º» »çÀÌÆ®ÀÇ ³»¿ëÀº ¼Ò¾Æ¿Ü°úÀÇ»ç ÇÑ ¼®ÁÖÀÇ °ÀÇ·Ï ³»¿ëÀÔ´Ï´Ù.
µû¶ó¼ º»ÀÎÀÇ Çã¶ô ¾øÀÌ ºÒ¹ýÀ¸·Î º¹»ç »ç¿ëÇÏ´Â °ÍÀº ¹ýÀû Ã¥ÀÓÀ» Áú ¼ö ÀÖ½À´Ï´Ù.
žƼö¼ú(Fetal Surgery)
¿¬¼¼ÀÇ´ë ¼Ò¾Æ¿Ü°ú 25Áֳ⠽ÉÆ÷Áö¿òÀÇ °ÀÇ·Ï
2000. 3
¿¬¼¼ÀÇ´ë ¿Ü°úÇб³½Ç ¼Ò¾Æ¿Ü°ú ÇÑ ¼®ÁÖ
1. ¼·Ð
ÃÖ±Ù µé¾î žÆÀÇ ±¸Á¶Àû ±âÇüÀ» Áø´ÜÇÏ´Â
¹æ¹ýÀº ÃÊÀ½ÆÄ¿Í MRIÀÇ ¹ßÀü¿¡ ÈûÀÔ¾î ºñ¾àÀûÀ¸·Î ¹ßÀüÇÏ¿´´Ù. ÀÌ·¯ÇÑ »êÀü Áø´Ü±â¼úÀÇ
¹ß´Þ·Î ¿ì¸®´Â žÆÀÇ ±¸Á¶Àû °áÇÔ¿¡ ´ëÇÑ º´Å »ý¸®¿¡ ´ëÇÑ °ü½ÉÀÌ Ä¿Áö°Ô µÇ¾úÀ¸¸ç
ÀÌ¿¡ ´ëÇÑ Áö½Äµµ ³Ð¾îÁø °ÍÀº »ç½ÇÀÌ´Ù. ÀÌ·Î ÀÎÇÏ¿© ¸¹Àº ¼±Ãµ¼º ±âÇüÀ» °¡Áø žƿÍ
»ê¸ð°¡ ÀûÀýÇÑ Ä¡·á°¡ °¡´ÉÇÑ ÀÇ·á½Ã¼³·Î Èļ۵Ǿî Àü¹® ÀÇ·áÀÎÀÇ »ó´ãÀ» ¹Þ°í Ãâ»ýÇÏ¿©
ÃÖ»óÀÇ Á¶°Ç¿¡¼ Ä¡·á¸¦ ¹ÞÀ½À¸·Î½á °ú°Åº¸´Ù Ä¡·á¼ºÀûÀÌ Çâ»óµÈ °ÍÀº »ç½ÇÀÌ´Ù.
±×·¯³ª ¾ÆÁ÷µµ ¼±Ãµ¼º Ⱦ°æ¸· Å»Àå °°Àº ÀϺΠ¼±Ãµ¼º ÁúȯÀÇ °æ¿ì¿¡´Â Ãâ»ý ÈÄ ÃÖ»óÀÇ
Ä¡·á¿¡µµ ºÒ±¸ÇÏ°í ³ôÀº »ç¸Á·üÀ» º¸ÀÌ°í ÀÖ´Ù. ÀÌ´Â À̵é ÁúȯÀÇ °æ¿ì ¼±Ãµ¼º ±¸Á¶Àû
°áÇÔÀÌ Å¾ƽñ⿡ Áß¿ä Àå±â(¼±Ãµ¼º Ⱦ°æ¸· Å»ÀåÀÇ °æ¿ì¿¡´Â Æó)ÀÇ ¹ß´ÞÀ» ½ÉÇÏ°Ô
