RESUMEN
La pseudoartrosis congénita de la clavícula es una malformación rara y benigna, caracterizada por la ausencia del tercio medio de la clavícula. Suele ser unilateral y mayoritaria en el lado derecho. La etiología es desconocida y se postulan diversas teorías etiopatogénicas (vascular, embriológica y genética).Puede detectarse en el período neonatal o, más frecuentemente, durante la infancia. En ocasiones, puede ser sintomática. Puede requerir tratamiento mediante reconstrucción quirúrgica por injerto óseo.Se presentan 2 casos, uno de diagnóstico neonatal y otro de 3 años de edad realizados con 24 h de diferencia. Se destaca la consideración de este diagnóstico como diferencial de fractura obstétrica o postraumática, displasia cleidocraneal y neurofibromatosis de tipo 1.
The congenital pseudoarthrosis of the clavicle is a rare and benign malformation, characterized by the absence of the middle third of the clavicle. It is usually unilateral and the majority on the right side. The etiology is unknown, postulating diverse etiopathogenic theories (vascular, embryological and genetic).It can be detected in the neonatal period or, more frequently, during childhood. Occasionally it can be symptomatic. It may require treatment by surgical reconstruction by bone graft. Two cases are presented, one of neonatal diagnosis and another one of 3 years of age performed with 24 hours of difference. We emphasize on its consideration as a differential diagnosis of obstetric or post-traumatic fracture, cleidocranial dysplasia and neurofibromatosis type I.
Asunto(s)
Humanos , Femenino , Recién Nacido , Preescolar , Seudoartrosis/congénito , Clavícula/anomalías , Seudoartrosis/diagnóstico por imagen , Anomalías Congénitas , Diagnóstico DiferencialRESUMEN
The congenital pseudoarthrosis of the clavicle is a rare and benign malformation, characterized by the absence of the middle third of the clavicle. It is usually unilateral and the majority on the right side. The etiology is unknown, postulating diverse etiopathogenic theories (vascular, embryological and genetic). It can be detected in the neonatal period or, more frequently, during childhood. Occasionally it can be symptomatic. It may require treatment by surgical reconstruction by bone graft. Two cases are presented, one of neonatal diagnosis and another one of 3 years of age performed with 24 hours of difference. We emphasize on its consideration as a differential diagnosis of obstetric or post-traumatic fracture, cleidocranial dysplasia and neurofibromatosis type I.
La pseudoartrosis congénita de la clavícula es una malformación rara y benigna, caracterizada por la ausencia del tercio medio de la clavícula. Suele ser unilateral y mayoritaria en el lado derecho. La etiología es desconocida y se postulan diversas teorías etiopatogénicas (vascular, embriológica y genética). Puede detectarse en el período neonatal o, más frecuentemente, durante la infancia. En ocasiones, puede ser sintomática. Puede requerir tratamiento mediante reconstrucción quirúrgica por injerto óseo. Se presentan 2 casos, uno de diagnóstico neonatal y otro de 3 años de edad realizados con 24 h de diferencia. Se destaca la consideración de este diagnóstico como diferencial de fractura obstétrica o postraumática, displasia cleidocraneal y neurofibromatosis de tipo 1.
Asunto(s)
Clavícula/anomalías , Seudoartrosis/congénito , Preescolar , Clavícula/diagnóstico por imagen , Diagnóstico Diferencial , Femenino , Humanos , Recién Nacido , Seudoartrosis/diagnóstico por imagen , RadiografíaRESUMEN
BACKGROUND: Pseudomyxoma peritonei (PMP) is a rare, slowly progressive disease whose prognosis depends primarily on the completeness of cytoreduction. The value of intraoperative hyperthermic intraperitoneal chemotherapy (HIPEC) and of additional factors predicting long-term outcome and disease-free survival (DFS) remains poorly understood. This study aims to analyse survival rates and prognostic factors in patients undergoing maximal cytoreduction and HIPEC. METHODS: Thirty patients were selected from a prospective database of records for patients undergoing cytoreduction and HIPEC with mitomycin C or paclitaxel. Overall survival (OS), DFS, and the prognostic factors influencing them, were examined using multivariate analysis. RESULTS: Median follow-up was 44 months (range, 8-144). Histological classification of PMPs was DPAM in 6/30 of cases, PMCA-I in 10/30 and PMCA in 14/30. Complete cytoreduction (CC-0 and CC-1) was achieved in 28/30 of patients and CC-2 in 2/30. Median OS was 111 months (range 0-230) and five-year OS rate was 67%. Median DFS was 53.5 months (range 0-120) and 5-year DFS rate was 44%. Incomplete cytoreduction, lymph node involvement and PCI>20 were associated with poor prognosis for OS, while lymph node involvement, elevated CA-125 levels, unfavourable histology and previous chemotherapy were associated with poor outcomes for DFS. There was morbidity of Grade 3 or higher in 9/30. Post-operative mortality occurred in 1 case. CONCLUSION: Cytoreduction plus peritonectomy procedures combined with HIPEC is a safe treatment and could improve survival rates. Since the optimal cytoreduction is the primary prognostic factor, patients should be centralised under the care of experienced teams.