¿Se cumplen los tratamientos fisioterapéuticos infantiles? La importancia del profesional sanitario / Is there compliance in child physiotherapy treatments? The importance of the health care professional

Objetivos: Estimar el grado de adhesión terapéutica al tratamiento rehabilitador en enfermedad motora del desarrollo de niños entre 0 y 6 años e identificar las variables psicosociales predictoras de la misma. Método Estudio transversal retrospectivo en el que se entrevistó a todos los cuidadores principales de los niños que asistían a tratamiento fisioterapéutico en el Servicio de Fisioterapia del Hospital General Universitario de Alicante y el Centro de Atención Temprana de Alicante. En total, se realizaron 88 entrevistas, de las que solo se analizaron 79 por errores en la cumplimentación. Las variables psicosociales evaluadas fueron: apoyo social, confianza hacia el profesional, autoeficacia percibida y ansiedad. Resultados El modelo de regresión logística binaria realizado para pronosticar la probabilidad de adhesión al tratamiento por el cuidador principal indicó que en un 86,7% de los casos la adhesión al tratamiento estuvo pronosticada por el grado de confianza que los padres tienen en los fisioterapeutas, así como por su percepción de apoyo social. No resultó estadísticamente significativo para otras variables (autoeficacia percibida, ansiedad o las variables sociodemográficas).Conclusiones Este estudio muestra la importancia de variables relacionadas con el entorno socioprofesional frente a variables individuales en la adhesión al tratamiento rehabilitador. Entre ellas, destaca el papel del fisioterapeuta como motivador de conductas de adhesión en los padres o responsables de los niños. Este resultado subraya la necesidad de formación de los fisioterapeutas en habilidades de comunicación y apoyo emocional para mejorar la implicación de los adultos en este tipo de tratamientos para niños con afección del desarrollo

Objectives: To estimate the level of patient compliance regarding the rehabilitation treatment in 0 to 6 year old children with developmental motor conditions and to identify the predicted psychosocial variables. Method: A retrospective, cross-sectional study was performed by interviewing the caregivers of children undergoing physiotherapy in the General University Hospital of Alicante and the Center for Early Intervention in APSA Alicante, Spain. A total of 88 interviews were performed, 79 of which were analyzed and 9 of which were discarded due to errors in filling out the questionnaires. The psychosocial variables evaluated were social support, confidence in the professional, perceived self-efficacy and anxiety. Results: The binary logistic regression model performed to predict the probability of patient compliance perceived by the principal caregiver indicated that in 86.7% of the cases, patient compliance was predicted by the degree of confidence the parents had in the physiotherapist as well as their perception of social support. No statistically significant results were detected in the other variables (perceived self-efficacy, anxiety or sociodemographic variables).Conclusions: This study has demonstrated the importance of the variables related to the social-professional environment versus the individual variables in the rehabilitating treatment compliance. Of these, it is important to note the role of the physiotherapist as a motivator towards compliance behaviors in the parents or tutors. This results emphasizes the need to educate physiotherapists in communication skills and emotional support in order to improve the involvement of the adults in these types of treatments for children with developmental conditions

Acumulación de neonatos con malformación anorrectal en 2011. ¿Solo azar? / Increase of newborns with anorectal malformation in 2011. Is it just by chance?

