The multidisciplinary challenge of anesthesia for ex utero intrapartum treatment: a case report / O desafio multidisciplinar da anestesia para procedimento intraparto extra-uterino: relato de caso

Abstract The Ex Utero Intrapartum Treatment (EXIT) is a surgical procedure performed in cases of expected postpartum fetal airway obstruction, allowing the establishment of patent airway while maintaining placental circulation. Anesthesia for EXIT procedure has several specific features such as adequate uterine relaxation, maintenance of maternal blood pressure fetal anesthesia and fetal airway establishment. The anesthesiologist should be aware of these particularities in order to contribute to a favorable outcome. This is a case report of an EXIT procedure performed on a fetus with a cervical lymphangioma with prenatal evidence of partial obstruction of the trachea and risk of post-delivery airway compromise.

Resumo O procedimento Intraparto Extra-Uterino (EXIT) é procedimento cirúrgico realizado em casos de previsão de obstrução de via aérea fetal no pós-parto, que permite estabelecer via aérea patente enquanto a circulação placentária é mantida. A anestesia para o procedimento EXIT apresenta várias características específicas, tais como relaxamento uterino adequado, manutenção da pressão arterial materna, anestesia fetal e estabelecimento da via aérea fetal. O anestesiologista deve estar ciente dessas especificidades para contribuir para desfecho favorável. Trata-se de relato de caso de procedimento EXIT realizado em feto com linfangioma cervical e evidência pré-natal de obstrução parcial de traqueia e risco de comprometimento de via aérea pós-parto.

Anesthesia for ex utero intrapartum treatment: renewed insight on a rare procedure / Anestesia para tratamento ex-útero intraparto: visão renovada sobre um procedimento raro

The ex utero intrapartum treatment is a rare surgical procedure performed in cases of expected postpartum fetal airway obstruction. The technique lies on a safe establishment of a patent airway during labor in anticipation of a critical respiratory event, without interrupting maternal-fetal circulation. Anesthetic management is substantially different from that regarding standard cesarean delivery and its main goals include uterine relaxation, fetal anesthesia and preservation of placental blood flow. We present the case of an ex utero intrapartum treatment procedure performed on a fetus with a large cervical lymphangioma and prenatal evidence of airway compromise. Modifications to the classic ex utero intrapartum treatment management strategies were successfully adopted and will be discussed in the following report.

O tratamento ex-útero intraparto é um procedimento cirúrgico feito em casos raros de obstrução esperada das vias aéreas fetais no pós-parto. A técnica tem como base o estabelecimento seguro de vias aéreas permeáveis durante o trabalho de parto em antecipação a um evento respiratório crítico, sem interromper a circulação materno-fetal. O manejo anestésico é substancialmente diferente daquele destinado à cesariana padrão e tem como principais objetivos o relaxamento uterino, a anestesia fetal e a preservação do fluxo sanguíneo placentário. Apresentamos o caso de um procedimento para tratamento ex-útero intraparto feito em feto com um grande linfangioma cervical e evidência pré-natal de comprometimento das vias aéreas. As modificações das estratégias adotadas no tratamento ex-útero intraparto clássico foram feitas com sucesso e serão discutidas no relato a seguir.