¾ïÁ¦ÇÏ°Ô µÇ°í µû¶ó¼ Ãâ»ý ÈÄ ¾Æ¹«¸® ÃÖ»óÀÇ Ä¡·á¸¦ ÇÏ´õ¶óµµ »êÀü¿¡ ÀÌ¹Ì Çü¼ºµÈ
Àå±âÀÇ ¹ß´ÞºÎÀüÀ» ±Øº¹ÇÏÁö ¸øÇϱ⠶§¹®ÀÌ´Ù. ÀÌ·± ¹®Á¦¸¦ ÇØ°áÇÏ°íÀÚ ÇÏ´Â ¾ÆÀ̵ð¾î·Î
¿¬±¸°¡ ½ÃÀÛµÈ ºÐ¾ß°¡ ¹Ù·Î ÅÂ¾Æ ¼ö¼úÀÌ´Ù. °ú°Å ¾à 20³â°£ÀÇ ¼±Áø±¹ÀÇ ½ÇÆÐ¿Í ¼º°øÀ»
°ÅµìÇϴ žƼö¼ú¿¡ ´ëÇÑ ¿¬±¸¿Í ÀÓ»ó½Ãµµ¸¦ ÅëÇÏ¿© ÀϺΠ¼±Ãµ¼º ±âÇü¿¡¼´Â žÆ
¼ö¼úÀÌ ¼º°øÀûÀ¸·Î ½ÃÇàµÇ°Ô µÇ¾úÀ¸¸ç ÇöÀç ±× °á°úµµ °ý¸ñÇÏ°Ô Çâ»óµÇ°Ô µÇ¾ú´Ù.
ÀÌ¿¡ º» ¿¬Á¦¿¡¼´Â ÇöÀç ÀÓ»ó Àû¿ëÀÌ ½ÃÇàµÇ°í ÀÖ´Â ÅÂ¾Æ ¼ö¼úÀ» ¼Ò°³ÇÏ°íÀÚ ÇÑ´Ù.
2. ÅÂ¾Æ ¼ö¼úÀÇ °³³ä
ÅÂ¾Æ ¼ö¼úÀ̶õ ¼±ÃµÀûÀΠžÆÀÇ ±¸Á¶Àû °áÇÔÀ»
Àڱà ³»¿¡¼ ¿Ü°úÀûÀ¸·Î ±³Á¤ÇÏ°í ³²Àº ¼öűⰣÀ» À¯Áö ÈÄ ºÐ¸¸ÇÏ´Â ¿Ü°úÀû Ä¡·á¹æ¹ýÀÌ´Ù.
±× ¸ñÀûÀº ÅÂ¾Æ ¼ö¼úÀ» ÅëÇÏ¿© žÆÀÇ ±¸Á¶Àû °áÇÔÀÌ ÃÊ·¡ÇÏ´Â Àå±âÀÇ ¹ß´Þ Àå¾Ö¸¦
Àڱà ³»¿¡¼ ¿¹¹æÇÏ°í ±³Á¤ÇÏ´Â °ÍÀÌ´Ù.
3. ÀÓ»ó Àû¿ëÀÌ µÇ°í Àִ žƼö¼úÀÇ ´ë»ó Áúȯ
ÅÂ¾Æ ¼ö¼úÀ̶õ žƿ¡°Ô¸¸ ħ½ÀÀû ¼ú½ÄÀ»
½ÃÇàÇÏ´Â °ÍÀÌ ¾Æ´Ï¶ó ¸ðü¿¡µµ »ó´çÈ÷ ºÎ´ãÀÌ µÇ´Â ħ½ÀÀû ¼ú½ÄÀ» ½ÃÇàÇÏ°Ô µÈ´Ù.
