[Transanal irrigations in patients with constipation and fecal incontinence: results, indications and follow up in our center]. / Irrigación transanal en pacientes con estreñimiento e incontinencia orgánica refractaria. Resultados, indicaciones y seguimiento en nuestro centro.

AIM OF THE STUDY: To present our results with transanal irrigation (TAI) for management of fecal incontinence and fecal constipation without response to other treatments. METHODS: Retrospective study of patients with fecal constipation and/or fecal incontinence treated with TAI between 2013 and 2017. A Rintala questionnaire(1) was carried out comparing pre and post-treatment results. This study was approved by the ethical committee. MAIN RESULTS: Twenty-five patients were included with a median age of 13 years old (range 6-44 years). Nineteen patients had spinal pathology (76%), four colorectal surgery (16%) and two functional constipation (8%). They presented fecal incontinence in 20% of cases, 12% of fecal constipation and 68% both conditions. After a mean follow-up of 1.5 years (1 month-4 years), 52% of the patients abandoned the treatment. The mean Rintala score was 6.8±4 before treatment, and after, resulted to 11.42±2.75 (p=0.001). The main complications throughout the treatment were pain (68%) and balloon leaks (28%). The patients declared as cause of treatment cessation: reduced mobility (15%), fear or misinformation (32%) and pain (76%). All patients with reduced mobility (n=3) left treatment, versus 45% (n=12) of the patients that had full mobility (p=0.17, OR 8.3 [IC95% 0.3-38]). Complementary treatments such as laxatives, enemas or digital extraction were abandoned in 55% of the patients. CONCLUSIONS: Using TAI seems to improve quality of life in patients with fecal constipation and fecal incontinence refractory compared to other treatments. Our abandonment rate was higher than expected, so we believe it is necessary to create a support group to improve follow-ups.

OBJETIVOS: Presentar nuestros resultados con el uso del irrigador transanal (IT) en el manejo intestinal de pacientes con incontinencia fecal o estreñimiento sin respuesta a otros tratamientos. METODOS: Estudio retrospectivo de pacientes con estreñimiento y/o incontinencia fecal refractaria tratados con IT (2013-2017) con el sistema Peristeen® (Coloplast). Se realizó cuestionario Rintala(1), comparando resultados pre y postratamiento. La realización del estudio fue aprobada por el comité de investigación clínica. RESULTADOS: Fueron incluidos en el estudio veinticinco pacientes, mediana de edad 13 años (6-44 años), cuatro no localizables. Diecinueve presentaban patología medular (76%), cuatro cirugías colorrectales (16%) y dos estreñimiento funcional (8%). El 20% aquejaba incontinencia, 12% estreñimiento y 68% ambas condiciones. Tras una mediana de seguimiento de 1,5 años (1 mes-4 años), el 52% abandonó el tratamiento. La puntuación media pretratamiento fue 6,8±4, frente a 11,42±2,75 tras su empleo (p=0,001), disminuyendo el número de escapes con repercusión positiva en actividades diarias. Las complicaciones principales fueron dolor (68%) y expulsión del balón (28%). Refirieron como causa de abandono: dificultad de uso por movilidad reducida (15%), miedo o desinformación (32%) y dolor (76%). El 100% de pacientes con movilidad reducida (n=3) abandonó el tratamiento, frente al 45% (n=12) en el resto (p=0,17, OR 8.3 [IC95% 0,3-38]). El 55% de los pacientes dejaron de utilizar laxantes, enemas o extracción digital. CONCLUSIONES: El uso de IT parece mejorar la calidad de vida en pacientes con estreñimiento e incontinencia de causa orgánica refractaria. La tasa de abandono fue mayor de la esperada, por lo que creemos necesaria la creación de un grupo de apoyo que mejore el seguimiento.

[Long-term renal function in Wilms tumor survivors]. / Función renal a largo plazo en supervivientes de tumor de Wilms.

