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.

Anesthetic management during anterior mediastinal mass resection in a pediatric patient. A case report. / Manejo anestésico para la resección de masa mediastínica anterior (MMA) en paciente pediátrico. Descripción de un caso clínico.

Complications induced by general anesthesia (GA) and neuromuscular relaxation (NMR) in anterior mediastinal mass (AMM) resection can be serious, especially when there are signs of compression of the airway or large vessels (dyspnea, orthopnea, etc.) (1). It is preferable to perform the procedure in spontaneous ventilation to avoid respiratory or cardiovascular collapse due to the supine position or to loss of negative intrathoracic pressure with GA and NMR. If the supine position and NMR are unavoidable, procedures should be performed in a step-wise manner, and rescue strategies should be prepared (rescue position, bronchoscope, sternotomy). Correct preoperative evaluation, adequate planning, and a multidisciplinary approach will ensure patient safety. We present the case of a child with a history of severe orthopnea and a diagnosis of AMM and lymphoblastic lymphoma (respiratory arrest and cardiovascular collapse during sedation for lumbar puncture and bone marrow biopsy) that did not respond to medical treatment and required resection surgery under GA with NMR.

[Laparoscopic adrenalectomy for neuroblastoma. Our serie and review of literature]. / Adrenalectomía laparoscópica en neuroblastoma. Presentación de nuestra serie y revisión de la literatura.

INTRODUCTION: Neuroblastoma is the most frequent adrenal mass in paediatric patients. Paediatric series about laparoscopic adrenalectomy are scarce, usually including adrenal masses from different origin. Series referring only to neuroblastoma are very rare. MATERIAL AND METHOD: We present 7 patients between 4 and 48 month of age. RESULTS: We performed 8 laparoscopic adrenalectomy and one biopsy. Lateral transperitoneal approach was used in all patients. We employed 3 ports on the left side and 4 on the right side. All tumours were extracted into a bag through the most posterior incision. One patient underwent a laparotomy because of important adherences. The average operative time was 88 minutes, and average time before discharging was 48 hours. CONCLUSIONS: Laparoscopic adrenalectomy is the gold standard in adult patients. Transperitoneal access achieves better working area. Paediatric patients don't have many surgical indications for adrenalectomy and the space inside the patient's body is smaller. Laparoscopy offers a less painful solution than open surgery, so that sooner discharging, and better scar's results. The outcome is similar to traditional surgery. Laparoscopy is an useful and safe procedure in paediatric patients with adrenal neuroblastoma, in very selected cases.

[Experience in performance of percutaneous endoscopic gastrostomy in 60 children]. / Experiencia en la colocación de la gastrostomía endoscópica percutánea en 60 niños.

The Percutaneous endoscopic gastrostomy (PEG) has radically changed the handling of the children with nutritional problems that until then were forced to receive nutritionals contributions by parenteral or enteral way, by nasogastric tube, conventional surgical gastrostomy or by central venous access. The objective of this work was to evaluate our experience with 60 patients, with diverse pathologies, that needed a PEG so that they could receive a suitable nutritional contribution. Were registered data of age, sex, reason for the accomplishment of the PEG, used surgical time, days of hospital stay and we analyzed the complications derived from the technique. There were two important complications. In one case one was a patient who presented a gastrocolic fistula as a result of the perforation of the colon in the accomplishment of the PEG and in another case a necrotizing fascitis in a girl who presented a choanal bilateral atresia. The rest of the complications were minor and they were treated without problems. The complications have been comparable to the registered in other series. Although the technique is simple and fast, is not free of risks, reason why it is necessary to inform suitably to the parents of the possibility of complications. In general, the degree of satisfaction of the parents was high.

[ Pulmonary inflammatory pseudotumor in a child]. / Pseudotumor inflamatorio de pulmón en el niño.

The Inflammatory Pseudotumor is a no neoplastic process characterised by an irregular growth of inflammatory cells. A case of 10 years old male is described, he was asymptomatic, diagnosed by a casual chest XR, with a spheric mass in the inferior lobe of the right lung. An excisional biopsy was done with the result of "Inflammatory Pseudotumor". The Inflammatory Pseudotumor is a rare process that can be found in different regions of the human body, with most frequent localization, in the lung. The image tests are not specific for the diagnosis that only can be done by biopsy. The recommended treatment is the complete resection.

[Yuxtapancreatic gastric duplication versus congenital pancreatic cyst]. / Duplicación gástrica yuxtapancreática versus quiste congénito de páncreas.

We report a clinic case of a child with juxtapancreatic retrogastric cyst tumor diagnosed by antenatal ultrasonography, he was operated on with 15 months of age. The pathologic diagnosis was of gastric duplication and the liquid inside the cyst had 1810 U/L of amylase. We review diagnosis and therapeutic aspects and literature overview.

[Gastric tonometry as monitoring system in abdominal surgery]. / Tonometría gástrica como sistema de monitorización en cirugía abdominal.

OBJECTIVE: The aim of this study is the evolution of the intramucosal pH (pHi) during the quirurgic and the immediate postoperative process in pediatric patients, operated in abdominal surgery longer than 90 minutes. MATERIAL AND METHODS: A prospective study including 25 patients was done. In all of them intraabdominal surgery was performed. The mean age was 5.6 +/- 3 years, range from 9 months to 13 years. The operative time was 207 +/- 76 minutes. The pHi was measured by means of a sigmoid tonometer introduced in the stomach. A tonometry was done hourly during the operative procedure, and every 8 hours during the postoperative period. RESULTS: 157 determinations of pHi were made with an average of 6.28 per patient. During the operative period the pHi decreased from 7.36 +/- 0.11 (30') to 7.22 +/- 0.11 (210'). During the postoperative, the pHi increased to 7.36 +/- 0.05, 24 hours later (p < 0.005). CONCLUSIONS: During the surgery there was a significant reduction of the pHi, which recovered 16 hours after in the postoperative.

[Left supraclavicular adenopathy with a neuroendocrine pattern of unknown cause: prostatic carcinoma metastasis]. / Adenopatía supraclavicular izquierda con patrón neuroendocrino de causa desconocida: metástasis por carcinoma de próstata.

Contribution of a case report of a male patient presenting with single left supraclavicular adenopathy of initially unknown origin and histologic pattern of neuroendocrine nature, finally traced to the prostate. It is believed that in any male over 45 diagnosed with carcinoma of unknown origin in the supradiaphragm lymph nodes, prostate carcinoma metastasis should also be ruled out using PSA immunostaining and by measuring serum PSA, serum alkaline phosphatase and DRE.

[Report of a new case of small-cell carcinoma of the bladder and review of the literature]. / Aportación de un nuevo caso de carcinoma de células pequeñas de localización vesical y revisión de la literatura.

We report a case of small cell carcinoma of the urinary bladder in 71-year-old male patient. Oat cell of the urinary bladder is extremely uncommon, and up to date only 135 cases have been reported in word literature. Histologic, microscopic, and immunohistochemical characteristics are similar to oat cell carcinoma of the lung and other extrapulmonary oat cell carcinomas. We conclude this report with immunohistochemical study with PGP 9.5, neuron-specific enolase a synaptophisine.