Introducción. Durante 2011 hemos diagnosticado 9 pacientes con malformaciones anorrectales (MAR). Esto supone 13.3 casos/10.000 RN vivos, frente a la incidencia estimada de 2 casos/10.000 RN vivos. Esta acumulación de MAR puede no ser debida al azar, por lo que hemos decidido su investigación. Pacientes y Métodos. Incluimos en el estudio todos los pacientes con MAR nacidos en el 2011. En todos ellos analizamos: su área geográfica, edad y estado de salud de los padres, gestación, exposición ateratógenos (especialmente, ingesta de lorazepan), sexo, edad gestacional, peso al nacimiento, exploración abdominal y del periné, defectos congénitos asociados, estudios por imagen realizados, tratamiento y situación actual. Resultados. Todos los pacientes proceden de nuestra provincia, yla edad materna es menor de 20 años en 2, o mayor de 30 en 7; existe predominio de varones (8/1) y MAR de localización baja en 4 de los9 pacientes. El 100% de los RN presentan algún tipo de malformación asociada en los genitales, en el sistema urinario o costovertebrales. La valoración clínica y el estudio por imagen proporcionaron el diagnóstico correcto en todos los casos. Hay que destacar la ausencia de dificultades en el tratamiento quirúrgico y la evolución clínica satisfactoria en todos los niños. Conclusiones. A la acumulación de casos con MAR se suman el predominio en varones así como la alta incidencia de malformaciones asociadas y de MAR de localización alta. No hemos encontrado una explicación epidemiológica a esta concentración de pacientes con MAR. Esto nos obliga a continuar con nuestra investigación por si se detecta alguna causa, de momento no conocida (AU)

Objective. In 2011 we have diagnosed 9 anorectal malformations(MAR). This means 13.3 cases/10.000 NB versusu the estimated incidence of 2 cases/10.000 NB. This accumulation of defects can not bedue to chance, so we have decided to focus on its research. Material and Methods. The study included all the patients with anorrectal malformations born in 2011. In all of them we analyzed: geographic area, age and health status of parents, pregnancy, exposureto teratogens, especially lorazepam intake, sex, gestational age, weight at birth, exploration of the abdomen and perineum, birth defects associated, imaging studies performed, treatment and current situation. Results. All patients came from our province, and maternal age is under 20 years in (..) (AU)

[Increase of newborns with anorectal malformation in 2011. Is it just by chance?]. / Acumulación de neonatos con malformación anorrectal en 2011. Solo azar?

OBJECTIVE: In 2011 we have diagnosed 9 anorectal malformations (MAR). This means 13.3 cases/10.000 NB versusu the estimated incidence of 2 cases/10.000 NB. This accumulation of defects can not be due to chance, so we have decided to focus on its research. MATERIAL AND METHODS: The study included all the patients with anorrectal malformations born in 2011. In all of them we analyzed: geographic area, age and health status of parents, pregnancy, exposure to teratogens, especially lorazepam intake, sex, gestational age, weight at birth, exploration of the abdomen and perineum, birth defects associated, imaging studies performed, treatment and current situation. RESULTS: All patients came from our province, and maternal age is under 20 years in 2 patientes and older than 30 in 7; male-dominated (8/1); low forms in 4 of 9 patients. 100% of newborns have some type of associated malformation, in genitals, urinary system or costovertebral. Clinical assessment and imaging study provided the right diagnosis in all the cases. It must be noted the absence of difficulties in the surgery treatment and a favorable outcome in all children. CONCLUSIONS: To this accumulation of cases with anorrectal malformation cases it is added a male predominanced, and a high incidence of associatedmalformations and high defects. We have not found an epidemiological explanation for this concentration of patients with anorrectal malformations. This forces us to continue our investigation if detected for some reason, currently not known.

[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.

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)

Determination of the anal position in newborns and in children with chronic constipation: comparative study in two European healthcare centres.

