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      LINFANGIOMA CERVICAL CAVERNOSO EN UN GEMELO: ANÁLISIS CRÍTICO DEL DIAGNÓSTICO Y MANEJO PERINATAL

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          Abstract

          Los tumores fetales son hallazgos infrecuentes y muchas veces no diagnosticados prenatalmente. El objetivo de este trabajo es presentar y discutir en forma critica el diagnostico y manejo prenatal de un gemelo portador en un linfangioma cervical cavernoso, incluyendo el procedimiento EXIT

          Translated abstract

          Fetal cervical tumors are uncommon, and not always prenataly diagnosticated. We describe the prenatal findings and prenatal management in a case of cervical cavernous lymphangioma in one twin, including EXIT procedure

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          The EXIT procedure: experience and outcome in 31 cases.

          The EXIT (ex utero intrapartum treatment) procedure, although initially designed for reversal of tracheal occlusion in fetuses with congenital diaphragmatic hernias (CDH), has been adapted to treat a variety of fetal conditions. A retrospective chart review of all consecutive EXIT procedures since 1996 was conducted. Thirty-one women underwent the EXIT procedure, with an average maternal age of 29 years (range, 20 to 38), and average gestational age of 34 weeks (range, 29 to 40). The indication was airway obstruction from fetal neck mass in 13, and reversal of tracheal occlusion from in utero clipping in 13. Singular indications included an EXIT-to-ECMO (extracorporeal membrane oxygenation) procedure for a fetus with CDH and a cardiac defect (n = 1), congenital high airway obstruction syndrome (CHAOS, n = 1), resection of a very large congenital cystic adenomatoid malformation of the lung (CCAM) on uteroplacental bypass (n = 1), unilateral pulmonary agenesis (n = 1), and thoracoomphalopagus conjoined twins. The mean duration on uteroplacental bypass (from uterine incision to umbilical cord clamping) was 30.3 plus minus 14.7 minutes (range, 8 to 66). No fetus experienced hemodynamic instability during uteroplacental bypass as recorded by fetal heart rate (FHR), pulse oximeter, and fetal echocardiography, except for one instance of reversible bradycardia from umbilical cord compression. The mean FHR and fetal saturation were 153.0 plus minus 38.5 beats per minute and 71.2% plus minus 19.9%, respectively. Five fetuses required a tracheostomy. Only 1 death occurred during an EXIT procedure because of inability to secure the airway secondary to extensive involvement by a lymphangioma. The average cord pH and pCO(2) were, respectively, 7.20 plus minus 0.11 and 63.2 plus minus 14.6. Two maternal complications occurred: bleeding from a hysterotomy site and dehiscence of an old hysterotomy scar noticed at a subsequent cesarean section. The average maternal blood loss was 848.3 plus minus 574.1 mL. The EXIT procedure was used successfully to ensure uteroplacental gas exchange and fetal hemodynamic stability during a variety of surgical procedures performed to secure the fetal airway or ensure successful transition to postnatal environment. Copyright 2002 by W.B. Saunders Company.
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            The ex utero intrapartum treatment procedure: Looking back at the EXIT.

            The ex utero intrapartum treatment (EXIT) procedure was developed originally for management of airway obstruction after fetal surgery, and indications have continued to expand for a variety of fetal anomalies. The authors review their single-institution experience with EXIT. Retrospective review of all patients who underwent an EXIT procedure from 1993 to 2003 (n = 52) was performed. Variables evaluated include indication for EXIT, gender, gestational age at EXIT, birth weight, maternal blood loss, operative complications, operative time, and survival rate. Technique, personnel, and anesthesic management were reviewed. Long-term follow-up was available for all patients. Fifty-one of 52 patients were born alive; currently, 27 of 52 patients (52%) are alive. All deaths have been in patients with congenital diaphragmatic hernia. Forty-five patients underwent EXIT for reversal of tracheal occlusion for congenital diaphragmatic hernia. Of these patients, 30 underwent tracheal clip removal. Two patients had repair of tracheal injury from clipping at EXIT. Fifteen patients underwent bronchoscopy and tracheal balloon removal. Five patients underwent EXIT procedure for neck masses. Tracheostomy was performed in 3 of these patients. One patient was intubated successfully, and 1 patient underwent resection of the neck mass while on placental support. Two patients underwent EXIT procedure and tracheostomy for congenital high-airway obstruction syndrome. Average gestational age at delivery was 31.95 +/- 2.55 weeks. Average birth weight was 1,895 +/- 653 g. Average maternal blood loss was 970 +/- 510 mL. Average operating time on placental support was 45 +/- 25 minutes with a maximum of 150 minutes. EXIT procedures can be performed with minimal maternal morbidity and with good outcomes. It is an excellent strategy for establishing an airway in a controlled manner, avoiding "crash" intubation or tracheostomy. Longer procedures on placental support allowing for definitive management of neck masses and airway obstruction have been realized. EXIT procedures have evolved from an adjunct to fetal surgery to a potentially life-saving procedure in fetuses with airway compromise at birth.
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              The fetal circulation and essential organs-a new twist to an old tale.

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                Author and article information

                Journal
                rchog
                Revista chilena de obstetricia y ginecología
                Rev. chil. obstet. ginecol.
                Sociedad Chilena de Obstetricia y Ginecología (Santiago, , Chile )
                0048-766X
                0717-7526
                2007
                : 72
                : 5
                : 334-341
                Affiliations
                [04] orgnameHospital Panguipulli Chile
                [03] orgnameHospital Regional de Valdivia orgdiv1Unidad de Pabellón y Anestesia Chile
                [01] orgnameUniversidad Austral de Chile orgdiv1Instituto de Obstetricia y Ginecología Chile
                [02] orgnameHospital Regional de Valdivia orgdiv1Servicio de Pediatría Chile
                [05] orgnamelmagenología Valdivia Chile
                Article
                S0717-75262007000500010 S0717-7526(07)07200510
                10.4067/S0717-75262007000500010
                8bbfff14-8f87-4c07-b77c-107037548a5f

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 20, Pages: 8
                Product

                SciELO Chile

                Categories
                Caso Clínico

                prenatal diagnosis,ultrasonography,EXIT,Linfangioma cervical,diagnóstico prenatal,fetal cervical tumor,Cervical limphangyoma,magnetic resonance,ultrasonografía,tumor cervical fetal,doppler,resonancia magnética

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