Surgical Treatment of Difficult Cervicofacial Lymphangioma in Children / 소아외과

PURPOSE: Cervical lymphangiomas are rare lymphovascular malformations arising in the neck, which form huge fluid-containing cysts. Treatment of the malformation consists of surgery and sclerotherapy. However, the optimal approach is still controversial. Here, we describe a series of cervical lymphangiomas which have been treated with surgical approaches. METHODS: We retrospectively investigated the medical records of 82 patients who had been diagnosed with cervicofacial lymphangioma from 2001 to 2012 in our center. A closed suction drainage with negative pressure was placed on the operative lesion following excision to prevent reaccumulation of lymphatic fluid and the drainage tube was removed after injecting OK-432 through the tube. RESULTS: Twelve patients underwent surgical excision of cervical lymphangioma. The median patient age was 3 months at the time of the operation. The patients have been followed-up over a period of 34 months. When lesions were located near vital organs such as the trachea or carotid artery or did not respond to repetitive OK-432 injections, surgical treatment might bring good outcomes. However, swallowing difficulty, lip palsy, or dyslalia due to adjacent nerve damage temporarily appeared as postoperative complications. Five children had tracheostomy due to tracheal or subglottic stenosis and 2 patients had gastrostomy due to aspiration while they eat after surgery. CONCLUSION: Surgery for cervicofacial lymphangioma should be conducted carefully in selective cases. A well thought-out surgical plan with a multidisciplinary surgical team approach and placement of closed suction drainage tube after surgery and adjuvant OK-432 sclerotherapy through drainage tube seem to be helpful for good outcome.

E X I T (ex-utero intrapartum therapy) en linfangioma cervical fetal / EXIT procedure (ex-utero intra-partum therapy) for fetal neck lymphangioma

Se presenta un caso clínico de una embarazada primigesta de 17 años, con un feto con gran masa cervical a las 20 semanas, se diagnostica como linfangioma cervical. La evaluación prenatal concluye que existe gran riesgo de asfixia perinatal por obstrucción de la vía aérea superior, se resuelve el parto mediante procedimiento EXIT (ex-utero intrapartum therapy) a las 37 semanas. Se logra realizar intubación con larin-goscopia directa, con un tiempo de by-pass uteroplacentario de 7 minutos. Se obtiene un recién nacido de 3300 g, al segundo día se opera del tumor con buenos resultados. Se revisa el protocolo del procedimiento EXIT en sus aspectos anestésicos, obstétricos, quirúrgicos y neonatológicos. Se concluye que el EXIT debe ser planteado en todo caso en que se sospeche obstrucción de la vía aérea superior y puede ser realizado en hospitales que cuenten con equipamiento habitual y un equipo médico multidisciplinario.

We report a case of primigravida patient, 17 years old, with a fetus showing a large cervical mass at 20 weeks of gestation and was diagnosed as a cervical lymphangioma. The prenatal evaluation concludes that there exists a great risk of perinatal asphyxia due to obstruction of the upper airway and therefore it is decided to perform a cesarean section at 37 weeks of gestation, using an EXIT procedure (ex-utero intra-partum therapy). We perform intubation with a semi- rigid tube having a by-pass time utero-placental of 7 minutes, obtaining a newborn of 3300 g at birth. The newborn is operated two days after birth removing the cervical tumor with good results. We review the protocol of the EXIT procedure concerning aspects related to anesthesia, obstetrics, surgery and neonatal care. We conclude that EXIT should be considered in all cases in which obstruction of the upper airway is suspected, and can be performed in hospitals that have basic surgical facilities and a multidisciplinary team.

Linfangioma cervical fetal: diagnóstico prenatal y resultado perinatal

Se presenta el caso de un linfangioma cervical fetal, tumor de presentación infrecuente y potencial emergencia perinatológica, en relación al compromiso de las vías aéreas fetales. La ecografía y la resonancia nuclear magnética (RMN) son las herramientas para establecer el diagnóstico y pronóstico perinatal de los fetos portadores de linfangioma cervical, así como de otros tumores cervicales. El procedimiento EXIT coordinado con un equipo multidisciplinario debe ser planificado en casos en que el feto presenta un riesgo alto de obstrucción de la vía aérea.

A case of fetal cervical lymphangioma is reported, tumor of infrequent presentation and potential perinatologic emergency in relation to superior fetal airways compromise. Ultrasound and nuclear magnetic resonance are the main tools to establish diagnosis and perinatal prognosis of fetuses carrying cervical lymphangioma as well as other cervical tumors. EXIT procedure coordinated with a multidisciplinary equipment must be planned in cases when the fetus displays a high risk of upper airway obstruction.