Pediatric perianal Crohn's disease behavior in the era of biologic therapy. / Comportamiento de la afectación perianal en pacientes pediátricos con enfermedad de Crohn en la era de la terapia biológica.

AIM OF THE STUDY: To describe perianal Crohn's disease behavior and the role of biological therapy in a sample of pediatric patients. METHODS: A retrospective study of pediatric patients with Crohn's disease (CD) treated in our institution from 2017 to 2021, with a minimum follow up period of 6 months, was conducted. Patients were divided whether they had perianal disease (PD) or not. Baseline characteristics, extension of disease, growth failure rate, aggressive pattern rate, use of biological therapy and need for surgery, among other variables, were compared between both groups. Clinical and/or radiological improvement in the last 6 months of follow up was considered good control of PD. RESULTS: Seventy eight pediatric patients with CD were included. Median age at diagnosis was 10.5 years, and median follow up time was 3.8 years. 64.1% patients were male. Of all, 15 (19.2%) had perianal disease, of which 10 had fistulizing findings and 5 had non fistulizing findings. PD was presented at diagnosis in 8 patients, and the rest developed it in a median time of 1 year from diagnosis. PD was associated with growth failure (p = 0.003), use of biological therapies (p = 0.005), and need for second line of biologics (p = 0.005). Most patients (12/15, 80%) had good control of PD with the treatment received. CONCLUSIONS: CD patients with PD seem to need a more aggressive treatment, with biological therapies playing a key role for its handling nowadays. These patients require close nutritional evaluation that ensures proper development and growth.

OBJETIVO DEL ESTUDIO: Describir el comportamiento de la enfermedad de Crohn perianal y el papel de la terapia biológica en una muestra de pacientes pediátricos. METODOS: Estudio retrospectivo de pacientes pediátricos con enfermedad de Crohn (EC) tratados en nuestro centro entre 2017 y 2021, con un seguimiento mínimo de seis meses. Los pacientes se dividieron en función de si tenían enfermedad perianal (EP) o no. Se compararon entre ambos grupos las características iniciales, la extensión de la enfermedad, el índice de retraso en el crecimiento, el índice de patrón agresivo, el empleo de terapia biológica y la necesidad de cirugía, entre otras variables. Se consideró un buen control de la EP una mejoría clínica o radiológica en los 6 últimos meses de seguimiento. RESULTADOS: Se incluyeron 78 pacientes pediátricos con EC. La edad mediana en el momento del diagnóstico fue de 10,5 años, y el tiempo mediano de seguimiento fue de 3,8 años. El 64,1% de los pacientes eran varones. Del total, 15 (19,2%) tenían enfermedad perianal, de los cuales 10 presentaban hallazgos fistulizantes y 5 no fistulizantes. La EP estaba presente en el momento del diagnóstico en 8 pacientes, y el resto la desarrolló en una mediana de 1 año desde el diagnóstico. La EP se asoció con retraso en el crecimiento (p = 0,003), empleo de terapias biológicas (p = 0,005) y necesidad de una segunda línea de terapia biológica (p = 0,005). La mayoría de los pacientes (12/15, 80%) tuvieron un buen control de la EP con el tratamiento recibido. CONCLUSIONES: Los pacientes de EC con EP parecen necesitar un tratamiento más agresivo, en el que las terapias biológicas desempeñan hoy en día un papel fundamental. Estos pacientes precisan de una estrecha evaluación nutricional que garantice su correcto crecimiento y desarrollo.

Routine anti-reflux surgery combined with gastrostomy in children: is it really necessary? Our single-center experience. / ¿Es realmente necesario asociar una técnica antirreflujo a la gastrostomía de forma rutinaria? Experiencia en nuestro centro.