µû¶ó¼ ¸ðü¿¡ ´ëÇÑ ºÒÀÌÀÍ°ú ¼ö¼ú·Î½á ¾ò´Â žÆÀÇ ÀÌÀÍ¿¡ ´ëÇÑ ÃæºÐÇÑ °í·Á°¡ žÆ
¼ö¼úÀÇ ´ë»óÀ» ¼³Á¤ÇÔ¿¡ »ý°¢ÇÏ¿©¾ß ÇÒ Áß¿äÇÑ »çÇ×ÀÌ´Ù. ¶ÇÇÑ ÅÂ¾Æ ¼ö¼úÀÌ ¸ðüÀÇ
´ÙÀ½ Àӽſ¡ ºÒÀÌÀÍÀ» ¹ÌÃļ´Â ¾ÈµÇ°Ú´Ù. »êÀü Áø´ÜµÈ ¼±Ãµ¼º ÁúȯÀ» Ä¡·á ¹æħ¿¡
µû¶ó¼ ºÐ·ùÇϸé Ç¥ 1°ú °°´Ù. À̸¦ »ìÆ캸¸é 1) bilateral renal agenesis³ª anencephaly°°ÀÌ
Ãâ»ý ÈÄ »ç¸ÁÇÒ ¼ö¹Û¿¡ ¾ø´Â ÁúȯÀº À¯»êÀÇ ´ë»óÀÌ µÇ¾î¾ß ÇÒ °ÍÀ̸ç, 2) esopahgeal
atresia¿Í °°Àº ÁúȯÀº ÀÓ½ÅÀ» Áö¼ÓÇÏ°í Á¤»ó ºÐ¸¸ÈÄ ±³Á¤ÇÏ¿©¾ß ÇÒ °ÍÀ̸ç, 3) sacrococcygeal
teratoma°°ÀÌ »êµµ¸¦ Åë°úÇÏ´Â µ¿¾È Á¾±«°¡ ÆÄ¿µÇ°í Á¤»ó Áú½Ä ºÐ¸¸ÀÌ Èûµç °æ¿ì¿¡´Â
ÀÓ½ÅÀ» Áö¼ÓÇÑ ÈÄ Á¦¿Õ Àý°³¼úÀÌ ¼±ÅõǾî¾ß ÇÒ °ÍÀ̸ç, 4) urinary tract obstruction
°°ÀÌ Á¡ÁøÀûÀ¸·Î Àå±âÀÇ ¹Ì¼º¼÷À» ÃÊ·¡ÇÏ´Â °æ¿ì¿¡´Â ºÐ¸¸ ÈÄ »ýÁ¸ÀÌ °¡´ÉÇÏ´Ù°í
ÆÇ´ÜµÇ¸é °¡´ÉÇÑ »¡¸® ºÐ¸¸ÇÏ¿© À̸¦ ±³Á¤ÇÏ¿©¾ß ÇÒ °ÍÀÌ´Ù. ±×·¯³ª ÀÓ½ÅÀÇ Áö¼ÓÀÌ
Àå±âÀÇ ½É°¢ÇÑ ¹Ì¼º¼÷À» ÃÊ·¡ÇÏ¿© Ãâ»ý ÈÄ »ýÁ¸ÀÌ Èûµé´Ù°í ÆǴܵǸç Àڱà ³» ¼ö¼ú·Î½á
Àå±âÀÇ ¹Ì¼º¼÷À» ¿¹¹æÇÏ¿© Ãâ»ý ÈÄ »ýÁ¸ÀÌ °¡´ÉÇÑ ÁúȯÀ̶ó°í ÆÇ´ÜµÉ °æ¿ì¿¡´Â žÆ
¼ö¼úÀÇ ´ë»óÀÌ µÈ´Ù(5). À̵éÀ» ´ë»óÀ¸·Î ÇÏ´Â ÅÂ¾Æ ¼ö¼úÀº º¸Åë ÀӽŠÁ¦ 18ÁÖ¿¡¼
30ÁÖ¿¡ ½ÃÇàµÇ°Ô µÈ´Ù.
