Cirugía guiada por tromboelastometría en neuroblastoma complicado con coagulación intravascular diseminada / Thromboelastometry-guided surgery in neuroblastoma complicated with disseminated intravascular coagulation

Introducción: La coagulación intravascular diseminada (CID) es una urgencia oncológica poco común. Describimos el caso de un neuroblastoma pediátrico complicado con CID que precisó de cirugía guiada por tromboelastometría. Caso clínico. Paciente de seis años diagnosticada de neuroblastoma suprarrenal de riesgo intermedio que desarrolló CID asociada al tumor tras el primer ciclo de quimioterapia. Permaneció estable sin hemorragia clínica, decidiéndose una resección tumoral de urgencia guiada por tromboelastometría intraoperatoria. La CID se resolvió poco después de la cirugía, consiguiéndose una remisión total. Conclusión. El tratamiento de la patología subyacente es clave a la hora de manejar la CID. La tromboelastometría puede guiar la terapia orientada a objetivos, también en cirugías realizadas en pacientes pediátricos. No obstante, hacen falta mayores estudios que analicen su aplicabilidad en distintos contextos clínicos, como la CID relacionada con cáncer.(AU)

Background: Disseminated intravascular coagulation (DIC) is a rare oncological emergency. We report a pediatric neuroblastoma complicated with DIC which required thromboelastometry-guided surgery. Observation. A 6-year-old female diagnosed with intermediate risk adrenal neuroblastoma developed tumor-related DIC after chemotherapy first cycle. She remained stable without clinical bleeding and emergent tumor resection guided by intraoperative-thromboelastometry was decided. DIC resolved early after surgery and complete remission was achieved. Conclusion. Treatment of the underlying condition is critical to manage DIC. Thromboelastometry can guide goal-directed therapy, including surgery in pediatric patients. However, larger studies are needed to examine its applicability in different clinical settings, such as cancer related DIC.(AU)

Thromboelastometry-guided surgery in neuroblastoma complicated with disseminated intravascular coagulation. / Cirugía guiada por tromboelastometría en neuroblastoma complicado con coagulación intravascular diseminada.

BACKGROUND: Disseminated intravascular coagulation (DIC) is a rare oncological emergency. We report a pediatric neuroblastoma complicated with DIC which required thromboelastometry-guided surgery. OBSERVATION: A 6-year-old female diagnosed with intermediate risk adrenal neuroblastoma developed tumor-related DIC after chemotherapy first cycle. She remained stable without clinical bleeding and emergent tumor resection guided by intraoperative-thromboelastometry was decided. DIC resolved early after surgery and complete remission was achieved. CONCLUSION: Treatment of the underlying condition is critical to manage DIC. Thromboelastometry can guide goal-directed therapy, including surgery in pediatric patients. However, larger studies are needed to examine its applicability in different clinical settings, such as cancer related DIC.

INTRODUCCION: La coagulación intravascular diseminada (CID) es una urgencia oncológica poco común. Describimos el caso de un neuroblastoma pediátrico complicado con CID que precisó de cirugía guiada por tromboelastometría. CASO CLINICO: Paciente de seis años diagnosticada de neuroblastoma suprarrenal de riesgo intermedio que desarrolló CID asociada al tumor tras el primer ciclo de quimioterapia. Permaneció estable sin hemorragia clínica, decidiéndose una resección tumoral de urgencia guiada por tromboelastometría intraoperatoria. La CID se resolvió poco después de la cirugía, consiguiéndose una remisión total. CONCLUSION: El tratamiento de la patología subyacente es clave a la hora de manejar la CID. La tromboelastometría puede guiar la terapia orientada a objetivos, también en cirugías realizadas en pacientes pediátricos. No obstante, hacen falta mayores estudios que analicen su aplicabilidad en distintos contextos clínicos, como la CID relacionada con cáncer.