OBJECTIVE: To study gastroesophageal reflux (GER) in children undergoing gastrostomy in a single pediatric institution. MATERIAL AND METHODS: A retrospective study of patients undergoing gastrostomy from 2000 to 2017 was carried out. Demographic data, clinical data, progression, and complications were recorded. GER was considered positive in patients with clinical signs requiring antisecretory treatment, prokinetic treatment, or anti-reflux surgery to control symptoms. RESULTS: 207 patients with a median age of 2 years [R: 0.25-18] were included. Neurological impairment was the most frequent underlying condition (74%). Swallowing difficulty and undernourishment were the main surgical indications for gastrostomy. Prior to gastrostomy, 96 out of 207 patients (46%) showed GER symptoms. Combined fundoplication and gastrostomy was performed in 41 (43%) patients with preexisting GER, 6 of whom showed GER worsening (4 required redo fundoplication). 5 complications following fundoplication were noted - gastric perforation, sustained Dumping syndrome, and gastroesophageal stenosis. 55 out of 96 (57%) patients with preexisting GER underwent gastrostomy alone. Clinical signs disappeared in 16 of them (29%) and improved or stabilized in 19 (35%). GER worsening occurred in 20 patients (36%), with subsequent fundoplication being required in 10 cases. In patients with no previous clinical signs (111 out of 207), GER symptoms occurred following gastrostomy in just 18 cases (16%), and only 2 patients required fundoplication. CONCLUSIONS: In our experience, routine anti-reflux surgery combined with gastrostomy is not justified. Individualized fundoplication should be considered in case of medical treatment failure. Further studies with an adequate design are required to establish which patients could really benefit from this procedure.

OBJETIVO: Estudio del reflujo gastroesofágico (RGE) en los pacientes en los que se ha realizado una gastrostomía en nuestro centro. MATERIAL Y METODOS: Revisión de los pacientes intervenidos de gastrostomía en el periodo 2000-2017. Registro de datos demográficos, clínicos, evolución y complicaciones. Definimos RGE como la presencia de clínica compatible en pacientes que requirieron tratamiento médico o quirúrgico antirreflujo. RESULTADOS: Incluimos 207 pacientes con una mediana de edad de 2 años [r:0,25-18]. La patología subyacente más frecuente fue déficit neurológico (74%). Las indicaciones quirúrgicas fueron trastornos deglutorios y/o desnutrición. Previamente a la gastrostomía, 96/207 pacientes (46%) presentaban clínica de RGE. Se realizó funduplicatura asociada a gastrostomía en 41/96 (43%) de los pacientes con RGE previo. En 6/41 pacientes (15%) el RGE empeoró, requiriendo 4 de ellos una segunda funduplicatura. Se registraron 5 complicaciones tras funduplicatura (perforaciones gástricas, síndromes de Dumping prolongados y estenosis esofagogástrica). En 55/96 pacientes con RGE previo a la gastrostomía no se asoció funduplicatura. La clínica desapareció en 16/55 (29%), y mejoró o se estabilizó en 19/55 pacientes (35%). En 20/55 (36%) la sintomatología empeoró, y 10 de ellos precisaron una funduplicatura posterior. De los pacientes sin clínica previa de RGE (111/207), presentaron síntomas de RGE tras la gastrostomía 18/111 (16%), y solo 2 pacientes requirieron funduplicatura. CONCLUSIONES: Según nuestra experiencia, la funduplicatura de rutina asociada a la gastrostomía no está justificada. En caso de fracaso del tratamiento médico del RGE, una técnica antirreflujo debe plantearse de forma individualizada. Son necesarios estudios adecuadamente diseñados para definir qué pacientes realmente se beneficiarían de este procedimiento.

Acute abdomen in COVID-19 disease: the pediatric surgeon's standpoint. / Abdomen agudo en la enfermedad COVID-19. El punto de vista del cirujano pediátrico.

OBJECTIVE: To describe our experience in the diagnostic and therapeutic management of patients with acute abdomen as the main manifestation of SARS-CoV-2 infection. MATERIAL AND METHODS: A descriptive study of patients with clinical signs of acute abdomen diagnosed with COVID-19 and admitted at out healthcare facility from April 1 to May 10, 2020 was carried out. Clinical records were reviewed for data collection purposes. RESULTS: A series of 14 patients (9 male and 5 female) with a median age of 9.5 years was analyzed. All patients had abdominal pain. There were 11 patients with fever, 9 patients with vomit or diarrhea, and 9 patients with clinically suspected surgical pathology (acute appendicitis or peritonitis). Increased acute phase reactants and coagulation disorders were a common characteristic at blood tests. An abdominal ultrasonography was carried out in all patients, and a CT-scan was performed in 4 patients, which demonstrated inflammatory signs in the terminal ileum, the ileocecal valve and the ascending colon, as well as gallbladder edema. Conservative management was decided upon in all patients except one, and eight patients required intensive care admission for support treatment. CONCLUSIONS: Gastrointestinal symptoms can be the primary manifestation of the new coronavirus infection, which simulates an acute abdomen with a potentially unfavorable evolution. For an accurate diagnosis to be achieved, a good clinical record and a comprehensive physical exploration, as well as complementary tests in search of characteristic findings of COVID-19, should be carried out.