OBJETIVE: To evaluate long-term renal function and morbimortality in non-syndromic Wilms tumor (WT) survivors. METHODS: Retrospective study about WT patients treated in 1993-2017, according to SIOP protocols. Mortality, glomerular filtration rate (GFR), prevalence of hypertension and requirement of dialysis and renal transplant were evaluated. Chronic kidney disease (CKD) was defined as GFR <90 ml/min/1.73 m2. RESULTS: Thirty-nine children were treated in the 25 analyzed years. Median time of follow-up was 6 years (0.5-21 years). 48% (19 patients) debuted with stage I or II. Four cases had high-grade histo-logy. Mortality rate was 10%. GFR data were found in 37 patients. Chronic kidney disease (grade I-II) turned up in 6 patients (16%). No patient required renal replacement therapy or renal transplant. 16% of patients developed CKD in both unilateral and bilateral WT, (p>0.05); OR 1.04 (IC 95% 0.09-10.9). Identical results were obtained comparing patients treated with or without radiotherapy (16%). Children with stage I-III had CKD in 11% vs. 40% of patients with stage IV (p=0.12); OR 5.3 (IC 95% 0.61-45). None of them presented hypertension in addition. CONCLUSIONS: In the current study the prevalence of CKD was low but not negligible, although no patients required renal replacement therapy or renal transplant. Bilateral renal involvement and radiotherapy were not associated with CKD development. Metastatic disease determines a higher risk of CKD.

OBJETIVOS: Evaluar la función renal y la morbimortalidad a largo plazo, en supervivientes de tumor de Wilms (TW) no sindrómico. MATERIAL Y METODOS: Estudio retrospectivo de pacientes con TW entre 1993-2017 tratados según protocolos SIOP. Evaluamos mortalidad, filtrado glomerular (FG), prevalencia de hipertensión arterial (HTA), necesidad de diálisis y trasplante renal. Se definió enfermedad renal crónica (ERC) como FG <90 ml/min/1,73 m2. RESULTADOS: En los 25 años analizados se trataron 39 pacientes con edad media diagnóstica de 3,6 años (0,3-11 años). Mediana de seguimiento 6 años (0,5-21 años). El 48% (19 pacientes) debutaron con estadio I o II. Cuatro pacientes presentaron histología de alto riesgo (10%). La mortalidad fue del 10%. El 16% (6 pacientes) desarrolló ERC (grados I-II). Ningún paciente precisó terapia renal sustitutoria (TRS) o trasplante. La presencia de ERC tanto en enfermedad unilateral como bilateral fue del 16%, p>0,05; OR 1,04 (IC 95% 0,09-10,9). Se obtuvieron idénticos resultados (16%) comparando pacientes que recibieron radioterapia frente a aquellos que no. Los pacientes en estadio I, II y III presentaron una prevalencia de ERC del 11% vs. 40% en estadio IV (p=0,12); OR 5,3 (IC 95% 0,61-45). Ningún paciente asoció HTA crónica. CONCLUSIONES: En el presente estudio la prevalencia de ERC en supervivientes de TW no sindrómico es baja pero no desdeñable, aunque ninguno precisó trasplante renal o TRS. La presencia de enfermedad bilateral y la radioterapia no se asociaron al desarrollo de ERC. La enfermedad metastásica condiciona un riesgo mayor de ERC.

[Our experience with sirolimus for the treatment of complicated vascular anomalies]. / Nuestra experiencia con la utilización de sirolimus en el tratamiento de anomalías vasculares complejas.

AIM OF THE STUDY: mTOR inhibitors are being used to treat complex vascular anomalies (VA) without response to conventional treatments. We report our results in pediatric patients treated with sirolimus. METHODS: Retrospective review of patients treated with sirolimus between 2014 and 2017, analyzing vascular anomaly type, treatment response and complications. Treatment protocol included patients with complex vascular anomalies, after signing the informed consent. The initial dose was 0.8 mg/m2/12 h, verifying plasmatic levels. Favorable response was defined both in clinical and radiological terms. MAIN RESULTS: Sirolimus was employed in nine patients, median age 14 months old (1 month-14 years), 66% girls. Five complex micro-cystic lymphatic malformations (LM), one multifocal lynphangioendotheliomatosis with thrombocytopenia, one kaposiform lymphangiomatosis, one lymphatic-venous malformation and one kaposiform hemangioendothelioma (KHE) were treated. Median treatment was 4 months (IQR 2-18 months). Resolution or improvement was objectified in four patients (44%). KHE patient presented complete resolution after two months of treatment. Two patients with micro-cystic LM and the one with lymphatic-venous malformation improved after a median treatment of three months. Two patients presented rebound effect after discontinuing treatment. Three patients had hypertransaminasemia and hypercholesterolemia without requiring medical treatment. CONCLUSION: Sirolimus presented mild effects for treatment of complex VA in our study, but was highly resolutive at KHE.

