Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome: A Case Report of an Uncommon Condition.

The megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS), also known as Berdon syndrome, is a rare congenital condition that falls within the spectrum of visceral myopathies. It is characterized by the presence of megacystis, microcolon, and hypoperistalsis, which are secondary to gastrointestinal and urinary system dysmotility. It is frequently associated with other alterations in the gastrointestinal and genitourinary tracts. Although it is possible to make the diagnosis in the prenatal period, most cases are diagnosed after birth through genetic and imaging studies. Advances in treatment have led to a progressive increase in survival rates. We present the case of a newborn with congenital alterations described prenatally and with imaging findings characteristic of the syndrome.

Reemplazo esofágico mínimamente invasivo con tubo de Akiyama en un paciente pediátrico con quemadura esofágica / Minimally invasive esophageal replacement with Akiyama tube in a pediatric patient with esophageal burn

Introducción. La ingesta de cáusticos continúa siendo un problema de salud pública en los países en vía de desarrollo, por lo que a veces es necesario realizar un reemplazo esofágico en estos pacientes. Aún no existe una técnica estandarizada para este procedimiento. Caso clínico. Masculino de 10 años con estenosis esofágica por ingesta de cáusticos, quien no mejoró con las dilataciones endoscópicas. Se realizó un ascenso gástrico transhiatal por vía ortotópica mediante cirugía mínimamente invasiva como manejo quirúrgico definitivo .Discusión. Actualmente existen varios tipos de injertos usados en el reemplazo esofágico. La interposición colónica y gástrica son las que cuentan con mayores estudios, mostrando resultados similares. Conclusiones. La elección del tipo y posición del injerto debe ser individualizada, tomando en cuenta las características de las lesiones y la anatomía de cada paciente para aumentar la tasa de éxito.

Introduction. The ingestion of caustics continues to be a public health problem in developing countries, which is why sometimes is necessary to perform an esophageal replacement in these patients. There is still no standardized technique for this procedure. Clinical case. A 10-year-old male with esophageal stricture due to caustic ingestion, who did not improve with endoscopic dilations. A laparoscopic transhiatal gastric lift was performed orthotopically as definitive surgical management using minimally invasive surgery. Discussion. Currently there are several types of grafts used in esophageal replacement. Colonic and gastric interposition are the ones that have the most studies, showing similar results. Conclusions. Choice of type and position of the graft must be individualized, taking into account the characteristics of the lesions and anatomy of each patient, in order to increase the success rate.

Development and Validation of a Laparoscopy Simulation Model of Pyeloplasty for Pediatric Patients.

Introduction: Given the extensive training required for laparoscopic pyeloplasty and the difficulties achieving this training, there is a need to recreate the activity in a controlled environment, but high-fidelity models are unavailable or expensive. Our objective was to develop a model of pyeloureteral junction stenosis, resembling the anatomical details and consistency of natural tissue, for a replicable, cheaper, and realistic simulation model of laparoscopic pyeloplasty in children. Materials and Methods: A three-dimensional, printed synthetic model was created from magnetic resonance urography. The model comprises a plastic kidney as the reusable structure and a silicone renal pelvis and ureter as the interchangeable structure. We evaluated realism and performance with surgeons and residents at different levels of training, comparing operative time and complications of the procedure. Results: Twenty-four participants were recruited; 41.7% had previous experience in laparoscopic pyeloplasty, with 5.5 years of experience in laparoscopic surgery (interquartile range [IQR] 2-7.75). There were no cases of stenosis, but leaks accounted for 41.7%. The procedure lasted 72 minutes (IQR 55-90), with significant differences according to the level of training (85 minutes for residents, 68 minutes for pediatric surgeons and urologists, and 40 minutes for laparoscopic surgeons; P: .011) and years of previous experience in laparoscopic surgery (P: .003). Conclusions: A high-fidelity, replicable, and low-cost pyeloureteral stenosis model was developed to simulate laparoscopic pyeloplasty in pediatric patients.

Tratamiento laparoscópico de úlcera duodenal perforada mediante parche de Graham en un paciente pediátrico / Laparoscopic treatment of perforated duodenal ulcer using a Graham patch in a pediatric patient

Introducción. La úlcera duodenal perforada es una entidad de etiología no clara que rara vez ocurre en la población pediátrica. Generalmente se diagnostica de manera intraoperatoria y el tratamiento ideal incluye el uso del parche de epiplón o de ser necesario, la resección quirúrgica. Caso clínico. Se presenta un paciente de 12 años con abdomen agudo y diagnóstico tomográfico prequirúrgico de úlcera duodenal perforada, tratado por vía laparoscópica con drenaje de peritonitis y parche de Graham. La evolución y el seguimiento posterior fueron adecuados. Discusión. La úlcera duodenal perforada es una entidad multifactorial, en la que se ha implicado el Helicobacter pylori. El diagnóstico preoperatorio es un reto y el tratamiento debe ser quirúrgico. Conclusiones. Cuando se logra establecer el diagnóstico preoperatorio, se puede realizar un abordaje laparoscópico y el uso del parche de Graham cuando las úlceras son menores de dos centímetros.