OBJETIVOS: Describir nuestra experiencia en el manejo diagnóstico y terapéutico de los pacientes que han presentado abdomen agudo como principal manifestación de la infección por SARS-Cov-2. MATERIAL Y METODOS: Estudio descriptivo de los pacientes ingresados con clínica inicial de abdomen agudo que fueron diagnosticados de COVID-19 entre el 1 de abril y el 10 de mayo de 2020. Se ha realizado la revisión de historias clínicas para la recogida de datos. RESULTADOS: Describimos una serie de 14 pacientes (9 varones y 5 mujeres) con una mediana de edad de 9,5 años. Todos ellos consultaron por dolor abdominal acompañado de fiebre en 11 y vómitos o diarrea en 9, y la sospecha clínica inicial fue de patología quirúrgica (apendicitis aguda o peritonitis) en 9. En la analítica sanguínea se encontró como característica común elevación de reactantes de fase aguda y alteraciones de coagulación. Se realizó ecografía abdominal a todos los pacientes y tomografía computarizada en cuatro observándose signos inflamatorios en íleon terminal, válvula ileocecal, colon ascendente y edema de vesícula biliar. Se optó por un manejo conservador en todos los pacientes menos uno y ocho pacientes precisaron ingreso en cuidados intensivos para tratamiento de soporte. CONCLUSIONES: La infección por el nuevo coronavirus puede producir síntomas gastrointestinales como principal manifestación, simulando un abdomen agudo que en algunos casos puede evolucionar de forma desfavorable. Para el diagnóstico es preciso realizar una buena historia clínica y exploración física, así como pruebas complementarias en busca de hallazgos característicos de COVID-19.

[Neural crest disorders and Hirschsprung's disease]. / Neurocristopatías y enfermedad de Hirschsprung.

The term Neurocristopathy describes a group of conditions caused by aberrations in growth, migration and differentiation of neural crest cells. In this sense, Hirschsprung's disease is a consequence of a failure in neural crest cells migration to distal intestine and in consequence it is considered to be part of neurocristopathy concept. This concept includes several diseases that can present either alone or in combination as a syndrome. We present 4 patients suffering Hirschsprung's disease associated with others neurocristopathies: 1 Waardenburg's syndrome, 1 congenital central hypoventilation syndrome or Ondine's curse, 1 ganglioneuroblastoma and 1 patient with bilateral sensory deafness.

Neurocristopatías y enfermedad de Hirschsprung / Neural crest disorders and hirschsprung's disease

El término neurocristopatía describe un grupo de procesos que derivan de un defecto en el crecimiento, diferenciación y/o migración de las células de la cresta neural. En este sentido, la enfermedad de Hirschsprung es consecuencia de un defecto en la migración de las células de la cresta neural hacia el intestino distal y por lo tanto se considera que forma parte del concepto de neurocristopatía. Este concepto abarca múltiples y diversos desórdenes que pueden presentarse aislados o asociarse formando síndromes. Presentamos 4 casos de enfermedad de Hirschsprung asociado a otras neurocristopatías: 1 síndrome de Waardenburg, 1 síndrome de hipoventilación central congénita o curso Ondine, 1 ganglioneuroblastoma y 1 paciente que asociaba una hipoacusianeurosensorial bilateral (AU)

The term Neurocristopathy describes a group of conditions caused by aberrations in growth, migration and differentiation of neural crest cells. In this sense, Hirschsprung´s disease is a consequence of a failure in neural crest cells migration to distal intestine and in consequence it is considered to be part of neurocristopathy concept. This concept includes several diseases that can present either alone or in combination as a syndrome. We present 4 patients suffering Hirschsprung´s disease associated with others neurocristopathies: 1 Waardenburg´s syndrome, 1 congenital central hypoventilation syndrome or Ondine´s curse,1 ganglioneuroblastoma and 1 patient with bilateral sensory deafness (AU)

[Morpho-functional study of electrostimulated latissimus dorsi muscle flap for diaphragm substitution]. / Estudio morfofuncional de una plastia electroestimulada del músculo latissimus dorsi para sustitución diafragmática.