Síndrome de Horner pediátrico. A propósito de una serie de 14 casos en un hospital terciario / Paediatric Horner syndrome. A case series of 14 patients in a tertiary hospital

Antecedentes El síndrome de Horner (SH) se caracteriza por la triada de ptosis palpebral, miosis y anhidrosis facial. Debido a su amplia variedad de causas puede ocurrir en cualquier edad, siendo infrecuente en pediatría. La etiología y estudio diagnóstico del SH pediátrico (SHP) es motivo de controversia. ObjetivoDescribir las características clínicas de una serie de 14 niños diagnosticados de SH, incidiendo en la etiología del SH y en la evolución clínica que presentaron. Métodos Estudio observacional retrospectivo de pacientes menores de 14 años diagnosticados de SHP en nuestro centro entre el 01 de enero del 2009 y el 30 de abril del 2020. En función de la edad al diagnóstico, los casos se dividieron en congénitos (antes de los cinco meses) y adquiridos. Resultados Se reclutaron 14 pacientes, con una mediana de edad al diagnóstico de 8,5 meses. La causa más frecuente de SHP fue tumoral (6/14), siendo la neoplasia más representativa el neuroblastoma (4/14). De los casos adquiridos (8/14), la causa más frecuente fue iatrogénica (5/8), secundario a cirugía cérvico-torácica. La etiología principal del SH congénito (6/14) fue el neuroblastoma (4/6), siendo la primera manifestación clínica de la enfermedad en el 50% de los pacientes (2/4). Conclusiones El SH puede ser el primer signo de una enfermedad subyacente grave, como es el neuroblastoma. Por este motivo, es necesario realizar un adecuado estudio de extensión en todos los pacientes pediátricos diagnosticados de SH sin una causa clara atribuible. Es fundamental un examen riguroso para un diagnóstico precoz de estos pacientes (AU)

Background Horner syndrome (HS) is characterised by the triad of upper eyelid ptosis, miosis, and facial anhidrosis. Due to its wide variety of causes, it can occur at any age, and is uncommon in paediatrics. The aetiology and diagnostic approach of paediatric HS (PHS) is controversial. ObjectiveThe purpose of this study is to describe the clinical characteristics of a 14 case series, focusing on the aetiology of HS and the clinical evolution the patients presented. Methods A retrospective observational study was conducted on patients under 14 years-old (enrolled between 1 st January 2009 and 30th April 2020). Depending on the age at diagnosis (before or after the first 5 months of life), the study cases were divided into two groups: congenital or acquired. Results Fourteen patients, with a mean age of 8.5 months, were enrolled. The most frequent cause of PHS were tumours (6/14), with the most representative neoplasm being neuroblastoma (4/14). Of the acquired cases (8/14), the most frequent cause was iatrogenic (5/8), mainly secondary to cervical or thoracic surgery. The main origin of congenital HS (6/14) was neuroblastoma (4/6), being the first manifestation of the disease in 50% of patients (2/4). Conclusion HS may be the first sign of a major underlying disease, such as neuroblastoma. For this reason, children presenting with HS of unknown origin require imaging studies to exclude a life threatening disease. A thorough examination is essential for early diagnosis of these patients. (AU)

Paediatric Horner syndrome. A case series of 14 patients in a tertiary hospital.

BACKGROUND: Horner syndrome (HS) is characterised by the triad of upper eyelid ptosis, miosis, and facial anhidrosis. Due to its wide variety of causes, it can occur at any age, and is uncommon in paediatrics. The aetiology and diagnostic approach of paediatric HS (PHS) is controversial. OBJECTIVE: The purpose of this study is to describe the clinical characteristics of a 14 case series, focusing on the aetiology of HS and the clinical evolution the patients presented. METHODS: A retrospective observational study was conducted on patients under 14 years-old (enrolled between 1st January 2009 and 30th April 2020). Depending on the age at diagnosis (before or after the first 5 months of life), the study cases were divided into two groups: congenital or acquired. RESULTS: Fourteen patients, with a mean age of 8.5 months, were enrolled. The most frequent cause of PHS were tumours (6/14), with the most representative neoplasm being neuroblastoma (4/14). Of the acquired cases (8/14), the most frequent cause was iatrogenic (5/8), mainly secondary to cervical or thoracic surgery. The main origin of congenital HS (6/14) was neuroblastoma (4/6), being the first manifestation of the disease in 50% of patients (2/4). CONCLUSION: HS may be the first sign of a major underlying disease, such as neuroblastoma. For this reason, children presenting with HS of unknown origin require imaging studies to exclude a life threatening disease. A thorough examination is essential for early diagnosis of these patients.