Prenatal Diagnosis: Fetal Malformation 1. Incompatible with Life: Abort anencephaly, bilateral renal agenesis 2. Correct at Term: Wait esophageal atresia 3. Dystocia: C-Section very large tumor that might break during vaginal delivery 4. Progressive Ill Effect: Delivery Early urinary tract obstruction that is causing ongoing damage of kidney and lungs 5. Prevent Development: Correct in Utero (Fetal Surgery) congenital diaphragmatic hernia(CDH) lung hypoplasia ------> pulmonary failure congenital cystic adenomatoid malformation(CCAM) lung hypoplasia ------> fetal hydrops urinary obstruction: urethral valve hydronephrosis ------> renal failure lung hypoplasia ------> pulmonary failure sacrococcygeal teratoma high output failure ------> fetal hydrops twin twin transfusion syndrome (TTTS) vascular steal ------> fetal
hydrops through placenta |
4. ¿¬±¸ ´ë»óÀ¸·ÎÀÇ ÅÂ¾Æ ¼ö¼úÀÇ ´ë»ó Áúȯ
žƴ Ãâ»ý ÈÄ¿Í´Â ´Ù¸¥ µ¶Æ¯ÇÑ º´Å »ý¸®Àû
±âÀüÀ» °¡Áö°í ÀÖÀ¸¸ç À̸¦ Àß ÀÌ¿ëÇϸé ÀÓ»óÀûÀ¸·Î ÇØ°áµÇÁö ¾Ê´Â ¸¹Àº ¹®Á¦¸¦ ÇØ°á
ÇÒ ¼ö°¡ ÀÖ´Ù. ¾ÆÁ÷ ÀÓ»ó¿¡´Â º»°ÝÀûÀ¸·Î Àû¿ëµÇ°í ÀÖÁö´Â ¾ÊÀ¸³ª ÇöÀç È°¹ßÇÏ°Ô
¿¬±¸µÇ°í ÀÖ´Â ÁúȯÀº Ç¥ 2¿Í °°´Ù. ÀÌÁß meningomyoceleÀº ÃÖ±Ù ÅÂ¾Æ ¼ö¼úÀÇ ¾ÈÁ¤¼ºÀÌ
³ô¾ÆÁö¸é¼ ÀÓ»ó ¿¹°¡ ´Ã¾î³ª°í ÀÖ´Â Ãß¼¼ÀÌ´Ù.
1. Meningomyocele 2. Cleft Lip and Palate 3. Fetal Stem Cell Transplantation to treat hemoglobinopathy(i.e. thalassemia, sickle cell anemia) and immune deficiences 4. Hydrocephalus |
5. History of Fetal Surgery (University of California, San Francisco, UCSF)
1981: First open fetal surgery for urinary tract obstruction, urinary catheter for posterior urethral valve
1989: First successful open fetal surgery for CDH
1990: Uterine stapling device
Radiotelemeter (NASA's Sensor 2000 Program)
1990: Successful resection of congenital cystic adenomatoid malformation
1992: Successful resection of sacrococcygeal teratoma
1993-1996: NIH clinical tracheal occlusion (int. plug and ext. clip)
Fetal endoscopic tracheal clip (Fetendo clip)
1999: Fetendo intratracheal balloon
6. Maternal Fetal Intensive Care
ÅÂ¾Æ ¼ö¼ú Áß¿¡ žÆÀÇ È°·Â ¡Èĸ¦ monitorÇÏ´Â ¹æ¹ýÀ¸·Î´Â pulse oximeter°¡ À¯¿ëÇÏ°Ô ¾²À̸ç À̸¦ ÅëÇÏ¿© žÆÀÇ ½É¹Úµ¿¼ö¿Í »ê¼Ò ºÐ¾ÐÀÇ ÃøÁ¤ÀÌ °¡´ÉÇÏ´Ù(±×¸² 1).
Fig. 1. Intraoperative monitoring |
Fig. 2 Implantation of Radiotelemeter |
¶ÇÇÑ ¼ö¼ú Áß ½ÃÇàÇÏ´Â ÃÊÀ½Æĵµ žÆÀÇ ½ÉÀåÀÇ
»óŸ¦ °¨½ÃÇϴµ¥ À¯¿ëÇÏ°Ô »ç¿ëµÇ¾îÁø´Ù. ¼ö¼ú ÈÄ¿¡µµ Áö¼ÓÀûÀÎ ÅÂ¾Æ¿Í Àڱÿ¡
´ëÇÑ °¨½Ã°¡ ÇÊ¿äÇѵ¥ ÀÌ´Â ¼ö¼úÁß Å¾ƿ¡ »ðÀÔÇÏ¿© µÎ´Â radiotelemeter¿¡¼ ³ª¿À´Â
ÀüÆĸ¦ ¸ðü ¿ÜÀÇ receiver¸¦ ÀÌ¿ëÇÏ¿© ¹Þ¾Æ¼ žÆÀÇ ½ÉÀüµµ, ü¿Â, ÀڱüöÃàÀÇ
µ¥ÀÌÅ͸¦ ¾òÀ» ¼ö°¡ ÀÖ´Ù(±×¸² 2). ¼ö¼úÁ÷ÈÄ »ê¸ð´Â ÀÌ·¯ÇÑ Àåºñ°¡ °®Ãß¾îÁø maternal
fetal intensive care unit(MF-ICU)¿¡¼ 1-2ÀÏ °¨½Ã¸¦ ¹Þ°Ô µÈ´Ù.