Based on practical applications of the muscle latissimus dorsi (MLD) as much in reconstruction thoracic-abdominal defects as functionally in cardiomyoplasty, and in our experience in the electrostimulation with diaphragm pacing, we elaborated the primary objective of this work consisting of the creation of functional muscle plasty of MLD for substitution of the diaphragm. In two groups of cats formed by 6 animals each one, with section of the corresponding phrenic nerve, MLD plasty has been done to replace hemidiaphragm, conserving the thoracodorsal pedicle, by thoracic route in a group (intrathoracic plasty), and in the other by abdominal route (intraabdominal plasty). In a second intervention the stimulador electrode is implanted on thoracodorsal pedicle and the rest of components of the diaphragmatic pacing in a subcutaneous pocket. During a month progressive electroestimulation of MLD plasty is made, carrying out radiological and spirometric evaluation, that compares with evaluation control made before making plasty. After the sacrifice of the animals planimetric and histologic evaluation of the extirpated diaphragm were performed. Morphologic and functional results obtained allow us to affirm that functional plasty of MLD is an effective diaphragmatic substitute, although with a smaller degree of function than the original diaphragm, and functionally intraabdominal plasty has been superior to intratorácica plasty.

Estudio morfofuncional de una plastia electroestimulada del músculo latissimus dorsi para sustitución diafragmática / Morpho-functional study of electrostimulated latissimus dorsi muscle flap for diaphragm sustitution

Basándonos en las aplicaciones prácticas del músculo latissimus dorsi (MLD) o dorsal ancho tanto en reconstrucción de defectos toracoabdominales como funcionalmente en la cardiomioplastia, y en nuestra experiencia en la electroestimulación con marcapasos diafragmático, elaboramos el objetivo principal de este trabajo consistente en la creación de una plastia funcional muscular de MLD para sustitución del diafragma. En dos grupos de gatos de 6 animales cada uno, con sección del nervio frénico correspondiente, se ha practicado una plastia de MLD para sustituir a un hemidiafragma, conservando el pedículo tóracico dorsal, en un grupo por vía torácica (plastia intratorácica) y en el otro por vía abdominal (plastia intraabdominal). Posteriormente en una segunda intervención implantamos el electrodo estimulador sobre el pedículo toracodorsal y el resto de componentes del marcapasos diafragmático en un bolsillo subcutáneo. Durante un mes se realiza, de forma progresiva, electroestimulación de la plastia de MLD, llevando a cabo evaluación radiológica y espirométrica, que se compara con evaluaciones control practicadas antes de realizar la plastia. Después del sacrificio de los animales se practica valoración planimétrica e histológica del diafragma extirpado. Los resultados morfológicos y funcionales obtenidos nos permiten afirmar que la plastia funcional de MLD es un sustituto diafragmático efectivo, aunque con un menor grado de función que el diafragma original, y funcionalmente la plastia intraabdominal ha resultado superior a la plastia intratorácica (AU)

Based on practical applications of the muscle latissimus dorsi (MLD) as much in reconstruction thoracic-abdominal defects as functionally in cardiomyoplasty, and in our experience in the electrostimulation with diaphragm pacing, we elaborated the primary objective of this work consisting of the creation of functional muscle plasty of MLD for substitution of the diaphragm. In two groups of cats formed by 6 animals each one, with section of the corresponding phrenic nerve, MLD plasty has been done to replace hemidiaphragm, conserving the thoracodorsal pedicle, by thoracic route in a group (intrathoracic plasty), and in the other by abdominal route (intraabdominal plasty). In a second intervention the stimulador electrode is implanted on thoracodorsal pedicle and the rest of components of the diaphragmatic pacing in a subcutaneous pocket. During a month progressive electroestimulation of MLD plasty is made, carrying out radiological and spirometric evaluation, that compares with evaluation control made before making plasty. After the sacrifice of the animals planimetric and histologic evaluation of the extirpated diaphragm were performed. Morphologic and functional results obtained allow us to affirm that functional plasty of MLD is an effective diaphragmatic substitute, although with a smaller degree of function than the original diaphragm, and functionally intraabdominal plasty has been superior to intratorácica plasty (AU)

Tumores tiroideos en la infancia: aspectos clínicos diagnósticos y terapéuticos / No disponible