INTRODUCTION: The objective of this study was to determine the normal anal position index (API) in neonates and in a group of older children with chronic constipation, and to identify the incidence of anterior ectopic anus (AEA) in the two groups. MATERIALS AND METHODS: A prospective random study was carried out in two European hospitals determining the API in 1,012 neonates (529 in Group A, and 483 in Group B) and in 64 children suffering chronic constipation of ages between 3 months and 12 years (Group C). The API is defined as the ratio between the fourchette-anal and fourchette-coccygeal distances in girls and the scroto-anal and scroto-coccygeal distances in boys. The gestational age, weight, meconium evacuation and anal calibre were recorded in Groups A and B, and age, sex, and API in Group C. Statistical comparisons were made using Student's t test and Welch's t test for independent sample groups. RESULTS: The mean API in Group A was 0.53 ± 0.06 in males and 0.40 ± 0.05 in females, and in Group B 0.51 ± 0.06 in males and 0.39 ± 0.08 in females, with significant differences between the sexes (p < 0.0001) in both groups; 1.7% of the neonates in Group A and 2.4% of Group B presented AEA. 4/13 newborns females with AEA presented with severe constipation during their first months of life. For the 64 patients in Group C: 59.3% were female; the mean API was 0.47 ± 0.1 in males and 0.36 ± 0.1 in females (p < 0.0001). The incidence of AEA was 47% in females and 35% in males. CONCLUSIONS: In the neonates (Groups A and B), the position of the anus in the perineum was more anterior in females than in males (p < 0.0001); 31% of the newborns females with AEA presented with constipation in their first months of life. In the Group C patients, there was a high incidence of AEA, especially in the females, and we consider it to be the probable cause of this defecation disorder.

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)

[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.

[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.

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)

Repercusión de distintos tipos de colectomía sobre el estado de nutrición y tipo de heces / Repercussion of different types of colostomy on the nutrition condition and type of stools

Introducción. Las colectomías se consideran, en general, procedimientos no causantes de problemas graves de morbimortalidad. No parece lógico que la pérdida total o parcial del colon, cuya anatomía y función altamente diferenciadas son reconocidas, debiera tolerarse sin repercusiones detectables clínicamente, aún por procedimientos simples ,como los utilizados en este estudio. Pacientes y métodos. Se revisan 31 pacientes sometidos a colectomía en nuestro centro, una media de 67.26 meses antes del presente estudio. Se dividen en 3 grupos: 14 pacientes sometidos a reseción de la unión ileocecal (RUIC), 7 a colectomía total (CT) y 10 a colectomía distal (CD). Se valora en todos los pacientes la frecuencia y consistencia de las deposiciones, así como el crecimiento corporal (peso, talla e índice nutricional) y la composición corporal (pliegue tricipital, perímetro braquial y porcentaje de grasa corporal).Resultados. La mayoría de pacientes sometidos a RUIC o a CT (..) (AU)

Introduction. Colectomies are considered, in general, not causing of serious problems of morbidity and mortality. It does not seem logical that the total or partial loss of the colon, whose anatomy and function are highly differentiated recognized, should be tolerated without clinically detectable impact, even for simple procedures such as those used in this study. Patients and methods. We have reviewed 31 patients undergoing colectomy in our center, an average of 67.26 months before the present study. They are divided into 3 groups: 14 patients undergoing resection of ileocecal junction (RUIC), 7 to total colectomy (CT) and 10 to distal colectomy (CD). We have studied the frequency and consistency of depositions in all patients, as well as body growth (weight, height and nutritional index) and body composition (triceps skinfold, brachial (..) (AU)

[Familial adenomatous polyposis: Gardner's syndrome]. / Poliposis adenomatosa familiar: síndrome de Gardner.

Familial adenomatous polyposis (PAF) associated to soft tissue tumors or osteomas constitutes the Gardner's syndrome of autosomal dominant inheritance. The risk of colorectal cancer in these patients is 100%. We present a patient with Gardner's syndrome who was had colectomy at early age. An eleven years old boy he was evaluated due to a family history of PAF and subcutaneous tumors (occipital and left thigh). Genetic profile shows a mutation in gene APC and the colonoscopy confirms the polyposis; the biopsy also suggested moderate dysplasia. When the patient reached the age of twelve, a total colectomy with colorectal mucosectomy was performed. Cystic subcutaneous lesions (epidermoid cysts) were also excised. In the postoperative period there were no complications. The prophylactic colectomy, is the only effective treatment to prevent the colorectal cancer. Gardner's syndrome patients requires periodic controls to rule out the appearance of new tumors or anomalies in the retine. The duodenoscopy is essential in the follow up of these patients because of the frequency of duodenal affectation.

