecha clínica de EH. Se valoran las indicaciones de la MA, la edad gestacional, peso, días de vida al test y la realización o no de otros métodos diagnósticos. Se estudia, en reposo, las presiones (mmHg) en la ampolla rectal (AR), cana lanal proximal (CAP) y canal anal distal (CAD), y en la fase de estimulación se investiga la presencia o ausencia del reflejo inhibidor del (..) (AU)

Objective. To determine the usefulness of anorectal manometry(AM) in neonatal screening for Hirschsprung’s disease (HD). Material and methods. We review the anomanometric studies of 98 newborn (63 males and 35 females) with clinical suspicion of HD, noting the indications of AM, gestational age, weight, age in days at the test, and whether or not other diagnostic methods were performed. Studied at rest were the pressures (mmHg) in the rectal ampulla (RA), proximal anal canal (PAC), and distal anal canal (DAC), and, in the stimulation phase, the presence or absence of a recto-anal inhibitory reflex(RAIR). In patients with no RAIR, the study was repeated weekly until (..) (AU)

Malformaciones digestivas y su asociación a patología sindrómica y defectos genéticos / Digestive malformations and their associations to syndrome condition and genetic defects

Introducción. La incidencia de patología sindrómica y defectos genéticos en pacientes con malformaciones digestivas es muy superior a la de la población general. Revisamos nuestra experiencia en neonatos con malformaciones o patología digestiva congénita para conocer la incidencia en ellos de defectos genéticos y presentación sindrómica, a efectos de realizar su valoración y tratamiento más completos. Material y métodos. Se reclutan 161 pacientes atendidos por presentar este tipo de patología. Se valora: tipo de malformación (aisladao sindrómica), asociación a complejos polimalformativos reconocidos, realización de estudio genético y sus resultados. Resultados. El diagnóstico principal, de mayor a menor incidencia, es mal rotación intestinal (17,18%), enfermedad de Hirschsprung (..) (AU)

Introduction. The incidence of disease syndromes and genetic defects in patients with malformations is much higher than in the general population. We reviewed our experience in infants with gastrointestinal malformations to know the incidence of genetic defects and syndromic presentation, for purposes of carrying out the most complete assessment and treatment. Material and methods. We recruited 161 patients with one or more malformations or congenital gastrointestinal pathology. We evaluated: type of malformation (isolated or syndromic), association with polimalformative complex, genetic testing and its results. Results. The main diagnosis, from highest to lowest incidence, is (..) (AU)

Atresia de esófago en el síndrome de Goldenhar / Esophageal atresia in the goldenhar syndrome

Entre los múltiples defectos congénitos asociados a la atresia de esófago no suelen incluirse los característicos del síndrome de Goldenhar. Sí se ha comunicado la relativa alta incidencia, aproximadamente del 5%, de atresia de esófago en pacientes con síndrome de Goldenhar. Nuestra experiencia incluye dos pacientes con esta asociación que presentan problemas anestésicos y complicaciones quirúrgicas relacionados con la anastomosis esofágica y el reflujo gastroesofágico (AU)

Among the multiple congenital defects associated to esophagueal atresia, the characteristic ones of the Goldenhar syndrome usually are not included. The high incidence has been reported, about 5% of esophagueal atresia in patients with Goldenhar syndrome. Our experience includes two patients with this association who presented anesthetic problems and surgical complications associated with gastroesophageal reflux and esophageal anastomosis (AU)

[Surgical closure of ductus arteriosus in the extreme or very extreme preterm infant]. / Cierre quirúrgico del conducto arterioso en el pretérmino extremo o muy extremo.

OBJECTIVES: The surgical close of patent ductus arteriosus in the extreme or very extreme premature presents difficulties, because of its anaesthesia, surgical intervention and the clinical circumstances that usually accompany this pathology. We reviewed our experience in order to understand the factors that have contributed to the obtained results. METHODS: In all the reviewed patients we evalue: gestational age, birth weight, drug treatment, surgical close, incidence of bronchopulmonary dysplasia, necrotizing enterocolitis and renal failure, time of hospitalization, mortality and its causes. RESULTS: The sample is constituted of 26 prematures whose average gestational age is 26.24 weeks and average weight 885 g. 22 were treated with indomethacin, at an average age of 7 days, and all of them underwent surgical close, at an average age of 19 days (0-50). 10 patients suffered bronchopulmonary dysplasia, operated when they were 28 days of life. 7 suffered renal failure, 5 of them after drug treatment. 6 suffered necrotizing enterocolitis. 7 died (27%), 3 of them because of prematurity and preoperative severe pathology, 2 because of heart disease, and 2 because of severe necrotizing enterocolitis. CONCLUSIONS: Surgical ligation of patent ductus arteriosus in these patients is well tolerated, especially if the attention to prevent bronchopulmonary dysplasia, necrotizing enterocolitis and renal failure is optimized. Mortality in our series is not very different from that provided for the total of prematures with similar gestational age and birth weight, regardless of their pathology.

[The fetal ultrasonography in the second quarter: interest for the pediatric surgeon]. / La ecografía fetal en el segundo trimestre: interés para el cirujano pediatra.

INTRODUCTION: The paediatric surgeon should know the details of prenatal ultrasound diagnosis to participate in advising parents about the continuation of the pregnancy, and to plan the prenatal and postnatal treatment. Our objectives are: to determine the incidence of congenital anomalies detected with ultrasound in the 2nd trimester, the number of these diagnoses which is confirmed at birth and what is the usefulness of this test when advising parents. PATIENTS AND METHODS: We reviewed the prenatal ultrasound in 2nd trimester reports made from January 2005 to July 2009. We note the ultrasound findings, the maternal and gestational age. The evolution of anomalies of the fetuses and the number of spontaneous and volunteers abortions are noted. Finally, the diagnoses are checked in the newborns. RESULTS: 10,256 ultrasonographies are made in this period. 209 stories of pregnant women (2%), which present fetal pathology amenable to general surgical treatment, are accessible for study. These include: 182 urologic disorders (85.44%), 13 digestive disorders (6.1%), 6 thoracic disorders (2.8%), 6 multiple malformations (2.8%), 4 maxillofacial disorders (1.88%) a sacrococcygeal teratoma (0.47%), an umbilical cord cyst (0.47%), 7 pregnancies are spontaneous abortion and 7 are terminated voluntarily. 183 stories of newborns are reviewed, we can confirm 48 uropathies (26.37%), 4 digestive malformations (30.77%), 4 thoracic disorders (66.66%), 2 maxillofacial disorders (50%) and 1 teratoma. CONCLUSIONS: Ultrasonography in the 2nd trimester detects almost 2% of specific fetal malformations. False positives are common in all diagnostic groups. The number of abortions and the terminations of pregnancy is low, most of them are polymalformated fetuses. We believe that ultrasonography in the 20th week have low value to counsel the termination of pregnancy, because only 7 of the 160 terminations indicated in our hospital during the study period, correspond to fetuses with pathology detected in the 2nd trimester. We recognize the usefulness of this test but, the false positive and favorable developments in a high percentage of fetuses, make us to be cautious to advise the continuation of pregnancy and the indication of treatment.