El objetivo de este trabajo consiste en analizar de forma retrospectiva los aspectos clínicos, anatomopatológicos, diagnósticos y terapeúticos de los tumores tiroideos recogidos en el Servicio de Endocrinología del Hospital Infantil Universitario Niño Jesús desde 1989 a 2004. Pacientes. Un total de catorce pacientes han sido diagnosticados de patología tumoral tiroidea. Sólo uno de ellos había recibido irradiación ene l área cervicocefálica en el curso de un traplante de médula ósea. Dos pacientes tenían historia familiar de síndrome de MEN tipo 2ª. Resultados. La edad media al diagnóstico de los 14 pacientes revisados, 11 niñas (79%) y 2 niños (21%), fue de 10,2 años (5-14 años); de ellos, 6 tenían una edad inferior a 6 años al diagnóstico. El principal signo que condujo al diagnóstico fue la aparición de bocio con o sin modularidad tiroidea (n=10; 71%). De los restantes 4 pacientes, uno consultó por adenopatías cervicales (7%), dos fueron diagnosticados en el seguimiento por historia familiar de síndrome MEN2A (12%) y el último, que no presentaba bocio, pero sí adenopatías cervicales, fue diagnosticado casualmente al observarse metástasis pulmonares en una radiografía realizada por otro motivo (7%). Los diagnósticos anatomopatólogos fueron: carcinoma papilar (n=7; 50%), carcinoma folicular (n=2; 14%), carcinoma medular (n=2; 14%), adenoma folicular (n=1; 7%), adenoma tóxico (n=1; 7%) y poliadenomatosis (n=1,7%). En 11 de los pacientes el tumor estaba confinado al tiroides, mientras que 3 pacientes (22%) presentaban metástasis en el momento del diagnóstico, 2 ganglionares y uno ganglionares y pulmonares. Todos los pacientes, salvo el que presentó un adenoma tóxico (hipertiroidismo clínico y bioquímico), se encontraban eutiroideos. El tratamiento fue quirúrgico en el 100% de los casos: tiroidectomía total con limpieza ganglionar (11 carcinomas y 1 poliadenomatosis) o hemitiroidectomía (2 adenomas). Los pacientes con carcinoma papilar y folicular recibieron terapia ablativa con I131 tras la cirugía y, posteriormente, levotiroxina a dosis supresoras del TSH (…) (AU)

The main objective of this study was to retrospectively analyze the clinical, pathological, diagnostic and therapeutic elements related to the patients with thyroid carcinoma admitted to the Department of Endocrinology of the Hospital Infantil Universitario Niño Jesús form 1989 until 2004. Patients. Fourteen patients (11 girls (70%) and 3 boys (21%)) with thyroid carcinoma were studied. The mean age at diagnosis was 10.3 yrs (5-14 yrs); with six patients less than 6 yrs of age at diagnosis. Only one patient received irradiation in the neck-head area during bone marrow transplantation. Two patients had familial antecedents of Men syndrome type 2A. Results. The most important clinical sign was the existence of goiter with or without thyroid nodule (n=10; 71%). Among the other four patients, one exhibited cervical adenopathy (7%), two were diagnosed during follow-up of familial history of MEN syndrome type 2A (12%) and the remaining patient was diagnosed when pulmonary metastasis was observed in a thoracic X-ray (7%). The pathological diagnoses were as follows: papilar carcinoma (n=7; 50%), follicular carcinoma (n=2; 14%), medular carcinoma (n=2; 14%), follicular adenoma (n=1; 7%), toxic adenoma (n=1; 7%) and polyadenomatosis (n=1; 7%). In eleven patients the tumor was only localized in the thyroid gland (78%); while the presence of metastasis was evident in three patients (22%). In two cases it was gangliar and one case pulmonary and gangliar. All children were euthyroid, except the patient with toxic adenoma (clinical and biochemical hyperthyroidism). Surgical treatment was employed in all cases: total thyroidectomy with ganglionectomy in 12 cases (11 carcinomas and one polyadenomatosis) or hemithyroidectomy in two patients with adenoma. All patients with either papilar or follicular carcinoma received I131 after surgery and later levothyroxine to suppress TSH levels (…) (AU)

Miositis osificante circunscrita axilar / Myositis ossificans circumscripta in the axilla