Poliposis adenomatosa familiar: síndrome de Gardner / Familial adenomatous polyposis: Gardner´s syndrome

La asociación de poliposis adenomatosa familiar (PAF) con tumores de tejidos blandos u óseos constituye el síndrome de Gardner, de herencia autosómica dominante. Estos pacientes tienen un riesgo de cáncer de colon de un 100%. Se presenta un paciente con síndrome de Gardner al que se le practicó colectomía a una edad temprana. Niño de 11 años, remitido por antecedentes familiares de PAF y tumoraciones subcutáneas (occipital y en muslo izquierdo). El estudio genético demuestra mutación del gen APC y la colonoscopia confirma la poliposis, observando en la biopsia displasia moderada. A los 12 años se practica colectomía total con mucosectomía y exéresis de las lesiones subcutáneas (quistes epidermoides). El postoperatorio cursa sin complicaciones. La colectomía profiláctica es el único tratamiento eficaz para prevenir el cáncer de colon. Estos pacientes requieren controles periódicos para descartar la aparición de nuevas tumoraciones o anomalías en la retina. La duodenoscopia es imprescindible en el seguimiento de estos pacientes dada la frecuente afectación duodenal (AU)

Familial adenomatous polyposis (PAF) associated to soft tissue tumors or osteomas constitutes the Gardner´s syndrome of autosomal dominant inheritance. The risk of colorectal cancer in these patients is 100%. We present a patient with Gardner´s syndrome who was had colectomy at early age. An eleven years old boy he was evaluated due to a family history of PAF and subcutaneous tumors (occipital and left thigh).Genetic profile shows a mutation in gene APC and the colonoscopy confirms the polyposis; the biopsy also suggested moderate dysplasia. When the patient reached the age of twelve, a total colectomy with colorectal mucosectomy was performed. Cystic subcutaneous lesions (epidermoid cysts) were also excised. In the postoperative period there were no complications. The prophylactic colectomy, is the only effective treatment to prevent the colorectal cancer. Gardner’s syndrome patients requires periodic controls to rule out the appearance of new tumors or anomalies in the retine. The duodenoscopy is essential in the follow up of these patients because of the frequency of duodenal affectation (AU)

Utilidad de la manometría anorrectal para el diagnóstico y tratamiento de la encopresis / Utility of anorectal manometry in the diagnosis and treatment of encopresis