Introduction. Perforated duodenal ulcer is an entity of unclear etiology that rarely occurs in the pediatric population. It is usually diagnosed intraoperatively and the ideal treatment includes the use of the omentum patch or, if necessary, surgical resection. Clinical case. A 12-year-old patient with acute abdomen and preoperative tomographic diagnosis of perforated duodenal ulcer, treated laparoscopically with peritonitis drainage and Graham patch is presented. The evolution and subsequent follow-up were adequate. Discussion. Perforated duodenal ulcer is a multifactorial entity, in which Helicobacter pylori has been implicated. Preoperative diagnosis is challenging and treatment must be surgical. Conclusions. When the preoperative diagnosis is established, a laparoscopic approach and the use of the Graham patch can be performed when the ulcers are less than two centimeters.

Malformaciones linfáticas abdominales en una población pediátrica: experiencia en un centro de referencia de Medellín, Colombia / Abdominal lymphatic malformations in a pediatric population: Experience in a referral center in Medellín, Colombia

Introducción. Los linfangiomas son anormalidades benignas del sistema linfático, que corresponden a dilataciones quísticas de estos vasos y se localizan especialmente en el cuello. Solo el 10 % de todas estas malformaciones se encuentran en el abdomen y presentan síntomas variables de acuerdo al tamaño y su ubicación especifica, siendo el dolor abdominal el principal síntoma. Métodos. Se presentan cinco pacientes pediátricos con malformaciones linfáticas abdominales. Se describen su cuadro clínico, localización, tratamiento y la experiencia en el manejo de dicha patología en un hospital de referencia. Resultados. Los métodos más apropiados para hacer una aproximación diagnóstica son la ecografía, la tomografía computarizada y la resonancia nuclear magnética. Dentro de las opciones descritas para el tratamiento están la farmacológica, la escleroterapia y la resección quirúrgica, tanto por vía abierta como por laparoscopia. Conclusión. Existe una variedad de métodos para realizar la resección de los linfangiomas abdominales, pero la cirugía sigue siendo la más efectiva, especialmente cuando se cuenta con la laparoscopia como una herramienta terapéutica.

Introduction. Lymphangiomas are benign abnormalities of the lymphatic system, which correspond to cystic dilations of these vessels and are located especially in the neck. Only 10% of all these malformations are found in the abdomen and present variable symptoms according to size and their specific location, with abdominal pain being the main symptom. Methods. Five pediatric patients with abdominal lymphatic malformations are presented. Their clinical presentation, location, treatment and experience in the management of this pathology in a referral hospital are described. Results. The most appropriate methods to make a diagnostic approach are ultrasound, computed tomography and magnetic resonance imaging. Among the options described for treatment are pharmacological, sclerotherapy and surgical resection, both open and laparoscopic. Conclusion. There are a variety of methods for resecting abdominal lymphangiomas, but surgery remains the most effective, especially when laparoscopy is used as a therapeutic tool.

Multicenter Evaluation with Eckardt Score of Laparoscopic Management with Heller Myotomy and Dor Fundoplication for Esophageal Achalasia in a Pediatric Population in Colombia.

Background: Esophageal achalasia is a rare, chronic, and progressive neurodegenerative motility disorder that is characterized by a lack of relaxation of the lower esophageal sphincter. Laparoscopic Heller myotomy (LHM) is the ideal in our population. Multiple surgical and medical treatments have been raised. However, there has been a need to expand studies and generate a clear algorithm for an ideal therapeutic algorithm. Methods: Clinical record was retrospectively analyzed of patients who underwent LHM and Dor fundoplication evaluated with Eckardt score, at four Colombian medical centers between February 2008 and December 2018. Results: There were a total of 21 patients (12 males and 9 females, ages 8 months to 16 years). The time from onset of symptoms to surgery was between 5 months and 14 years. One patient had esophageal mucosa perforation, 2 patients were converted to open surgery, and 1 patient had a postoperative fistula. All patients were discharged 3 to 9 days postoperatively, at which time they tolerated normal oral feeding. During follow-up, all the patients had an improvement in nutritional status and a greater functional recovery; 4 had reflux and 1 had reflux-like symptoms. Conclusion: LHM with Dor-type fundoplication maintains the effectiveness of open surgery with low postoperative morbidity and mortality and good functional results according to Eckardt score evaluation.