7. Preterm Labor after Fetal Surgery
ÅÂ¾Æ ¼ö¼ú¿¡ ÀÖ¾î¼ °¡Àå Å« ¹®Á¦À̸ç ÇöÀç±îÁö
ÇØ°áµÇÁö ¾Ê°í ÀÖ´Â ¹®Á¦´Â ÅÂ¾Æ ¼ö¼ú ÈÄ¿¡ 100% ¹ß»ýÇÏ´Â preterm laborÀÌ´Ù. ÀÌ´Â
ÀÌ½Ä ¼ö¼ú ÈÄ¿¡ º¼ ¼ö ÀÖ´Â °ÅºÎ¹ÝÀÀ¿¡ ºñÀ¯ ÇÒ ¼ö ÀÖ´Ù. À̸¦ ¹æÁöÇϱâ À§ÇÏ¿© ¼ö¼ú
ÈÄ ¿©·¯ tocolytic agent°¡ »ç¿ëµÇ¾î Áö°í ÀÖÀ¸¸ç °¡´ÉÇϸé open fetal surgery¸¦
ÇÇÇÏ°í ³»½Ã°æÀ» ÀÌ¿ëÇÑ endoscopic fetal surgery(fetendo)¸¦ ½ÃÇàÇÏ°í ÀÖ´Ù.
8. Fetus with Urinary Tract Obstruction
posterior urethral valve¿Í °°Àº urinary tract obstructionÀ» °¡Áø žÆÀÇ ÀϺδ hydronephrosis, renal dysplasia·Î ÀÎÇÑ renal failure¿Í lung hypoplasia·Î ÀÎÇÑ pulmonary failure°¡ ¹®Á¦°¡ µÇ¾î Ãâ»ýÈÄ »ç¸ÁÇÏ°Ô µÈ´Ù. ¾çÀÇ Å¾Ƹ¦ ÀÌ¿ëÇÑ urinary tract obstructionÀÇ µ¿¹°¸ðµ¨¿¡¼ ÀûÀýÇÑ ½Ã±â¿¡ urinary tract obstructionÀ» ÇØ°áÇØ ÁÖ´Â °æ¿ì¿¡´Â renal dysplasia¿Í lung hypoplasiaÀÇ °¡¿ªÀûÀÎ º¯È¸¦ º¼ ¼ö ÀÖ¾úÀ¸¸ç ÀÌ·¯ÇÑ º¯È´Â »ç¶÷ÀÇ Å¾ƿ¡¼µµ °üÂûÀÌ µÇ¾ú´Ù. ÇöÀç posterior urethral valve¿¡ ´ëÇÑ Å¾Ƽö¼úÀº ¹æ±¤¿¡ Harrison Double Pigtail Catheter¸¦ »ðÀÔÇϰųª(±×¸² 3) vesicostomy¸¦ Á÷Á¢ ½ÃÇàÇÏ°í ÀÖ´Ù. ÃÖ±Ù¿¡´Âfetal cystoscopy¸¦ ÅëÇÑ urethral valveÀÇ laser ablation ¹æ¹ýÀÌ ½ÃµµµÇ°í ÀÖ´Ù. ¼ö¼ú ´ë»ó žÆÀÇ ¼±ÅÃÀº (±×¸² 4)¿¡ µû¸¥´Ù.
Fig. 3 Harrison Double Pigtail Catheter |
Fig. 4 Algorithm for urinary tract obstruction |
9. Fetus with Congenital Cystic Adenomatoid Malformation(CCAM)
¾ÆÁÖ Å« CCAMÀ» °¡Áø žÆÀÇ °æ¿ì Á¾±«°¡ lung hypoplasia¿Í venous returnÀ» °¨¼Ò½ÃÄÑ fetal hydropsÀ» ÃÊ·¡ÇÏ°í ÀÌ·Î ