Factores asociados al desenlace en niños con mal rotación intestinal manejados en el Hospital Infantil de San Vicente Fundación, Medellín, Colombia / Factors associated with outcome in children with intestinal malrotation managed at the Hospital Infantil of San Vicente Fundación, Medellín, Colombia

Introducción. El intestino primitivo rota durante la vida embrionaria. Cuando ocurre de forma inadecuada aparece la malrotación intestinal que puede llevar a la obstrucción o al vólvulo del intestino medio. La incidencia disminuye con el aumento de la edad. La malrotación intestinal es una de las principales causas de complicaciones del tracto gastrointestinal en la edad pediátrica. Métodos. Estudio retrospectivo, observacional, transversal y analítico, de la experiencia durante 10 años en pacientes menores de 15 años con diagnóstico de malrotación intestinal, tratados en el Hospital Infantil de San Vicente Fundación, en Medellín, Colombia. Se buscó la asociación entre variables demográficas, clínicas e imagenológicas con los desenlaces. Resultados. Se obtuvieron 58 pacientes con malrotación intestinal, 65 % menores de 1 año. En 29,3 % de los pacientes se hizo el diagnóstico con la presentación clínica; los síntomas predominantes fueron distensión abdominal y emesis. En el 24,1 % se confirmó el diagnóstico con imágenes. Las variables con una diferencia estadísticamente significativa a favor de encontrar una malrotación complicada fueron choque séptico (OR=11,7), síndrome de respuesta inflamatoria sistémica (OR=8,4) y deshidratación (OR=5,18). Conclusiones. La malrotación intestinal tiene complicaciones como perforación, peritonitis, vólvulo e intestino corto. El vólvulo se acompaña de shock y sepsis, con mortalidad hasta del 50 %. Las imágenes diagnósticas son una ayuda, pero no se puede basar la conducta médica en estas porque ninguna imagen garantiza el diagnóstico definitivo. Los signos de alarma son poco específicos. En menores de un año con emesis, distensión y dolor abdominal se debe sospechar malrotación intestinal.

Introduction. The primitive intestine rotates during embryonic life. When it occurs inappropriately, intestinal malrotation appears, which can lead to obstruction or midgut volvulus. The incidence decreases when age increases. Intestinal malrotation is one of the main causes of complications of the gastrointestinal tract in pediatric age. Method. Retrospective, observational, cross-sectional and analytical study of the experience over 10 years in patients under 15 years of age with a diagnosis of intestinal malrotation, treated at Hospital Infantil of San Vicente Fundación, in Medellín, Colombia. The association between demographic, clinical and imaging variables with the outcomes was sought. Results. There were 58 patients with intestinal malrotation, 65% under one year of age. In 29.3% of patients, intestinal malrotation was diagnosed clinically. The predominant symptoms were abdominal distension and emesis. In 24.1% the diagnosis was confirmed with imaging. The variables with a statistically significant difference in favor of finding a complicated malrotation were septic shock (OR=11.7), systemic inflammatory response syndrome (OR-8.4), and dehydration (OR=5.18). Conclusions. Malrotation has complications such as perforation, peritonitis, volvulus, and short bowel. Volvulus is accompanied by shock and sepsis, with mortality of up to 50%. Diagnostic images are helpful, but medical conduct cannot be based on them because no image guarantees a definitive diagnosis. The warning signs are not very specific. In children under one year of age with emesis, distension and abdominal pain, intestinal malrotation should be suspected.

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.

Ovarian Epithelioid Hemangioendothelioma in a Pediatric Patient: A Case Report.

We present a case of an eight-month-old girl who was brought to the emergency department with bloody stools. An initial ultrasound reported a mass in the left iliac fossa that was further characterized by magnetic resonance imaging (MRI) as a hypervascular ovarian tumor. Prior to surgical resection of the tumor, abdominal arteriography with selective embolization and vessel occlusion was performed. Pathology reported epithelioid hemangioendothelioma of the left ovary. This condition has not been previously reported in girls. In this case report, we describe the ultrasound, MRI, and arteriographic findings with a histopathologic correlation of an adnexal tumor that is unknown in the pediatric female population until now.

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.