La miositis osificante circunscrita (MOC) es un proceso benigno raro y hereditario de formación heterotópica de hueso en tejidos blandos y músculo, rara vez encontrada en la infancia. Su localización más frecuente son las extremidades. Se presenta una adolescente de 15 años que sin traumatismo previo presentó una tumoración en axila. Las pruebas de imagen practicadas (radiografía simple, ecografía) no permiten establecer el diagnóstico, ni tampoco excluir malignidad. La biopsia de la tumoración y el estudio histopatológico permitió alcanzar el diagnóstico de miositis osificante circunscrita. En algunos artículos publicados se sugiere que la resonancia magnética (RM) es la primera herramienta en el diagnóstico precoz de esta entidad y evitar la intervención quirúrgica; sin embargo, en muchos casos todavía el diagnóstico es histológico. Debe diferenciarse sobre todo de procesos malignos (sarcomas) y de la fibrodisplasia osificante progresiva. La MOC tiene un buen pronóstico en la infancia (AU)

[Myositis ossificans circumscripta in the axilla]. / Miositis osificante circunscrita axilar.

Myositis ossificans circumscripta (MOC) is a rare benign hereditary disorder characterized by heterotopic bone formation in soft tissue and muscle. This disorder is rarely seen in childhood. MOC is most commonly located in the extremities. We describe a 15-year-old girl with nontraumatic MOC in the axilla. Imaging studies (radiographic, ultrasound) did not provide the correct diagnosis and did not rule out malignancy. Incisional biopsy of the mass and histopathologic examination gave the diagnosis of MOC. Some reports suggest that magnetic resonance imaging may be the first-choice diagnostic option and may avoid unnecessary biopsy. However, in many cases diagnosis is still histological. Differential diagnosis between these lesions and malignant sarcomas and fibrodysplasia ossificans progressiva is required. This entity has a good prognosis in childhood.

[Epidermoid splenic cysts and partial splenectomy]. / Quistes epidermoides de bazo y esplenectomía parcial.

We communicate two cases of epidermoid splenic cyst of great size in four and six years old children, with pain in right hypochondrium in one of them, having been casual the discovery of the other one in ultrasound study of the abdomen. In both cases the abdominal ultrasound study was the most useful exploration for its diagnosis. The surgical treatment by means of partial splenectomy and conservation of more than a third of the organ it has been feasible in both patients, with a favorable evolution and without complications. The histopathologic study confirmed the discovery of epidermoid spleen cysts. Later on a clinical, analytic pursuit has been made and of these patients image with a very satisfactory evolution.

[Freeman-Sheldon syndrome: clinical manifestations and anesthetic and surgical management]. / Síndrome de Freeman-Sheldon: manifestaciones clínicas y manejo anestésico y quirúrgico.

We report a new sporadic case of Freeman-Sheldon syndrome. The parents were not blood relatives. The boy showed characteristic deformities of distal arthrogryposis in the hands and feet, as well as the typical features of "whistling face syndrome". In addition, the patient showed other clinical manifestations such as a large bilateral inguinal hernia and thoracic cage abnormalities. The latter abnormality led to serious episodes of bronchopneumonia that delayed the surgical repair of bilateral inguinal hernia. Knowledge of the sonographic characteristics of deformities of the extremities is essential to reach an early prenatal suspected diagnosis of Sheldon-Freeman syndrome, especially in families with a history of the syndrome. We describe the preanesthetic management, anesthetic method and surgical technique performed when the child was aged 9 months. The delay was due to recurrent episodes of bronchopneumonia.

Síndrome de Freeman-sheldon: manifestaciones clínicas y manejo anestésico y quirúrgico / Freeman-sheldon syndrome: clinical manifestations and anesthetic and surgical management

Se comunica un nuevo caso esporádico de síndrome de Freeman-Sheldon, de padres no consanguíneos. El niño presentaba deformidades características de artrogriposis distal en manos y pies y además la característica facies de "cara en silbido". Asimismo exhibía otras manifestaciones clínicas como gran hernia inguinal bilateral y deformidades de la caja torácica. Esta última malformación le condicionó y le provocó serios episodios bronconeumónicos, que demoraron el tratamiento quirúrgico de la hernia inguinal bilateral. Es preciso conocer las características ecográficas de las deformidades de las extremidades de este síndrome para lograr un diagnóstico de sospecha prenatal precoz de síndrome de Freeman-Sheldon, sobre todo en familias con historia previa. Se expone el manejo preanestésico, el método anestésico y el tratamiento quirúrgico que fue realizado a los 9 meses de edad, debido a la demora impuesta por los episodios repetitivos de bronconeumonía (AU)

[Anorexia nervosa or somatic disease]. / Anorexia nerviosa o enfermedad somática?