Antecedentes: La retroalimentación biológica (RAB), basada en procedimientos anomanométricos, ha mostrado su eficacia en el tratamiento de niños con encopresis secundaria. Se discute cuáles son sus indicaciones, qué variables de la manometría anorrectal podrían ayudar a establecerlas y si los buenos resultados de la RAB se mantienen a largo plazo. Objetivos : Conocer qué variables anomanométricas, además de las manifestaciones clínicas, pueden ser útiles para decidir qué pacientes podrían beneficiarse de la RAB, y los resultados de éste. Pacientes y métodos: Se realizó manometría anorrectal a todos los pacientes (n=88) remitidos por presentar eliminación incontrolada de heces tras un año o más de continencia normal, sucedida al menos una vez al mes durante al menos 6 meses, edad cronológica y mental igual o mayor de 4 años, ausencia de alteraciones psíquicas u orgánicas que la justifiquen y ausencia de respuesta al tratamiento médico. Las variables consideradas fueron las relacionadas con: perfil del canal anal, reflejo inhibidor del ano, reflejo de continencia, sensibilidad rectal, actividad del esfínter externo y maniobra defecatoria. Los pacientes se dividieron en dos grupos, según su afectación clínica y anomanométrica, y se sometió a RAB a los más afectados (n=41). Se valora la indicación y resultados de la RAB mediante evolución clínica y manometría anorrectal. Para el análisis estadístico se utilizaron el cálculo de la media y desviación estándar (DE); prueba de la chi cuadrado (X2 ) con corrección de Yates para la comparación de las variables cualitativas clínicas y anomanométricas; t de Student para la comparación de las variables cuantitativas; como pruebas no paramétricas la de Mann-Witney y, en caso de muestras apareadas, la de Wilcoxon. Resultados: En los pacientes sometidos a RAB se observó: tendencia a menor longitud del canal anal, presiones mayores en ampolla rectal (p < 0,001) y menores en canal anal (p < 0,05), tendencia a menor distensibilidad de la actividad del esfínter externo al provocar el reflejo inhibidor del ano, menor presencia del reflejo de continencia (p < 0,01), tendencia a menor sensibilidad rectal, y peor respuesta de la musculatura esfinteriana estriada y de la maniobra defecatoria (p < 0,001). El 78 por ciento de los pacientes tuvieron buena respuesta a la RAB, mejorando sus presiones en canal anal y la sensibilidad rectal (p < 0,001), con normalización en 11 de 15, de la maniobra defecatoria. Estos buenos resultados se mantuvieron en los 10 pacientes controlados a largo plazo, y la encopresis seguía en 8 de 10 pacientes controlados no sometidos a RAB (p < 0,001). Conclusiones: La manometría anorrectal detecta alteraciones, sobre todo en la función de la actividad del esfínter externo, útiles para la indicación de RAB en niños con encopresis secundaria. La RAB es capaz de curar a la mayoría de los pacientes de mayor gravedad, persistiendo su efecto a largo plazo (AU)

[Utility of anorectal manometry in the diagnosis and treatment of encopresis]. / Utilidad de la manometría anorrectal para el diagnóstico y tratamiento de la encopresis.

BACKGROUND: Biofeedback based on anomanometric techniques has been shown to be effective in the treatment of children with encopresis. The long-term efficacy of biofeedback and which variables of anorectal manometry (anorectal manometry) could help to establish biofeedback indications are currently the subject of debate. OBJECTIVES: To identify which variables of anorectal manometry, in addition to symptoms, could be useful in deciding which patients could benefit from biofeedback therapy and to assess the outcome of this treatment. PATIENTS AND METHODS: Anorectal manometry was performed in 88 patients, who were referred to our service complaining of soiling at least once a month for a minimum of 6 months after a period of normal continence of 1 year or more. The chronological and mental age of the patients was 4 years. All patients were otherwise in good health and had shown no response to medical treatment. The following variables were studied: anal canal profile, rectoanal inhibitory reflex (RAIR), continence reflex, rectal sensitivity, external anal sphincter (EAS) activity and defecatory maneuver. The patients were divided into two groups, according to clinical and anomanometric impairment, and the most affected patients (n = 41) underwent biofeedback therapy. The indications and outcome of biofeedback were assessed through clinical course and anorectal manometry. In the statistical analysis, the mean and standard deviation were calculated. The chi-squared test with Yates' correction was used to compare clinical and manometric qualitative parameters; Student's t-test was used to compare quantitative parameters; nonparametric tests consisted of the Mann-Whitney test and the Wilcoxon test was used for paired data. RESULTS: Patients treated with biofeedback therapy presented shorter anal canal, greater pressure in the rectal ampulla (P < 0.001), decreased pressure in the anal canal (P < 0.05), lesser distension of the EAS on provoking RAIR, lower presence of the continence reflex (P < 0.01), lower rectal sensitivity, and a worse response of the striated sphincteric muscle and of the defecatory maneuver (P < 0.001). Seventy-eight percent of the patients had a good response to biofeedback therapy. Pressure in the anal canal and rectal sensitivity improved (P < 0.001) with normality on straining in 11 out of 15 patients. These good results persisted in a long-term follow-up of 10 patients. Eight of 10 patients who did not undergo biofeedback therapy showed persistent encopresis (P < 0.001). CONCLUSIONS: Anorectal manometry detected disturbances, chiefly in the activity of the EAS, which are useful in indicating biofeedback therapy in children with secondary encopresis. Biofeedback therapy seems to produce favorable long-term results in the majority of the most severely affected patients.