Síndrome de Currarino: informe de un caso / Currarino’s syndrome: a case report

El síndrome de Currarino es una enfermedad hereditaria rara. La forma completa se caracteriza por la presencia de una malformación ano-rectal (generalmente estenosis), masa presacra y defectos óseos del sacro. El signo más importante es la presencia de estreñimiento desde el nacimiento. Se presenta un paciente de 13 meses de edad que consultó por estreñimiento crónico severo en quien se encontró la presencia de hemisacro y meningocele anterior, ambos hallazgos compatibles con una forma leve del síndrome de Currarino. Se realizó un manejo conservador y se inició polietilenglicol oral (PEG®), con una excelente respuesta al tratamiento. Se hace la presentación del caso junto con la revisión de la literatura actual con el fin de dar a conocer al personal médico la presencia del síndrome, enfatizar en puntos importantes que generen sospecha de su presencia y las diferentes opciones terapéuticas para su manejo.

The Currarino’s syndrome is a rare inherited disorder. The complete form is characterized by the presence of ano-rectal malformation (usually stenosis), presacral mass and bone defects of the sacrum. The most important symptom is the presence of constipation since birth. A 13 month old patient with chronic and severe constipation is presented. Among the studies the presence of hemisacrum and anterior meningocele were found, both findings consistent with a mild form of Currarino’s syndrome. A conservative management with polietilenglycol oral (PEG®), was initiated with an excellent response to treatment. The presentation of the case with a review of current literature is given to the medical staff in order to publicize the syndrome, as well as to emphasize key points that create suspicion of their presence and the different therapeutic options for its approach.

Síndrome de la arteria mesentérica superior o síndrome de Wilkie / Superior Mesenteric Artery Syndrome or Wilkie Syndrome

Describimos tres casos de síndrome de arteria mesentérica superior (SAMS), también conocido como síndrome de Wilkie, ileus duodenal crónico, o por órtesis ortopédicas (“cast syndrome”) El síndrome ocurre por la compresión de la tercera porción del duodeno entre la arteria mesentérica superior y la aorta. Los principales riesgos relacionados con la aparición del síndrome son la pérdida rápida de peso y las cirugías de columna vertebral. La presentación clínica del síndrome es variable e inespecífica, e incluye náuseas, vómito, dolor abdominal y pérdida de peso. El diagnóstico se basa en los hallazgos de la compresión duodenal por la arteria mesentérica superior por endoscopia, radiología contrastada y tomografía. El tratamiento se dirige a corregir la causa de base, la cual frecuentemente se relaciona con la pérdida de peso. Por lo tanto, el manejo es inicialmente conservador con nutrición suplementaria y se reserva la cirugía para aquellos casos que no respondan a la terapia nutricional.

We described three cases of superior mesenteric artery (SMA) syndrome, also known as Wilkie’s syndrome, chronic duodenal ileus, or cast syndrome. This syndrome occurs when the third portion of the duodenum is compressed between the SMA and the aorta. The major risk factors for development of SMA syndrome are rapid weight loss and surgical correction of spinal deformities. The clinical presentation of SMA syndrome is variable and nonspecific, including nausea, vomiting, abdominal pain, and weight loss. The diagnosis is based on endoscopic, radiographic and tomographic findings of duodenal compression by the SMA. The treatment of SMA syndrome is aimed at the precipitating factor, which usually is related to weight loss. Therefore, conservative therapy with nutritional supplementation is the initial approach, and surgery is reserved for those who do not respond to nutritional therapy.

Quiste epidermoide intratesticular / Epidermoid intratesticular cyst

Los quistes epidermoides intratesticulares son tumores raros que representan menos de 1 porcien de todas las masas testiculares. El diagnóstico diferencial de estas lesiones es difícil, ya que pueden simular ser malignas. La ausencia de marcadores tumorales elevados y las características ecográficas ayudan a realizar un diagnostico preoperatorio apropiado y un manejo conservador de esta lesión con preservación del tejido testicular. Presentamos el caso de un niño de 22 meses de edad con un quiste intratesticular derecho detectado por examen físico y ecografía testicular, a quien se le realizo una enucleación del quiste y preservación del testículo.