We have studied 18 patients with anorexia nervosa with antroduodenal manometry for 24 hours and also 24 hours oesophageal pH studies. After the first 12 hours of measurements we started treatment with Cisapride (n = 8) or Erythromycin (n = 10) in a blind study. The results of measurements reveal a severe gastroesophageal reflux in 4 patients. Antroduodenal manometry showed dysfunctions in gastric motility, without relation with weight loss or duration of the disease. Cisapride and more so Erythromycin favor gastrointestinal motility.

¿Anorexia nerviosa o enfermedad somática? / Anorexia nervosa or somatic disease?

Hemos efectuado un estudio con 18 pacientes diagnosticados de anorexia nerviosa, consistente en manometría antroduodenal y pHmetría de 24 horas. A las 12 horas de registro se inició tratamiento con cisaprida (n=8) o eritromicina (n=10) de forma aleatoria. Los resultados de la pHmetría revelan reflujo gastroesofágico severo en 4 pacientes. La manometría pone de manifiesto la existencia de alteraciones de la motilidad gástrica, sin relación con la pérdida de peso ni con el tiempo de evolución. La cisaprida y sobre todo la eritromicina, actúan favorablemente sobre las alteraciones de la motilidad (AU)

[Congenital absence of portal vein in a girl with biliary atresia treated with liver transplant]. / Ausencia congénita de vena porta en niña con atresia de vías biliares, sometida a trasplante hepático.

The congenital absence of the portal vein is a rare malformation, which has been generally discovered in association with another anomalies like a cardiac, gastrointestinal or genitourinary defects. With portal hypertension and hepatic cirrhosis, this anomaly is similar to spontaneous porto-systemic derivation, and for that not collateral venous drainage is present. In these patients, total interruption of mesentaric venous drainage during procurement of liver transplant produce a very important bowel and mesenteric edema, which can promote an injert fatal evolution. The authors present the first paediatric liver transplant, in a patient with portal venous agenesia, with de piggy-back technique was done and this complication was obviated, and a review of the literature about this issue is done.

An unusual domestic accident: a penetrating abdominal wound with intestinal evisceration in a child.

We report a penetrating abdominal injury due to a piece of glass, a very unusual domestic accident, in a 2-year-old boy who was admitted to the emergency department with an evisceration through the umbilicus. His mother reported that she was asleep when the boy came to her room, and did not know how it had happened. We primarily suspected child abuse, but the presence of a piece of glass in the boy's heel led us to think of a domestic accident. He had poured himself a glass of water, and then fell on the glass. We could not find a similar case in the literature on domestic accidents.

[The colonic atresia: an uncommon cause of neonatal intestinal obstruction]. / La atresia de colon: causa poco frecuente de obstrucción intestinal neonatal.

The intestinal atresia is a common cause of neonatal bowel obstruction, but the colonic atresia is an uncommon cause of neonatal intestinal obstruction. We present a newborn with congenital colon atresia who underwent laparotomy, revealing a colonic atresia type III. We recommend resection of the dilated proximal colon with primary anastomosis end to end if the patient is not perforated or in bad general conditions.

Transdiaphragmatic pressure in quadriplegic individuals ventilated by diaphragmatic pacemaker.

BACKGROUND: Electrophrenic pacing can be used in the management of ventilatory failure in quadriplegic patients. A study was undertaken to determine the pattern of transdiaphragmatic pressure (PDI) during the conditioning phase of electrophrenic pacing to see if it had a possible role in optimising the process of conditioning. METHODS: The tidal volume (TV) and PDI were measured in a group of six quadriplegic patients commencing ventilation by low frequency pulse stimulation (7-10 Hz) and low respiratory rate stimulation (< 10 breaths/min). RESULTS: Tidal volume increased between baseline and month 1 (4.33 ml/kg, p < 0.001) and between months 1 and 2 (3.00 ml/kg, p < 0.05) and then stabilised. PDI was higher during bilateral diaphragmatic pacing (mean (SD) 1.73 (0.30) kPa) than with either left (1.15 (0.34) kPa) or right (0.86 (0.37) kPa) unilateral pacing. PDI varied throughout the observation period, probably by interaction between recovery of the diaphragmatic fibres and the pacing regimen. CONCLUSIONS: Patients with quadriplegia due to high spinal injury can be maintained with ventilation by continuous electrophrenic pacing. The control criteria used in this study for pacing were tidal volume and the patient's tolerance, and the PDI measurement did not contribute any additional information to help with managing the conditioning process.