[Re-education of the anal sphincter in patients with myelomeningocele]. / Reeducación del esfínter anal en el mielomeningocele.

OBJECTIVE: The aim of this study was to analyze the usefulness of biofeedback techniques based on manometrical procedures in the treatment of defecation disorders in patients with myelomeningocele. PATIENTS AND METHODS: We studied 24 patients with myelomeningocele, aged 13 days to 15 years, who were referred to our Instrumental Diagnosis Unit for evaluation of anorectal function, or constipation and/or fecal incontinence study. Biofeedback was given to 12 patients who fulfilled the following criteria: age more than 5 years, existence of anorectal sensitivity with a perceptual threshold of less than 60 ml of rectal expansion, ability to contract and relax the gluteus muscles and thus be able to exercise pressure on the anal margin, and collaboration on the part of child and the family. As a complement defecatory maneuvers were performed. RESULTS: Between 4 and 65 sessions took place (35.14 19.97) after which rectal perceptual volume decreased from 24.66 14.13 to 3.33 2.95 ml and pressure on the anal margin increased from 45.62 17.82 to 114.37 13.99 mmHg. These differences were statistically significant (p < 0.001). Clear clinical improvement was produced in 10 of 12 patients who achieved good fecal continence with spontaneous depositions. Partial improvement was produced in 2 patients. CONCLUSIONS: We consider that biofeedback based on manometrical procedures is more useful than other conservative therapies in the treatment of defecatory problems in patients with myelomeningocele. In most patients this technique produces clinical improvement with satisfactory continence and without the drawbacks of other procedures.

Reeducación del esfínter anal en el mielomeningocele / Reeducation of the anal sphincter in patients with myelomeningocele

Objetivo: Analizar la utilidad de las técnicas de biorretroalimentación basadas en procedimientos anomanométricos en el tratamiento de los trastornos de la defecación de los pacientes con mielomeningocele. Pacientes y método: Estudio de 24 pacientes afectados de mielomeningocele, con edades comprendidas entre 13 días y 15 años, remitidos a la Unidad de Diagnóstico Instrumental para valoración funcional anorrectal o estudio de estreñimiento y/o incontinencia. Se realizó biorretroalimentación a 12 pacientes que reunieron los criterios preestablecidos: edad superior a 5 años, existencia de sensibilidad anorrectal con un umbral de percepción menor de 60 ml de distensión rectal, habilidad del niño para contraer y relajar los músculos glúteos y de esta forma poder ejercer presión sobre el margen anal, colaboración por parte del niño y su familia. Como complemento se realizan maniobras defecatorias. Resultados: Se realizaron entre 4 y 65 sesiones (35,14 ± 19,97) tras las cuales se consiguió una disminución del volumen de percepción rectal de 24,66 ± 14,13 a 3,33 ± 2,95 ml y un aumento de la presión sobre el margen anal que pasó de 45,62 ± 17,82 a 114,37 ± 13,99 mmHg. Estas diferencias fueron estadísticamente significativas (p < 0,001). Así mismo, se consiguió una mejoría clínica clara en 10 de los 12 pacientes, que pasaron a tener una continencia fecal adecuada con deposiciones espontáneas y una mejoría parcial en dos pacientes. Conclusiones: Consideramos que la biorretroalimentación basada en procedimientos anomanométricos es un medio más útil que otras terapias conservadoras, para los problemas defecatorios de los pacientes afectados de mielomeningocele, puesto que consigue una mejoría clínica en la mayoría de los pacientes, con una continencia adecuada y sin los inconvenientes de otros procedimientos (AU)