OBJETIVOS: Los inhibidores mTOR se están utilizando para el tratamiento de anomalías vasculares (AV) complejas sin respuesta a tratamientos convencionales. Presentamos nuestros resultados en pacientes pediátricos tratados con sirolimus. METODOS: Análisis retrospectivo de pacientes tratados con Sirolimus, entre 2014 y 2017, describiendo el tipo de anomalía vascular, respuesta al tratamiento y complicaciones derivadas de su empleo. Se incluyeron pacientes con anomalías vasculares complejas, tras firma del consentimiento informado y con dosis inicial de 0,8 mg/m2/12 horas, monitorizando niveles plasmáticos. Se definió respuesta favorable tanto en términos clínicos como radiológicos. RESULTADOS: Recibieron sirolimus 9 pacientes, mediana de edad de 14 meses (RIQ: 1 mes-14 años). El 66% fueron niñas. Se trataron 5 malformaciones linfáticas (ML) microquísticas complejas (en algunas como coadyuvante a otros tratamientos), 1 linfangioendoteliomatosis multifocal con trombopenia, 1 linfangiomatosis kaposiforme, 1 malformación mixta veno-linfática, 1 hemangioendotelioma kaposiforme (HEK). Mediana de tratamiento: 4 meses (2-18 meses). En 6 pacientes (66%) se objetivó resolución o mejoría de las lesiones. El paciente con HEK mostró resolución completa tras 2 meses de tratamiento. En dos pacientes con ML microquística y en el paciente con malformación mixta, se apreció mejoría clínica y disminución del tamaño de las lesiones tras una mediana de tratamiento de 3 meses. Se apreció efecto rebote en dos ML al suspender el tratamiento. Tres pacientes presentaron hipertransaminasemia e hipercolesterolemia, sin precisar tratamiento médico. CONCLUSIONES: En nuestro estudio, objetivamos que el sirolimus tuvo una eficacia moderada en el tratamiento de AV complejas, pero fue resolutivo en el HEK.

[Quality of life and long term results in Hirschsprung's disease]. / Calidad de vida y resultados a largo plazo en pacientes con enfermedad de Hirschsprung.

INTRODUCTION: The most frequent long term side effects of Hirschsprung's disease (HD) surgery are obstructive problems and fecal incontinence. In this study, we analyse long term functional results of the two most used surgical techniques (Duhamel-D- and De la Torre-dlT-) from the patient's point of view, through quality of life and functionality questionnaires. METHODS: We selected short segment HD patients ≥ 4 years) that were operated in our unit from 1996 until 2011. We employed two validated questionnaires: Bowel Function Score (BFS) and Gastrointestinal Quality of Life Index (GIQLI). Both questionnaires were also tested in two control age matched groups, each for every arm (controlD or control dlT). RESULTS: Among 28 preselected patients, 22 (78.6%) answered both questionnaires. Group D (10 patients) showed more constipation: 60% vs. 16.7% ( p = 0.01). Patients in group dlT (12 patients) showed more leaks: 58.3 vs. 10% (p = 0.03). Results of both questionnaires were higher (better) in Group D: 16 vs. 12.8 points (BFS, p = 0.007) and 74.1 vs. 69.8 (GIQLI, p = 0.17). The control group showed an overall better scores than HD patients: 17.2 vs. 14.3 (BFS p = 0.001) and 75.9 vs. 71.8 (GIQLI, p = 0.04). Separately, both groups of patients showed worse scores when compared with each control group. DISCUSSION: Our results are similar to other studies, where global scores of functional results and quality of life are worse in operated HD patients than in age matched controls. Fecal incontinence has more impact on social scores than constipation. Due to the fact that our dlT patients have more frequently fecal leaks, their scores are worse than in the D group. Nevertheless, patients in dlT group are younger and may improve their results as they get older, as it is often the case in operated HD patients.

[Functional results of Hirschsprung's disease patients after Duhamel and De la Torre procedures]. / Resultados funcionales en pacientes con enfermedad de Hirschsprung intervenidos mediante las técnicas de Duhamel y De la Torre.

