RESUMO
Resumen Introducción: Las malformaciones vasculares linfáticas (MVL), anteriormente llamadas linfangiomas, son malformaciones congénitas que se presentan en uno de cada 6,000 a 16,000 nacimientos. El sistema de clasificación más útil para el manejo del linfangioma se basa en el tamaño de los quistes. La resolución espontánea es infrecuente, por lo que el tratamiento expectante no se recomienda. El tratamiento clásico es la cirugía de escisión, pero puede afectar a estructuras vecinas o haber recidivas, por lo que se empezaron a estudiar sustancias esclerosantes, como el OK-432. La mayoría de los estudios incluyen pocos pacientes; los más grandes realizados en México se enfocan a lesiones específicas (macroquísticas) o únicamente a una región anatómica. Hasta la fecha, no existen estudios del uso de este fármaco en la población del norte de México. Método: Se describe la experiencia con OK-432 mediante un estudio retrospectivo, descriptivo, en los pacientes con MVL, de 2011 a 2016, en un hospital de referencia del norte de México. Resultados: Veintiséis pacientes con MVL recibieron tratamiento con OK-432. La mayoría fueron macroquísticos (69%), microquísticos (19%) y mixtos (12%). Del total de pacientes, 11 presentaron curación total. El 72% de la población estudiada tuvo una reducción de > 50% del tamaño de las lesiones con solo dos aplicaciones de tratamiento; no se presentaron recidivas. Se reportaron complicaciones en dos pacientes (hiperpigmentación de la piel). Conclusiones: El manejo con OK-432 demostró ser efectivo para el tratamiento de las MVL en un hospital de referencia del norte de México.
Abstract Background: Lymphatic vascular malformations (LVM) or formerly called lymphangiomas are congenital malformations present in about 1 out of 6000 to -16000 births. The most relevant classification system for lymphangioma management is based on the size of the cysts. Spontaneous resolution is uncommon; thus, expectant management is not recommended. The classic treatment is excisional surgery, but it can affect adjacent structures or have relapses, so, sclerosing substances like OK-432 are being studied. The majority of the studies are small in number of patients and are from Japan; the largest studies in Mexico are focused on specific lesions (macrocystic) or a determined anatomical region. To date, there are no studies of the population of the north of Mexico. Methods: The experience with OK-432 was described through a retrospective, descriptive study in patients with LVM, from 2011 to 2016, in a reference hospital of northern Mexico. Results: A total of 26 patients with LVM were treated with OK-432. The majority of the lesions were macrocystic (69 %), microcystic (19 %) and mixed (12 %). From the total number of patients, 11 fully healed, and 72 % of the study population had >50 % reduction in lesion size, with only 2 applications. There were no recurrences. Complications were reported in 2 patients who had skin hyperpigmentation. Conclusions: OK-432 probed to be an effective treatment for LVM in a reference hospital in the north of Mexico.
Assuntos
Feminino , Humanos , Masculino , Picibanil/uso terapêutico , Anormalidades Linfáticas/tratamento farmacológico , Linfangioma/tratamento farmacológico , Antineoplásicos/uso terapêutico , Picibanil/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento , Hiperpigmentação/induzido quimicamente , Anormalidades Linfáticas/patologia , Linfangioma/patologia , México , Antineoplásicos/efeitos adversosRESUMO
Resumen: Introducción: La pseudo-obstrucción colónica aguda, conocida también como síndrome de Ogilvie, es una condición gastrointestinal poco frecuente en pediatría. Se caracteriza por una marcada dilatación del colon en estudios de imagen y ausencia de obstrucción mecánica. Los pacientes se presentan clásicamente con dolor abdominal y abdomen distendido, timpánico con peristalsis presente, acompañado de náusea y vómito. Hasta el 40% de los pacientes canalizan gases o evacúan. Este caso se reporta debido a lo raro que es este síndrome en pacientes infantiles y a que no hay casos reportados en un paciente pediátrico post-trasplante renal. Caso clínico: Paciente masculino de 13 años de edad con antecedente de retraso psicomotor posterior a asfixia perinatal e insuficiencia renal crónica secundaria a hipoplasia renal bilateral. Fue tratado mediante diálisis peritoneal durante un año hasta la realización del trasplante renal. Se encuentra en manejo con inmunosupresores. Inició su padecimiento actual al presentar dolor abdominal leve acompañado de evacuaciones semilíquidas, posteriormente presentó distensión abdominal progresiva de hasta 78 cm de perímetro abdominal, por lo que se realizaron estudios de imagen. Se prescribieron medicamentos procinéticos, sin mejoría. Se realizaron 2 laparotomías exploratorias observándose bridas, sin datos de obstrucción mecánica. Se realizó una resonancia magnética abdominal, observándose importante dilatación intestinal sin datos de obstrucción mecánica. Se diagnosticó síndrome Ogilvie y se administró neostigmina, presentando resolución del cuadro clínico. Conclusiones: Se reporta este caso debido a que es un síndrome muy raro en la edad pediátrica, existe poca sospecha clínica y hacen falta guías de manejo para el diagnóstico y tratamiento en pacientes infantiles.