INTRODUCTION: Long term results of different surgical techniques in Hirschsprung's Disease (HD) are contradictory. There are still no long term large or multicentric reports about functional results of De la Torre technique. We have studied the mid term functional results of the patients operated on Duhamel (D) and De la Torre (dlT) pull-through procedures. PATIENTS AND METHODS: We collected data from medical records and telephone interviews of the HD patients operated in our unit in the last 16 years. RESULTS: 38 patients were found. Ages ranged from 1.5 to 21 years. Mean age was 7.7 years. Median follow up was 5.9 years. 33 (86.8%) had rectosigmoid disease and 5 (13.2%) had long segment disease. D procedure was performed in 17 (44.7%), Soave in 1 (2.6%), Duhamel-Lester-Martin in 4 (10.5%) and dlT pull-through in 16(42%). In the last visit record, 12 (31.6%), had constipation, and fecal leaks were noted in 11 (33.3%) of the 33 patients > or = 4 years old.. 10 patients (29.4% of the > or = 4 years old group) referred encopresis along the follow-up. Patients from the D group referred higher rates of constipation than those in the dlT group (53.3% vs 20% p=0.048). dlT patients referred more frequency of leaks (46,1% vs 13,3%, p=0,05) Children with very short resections (< or = 10 cm) were more prone to constipation than children with longer resections (66,6% vs 17.4% p=0.007), and less prone to present leaks (12.5% vs 47.3% p=0.08). Encopresis was similar in all groups. DISCUSSION: Both techniques show similar functional results in the mid term, although children in the D group were more prone to constipation and those in the dlT group presented more fecal leaks. All patients with EH need long follow-ups.

[Rhabdomyomatous mesenchymal hamartoma]. / Hamartoma mesenquimal rabdomiomatoso.

Rhabdomyomatous mesenchymal hamartoma is a rare congenital lesion which consists of randomly arranged striated muscle fibers interspersed with mesenchymal elements. We describe the clinical and histopathological features of a rhabdomyomatous mesenchymal hamartoma in a one year-old patient presenting a bilobulated lesion in the mid-cervical line. No associated congenital malformations were observed.

[Median raphe cysts of the perineum in childhood]. / Quistes del rafe medio perineal en la infancia.

Median raphe cysts of the perineum are uncommon congenital lesions. Congenital lesions rarely encountered in daily clinical practice. Their diagnosis in childhood is particularly rare. We report two children with these cysts requiring surgical removal. We review the embryological, diagnostic and therapeutic aspects of these cysts, which could be of interest to pediatricians and pediatric surgeons when faced with this pathology in children.

[Clinical response of different colonic segments to chemical denervation: an experimental study]. / Respuesta clínica de distintos segmentos del colon a la denervación química. Estudio experimental en ratas.

An experimental study in rats with chemical myenteric plexus destruction of different segments of large intestine is presented. Forty Wistar rats weighing 200-250 g were used. Four groups of ten animals each were made. Group I: control; group II: myenteric denervation of 4 cm segment of rectosigmoid; group III: myenteric denervation of 4 cm segment of descending colon; group IV: myenteric denervation of 4 cm segment of transverse colon. Myenteric denervation was produced by serosal application of 0.1% benzalconium chloride during 30 minutes. Animals were evaluated by clinical parameters (survival, weight change, food intake, nutritional state), radiological (barium enema) and histomorphometry. Group IV showed the greatest mortality, nutritional deterioration and colonic obstruction while the results of group II are similar to control group.

[Efficacy of extramucosal myotomy in an experimental model of intramural colonic obstruction. An experimental study in rats]. / Eficacia de la miotomía extramucosa en un modelo experimental de obstrucción cólica intramural. Estudio experimental en ratas.

An experimental study over the efficacy of myotomy in the treatment of intramural colon obstruction is presented. Forty Wistar rats were used. Four groups were made with ten animals each: Group I: control; Group II: myenteric denervation of four cm segment of transverse colon; Group III: myenteric denervation of 4 cm segment of transverse colon and myotomy; Group IV: myotomy of segment of transverse colon. Myenteric denervation was produced by serosal application of 0.1% benzalkonium chloride during 30 minutes. Animals were evaluated by clinical parameters (survival, weight change, food intake, nutritional state), radiological (barium enema) and Histomorphometry. The myotomy is an effective technique for the treatment of the experimental model of colon obstruction used.

[Neoformation of intestinal mucosa on dura mater patches. Application in the surgical treatment of short bowel syndrome. Experimental study in rats]. / Neoformación de mucosa intestinal sobre parches de duramadre. Aplicación en el tratamiento quirúrgico del síndrome de intestino corto. Estudio experimental en ratas.

The creation of intestinal neomucosa on grafts or materials implanted in the intestinal wall is one of the therapeutic procedures that has been tested in the short bowel syndrome. This paper presents the results obtained after intestinal implanting lyophilised dural patches in an experimental model of massive intestinal resection in rats. Data were contrasted with those of a control group subjected to massive resection without patches. The findings reveal: 1) high mortality related to the surgical technique; 2) slight improvement in the ponderal curve; 3) slowing down of intestinal transit; 4) neomucosa on the patch, with histological characteristic similar to normal. We conclude that although the creation of neomucosa on dural patches is feasible and conditions a slight improvement in the animal's nutritive status, application of the procedure is not practical in treatment for the short bowel syndrome due to the high mortality rate.

[The surgical treatment of the short bowel syndrome by creating reservoir pouches. An experimental study on rats]. / Tratamiento quirúrgico del síndrome de intestino corto mediante la construcción de bolsas frenadoras. Estudio experimental en ratas.

Intestinal pouches are one of the techniques for the surgical treatment of the short bowel syndrome. In a experimental study with rats, the influence of the intestinal pouches in a model of 85-90% small bowel resection are investigated. The pouches improve the transit time of the resected animal. However, there is no improvement in the weight curves after extensive small bowel resection. This negative effect is due to the establishment of a blind loop syndrome at the level of the pouch. The intestinal pouches have no place in the surgical treatment of the short bowel syndrome.

[Participation of the colon in the compensatory process after massive intestinal resection. An experimental study in rats]. / Participacion del colon en el proceso de adaptacion compensadora tras la reseccion intestinal masiva. Estudio experimental en ratas.

The colon participation in the adaptation process after massive intestinal resection (R.I.M.) has been rarely studied. This is an experimental study in rats to know the morphological modifications to the colon after R.I.M. of small bowel. A total of 90 Wistar rats weighing 200-250 grams were used, in two groups. Twenty animals constituted group I (control) and 70 were submitted to resection of 85-90 per 100 of the small bowel (group II, R.I.M.). Radiologic and weights controls has been performed. To end of study (four months),a the animals were sacrificed to obtain histologic samples of the large bowel for structural and morphometric studies. The results of this study to confirm the colon participation in the compensatory adaptation process after R.I.M. of small bowel.

[Acute acalculous cholecystitis secondary to immersion asphyxia. Implications in organ donation]. / Colecistitis aguda acalculosa secundaria a asfixia por inmersion. Implicaciones en la donacion de organos.

A case of acute acalculous cholecystitis in a two year-old girl that undergone drowning in sweet water is reported. Cerebral death was diagnosed and the organs donation was authorized. A biliary peritonitis secondary to gallbladder perforation was met during organs extraction. The liver was rejected.

[Segmentary agenesis of the intestinal muscle layer: a rare cause of intestinal obstruction. A case report]. / Agenesia segmentaria de la capa muscular intestinal: una causa rara de obstrucción intestinal. Presentación de un caso.

A very rare case of intestinal obstruction due to segmental agenesis of musculature of the small intestine is presented. In reviewing the literature, only nine cases of this entity has been published. Generally, the clinical evolution make necessary to perform several laparotomies, but the diagnosis is made by pathological findings. The prognosis is very poor.

[The neoformation of intestinal mucosa on dura mater patches. An experimental study in rats]. / Neoformación de mucosa intestinal sobre parches de duramadre. Estudio experimental en ratas.

The technics of growing of small bowel neomucosa is one of the possible surgical therapies for the treatment of the short bowel syndrome. We carried out an experimental study in rats implanting patches and tubes of dura mater in the antimesenteric border of intestine. The growth of normal neomucosa is demonstrated. The animal tolerance to the dura mater patch is established in 3-4 cms. We also have proved that dura mater tubes are inefficacious. The dura mater is a good support for the growth of the small bowel neomucosa; however, it's necessary to prove their utility in the surgical treatment of short bowel syndrome.