Abstract: Background: Acute colonic pseudo-obstruction, also known as Ogilvie syndrome, is a rare gastrointestinal syndrome in children. It is characterized by a marked dilatation of the colon evidenced by imaging and absence of mechanical obstruction. Patients typically present with abdominal pain and distended, tympanic abdomen, with peristalsis present, accompanied by nausea and vomiting. Up to 40% of patients can pass gas and/or have bowel movements. We decide to report this case because this syndrome is very rare in pediatric patients, and no cases have been reported in a post-renal transplant pediatric patient. Case report: 13 year old male patient with past medical history of psychomotor retardation due to perinatal asphyxia and chronic renal failure secondary to bilateral renal hypoplasia. Treated with peritoneal dialysis for one year until kidney transplant was performed. Currently under immunosuppressive regime. He began his condition with mild abdominal pain accompanied by semi-liquid stools, and progressive distention up to 78 cm of abdominal circumference in 72 hours, so image studies were performed. Managed with prokinetic drugs without any improvement. Two exploratory laparotomies observed flanges, without evidence of any mechanical obstruction. An abdominal magnetic resonance was performed, where important intestinal dilatation was observed with no evidence of mechanical obstruction. Ogilvie Syndrome was diagnosed, so management with neostigmine was established, which led to symptom resolution. Conclusions: This case is reported because this syndrome is very rare in children, there is little clinical suspicion and lack of management guides for diagnosis and treatment in patients of this age.
RESUMO
BACKGROUND: Acute colonic pseudo-obstruction, also known as Ogilvie syndrome, is a rare gastrointestinal syndrome in children. It is characterized by a marked dilatation of the colon evidenced by imaging and absence of mechanical obstruction. Patients typically present with abdominal pain and distended, tympanic abdomen, with peristalsis present, accompanied by nausea and vomiting. Up to 40% of patients can pass gas and/or have bowel movements. We decide to report this case because this syndrome is very rare in pediatric patients, and no cases have been reported in a post-renal transplant pediatric patient. CASE REPORT: 13 year old male patient with past medical history of psychomotor retardation due to perinatal asphyxia and chronic renal failure secondary to bilateral renal hypoplasia. Treated with peritoneal dialysis for one year until kidney transplant was performed. Currently under immunosuppressive regime. He began his condition with mild abdominal pain accompanied by semi-liquid stools, and progressive distention up to 78cm of abdominal circumference in 72hours, so image studies were performed. Managed with prokinetic drugs without any improvement. Two exploratory laparotomies observed flanges, without evidence of any mechanical obstruction. An abdominal magnetic resonance was performed, where important intestinal dilatation was observed with no evidence of mechanical obstruction. Ogilvie Syndrome was diagnosed, so management with neostigmine was established, which led to symptom resolution. CONCLUSIONS: This case is reported because this syndrome is very rare in children, there is little clinical suspicion and lack of management guides for diagnosis and treatment in patients of this age.
RESUMO
BACKGROUND: Hamartoma is a slow-growing, rare mixed benign tumor. In general, it does not produce symptoms, so it is more commonly found as an incidentaloma during autopsies or laparotomies. Incidence of splenic hamartomas is low, representing 0.001% of the general population. CLINICAL CASE: We report the case of a 39 year-old male without relevant antecedents. The evolution of his condition began 2 months prior with pyrosis and occasional pain in the upper hemi-abdomen. Abdominal ultrasound demonstrated a pseudocystic tumor in the spleen. Magnetic resonance showed four lesions in the spleen, predominating a large, bilobulated lesion in the inferior pole of 12 × 10 × 9 cm. A splenectomy was done without complications and the patient was discharged to home at third postoperative day. Pathological report showed a splenic hamartoma. CONCLUSIONS: Hamartomas of the spleen as in other localizations are benign lesions found as incidentalomas because only few produce symptoms. The final diagnosis is made histopathologically. Definitive treatment is splenectomy and treatment of choice is complete laparoscopic transabdominal splenectomy. Although there are few incidences in Mexico, it is of vital importance that the physician considers it among the differential diagnoses when evaluating a tumor.
Antecedentes: los hamartomas son tumores benignos mixtos muy raros, de crecimiento lento, que generalmente no provocan síntomas, por lo que es más frecuente encontrarlos como un incidentaloma en autopsias o laparotomías. La incidencia de los hamartomas del bazo es sólo de 0.001% entre la población general. Caso clínico: hombre de 39 años de edad, sin antecedentes relevantes, inició dos meses previos con pirosis y dolor ocasional en el hemiabdomen superior. En un ultrasonido de abdomen se observó una tumoración pseudoquística dependiente del bazo. La resonancia magnética reveló cuatro lesiones en el bazo, una de ellas de gran tamaño en el polo inferior, bilobulada, de 12 × 10 × 9 cm. Se realizó una esplenectomía. El paciente no sufrió complicaciones y fue dado de alta al tercer día. El reporte de patología indicó un hamartoma esplénico. Conclusiones: los hamartomas del bazo y otras localizaciones son lesiones benignas que se encuentran como incidelantomas. El diagnóstico se confirma por medio de estudio histopatólogico y su tratamiento es la esplenectomía, siendo la técnica preferida la transabdominal completa por vía laparoscópica. A pesar de que su baja incidencia en México, es de vital importancia que el médico lo considere como diagnóstico diferencial al evaluar una tumoración.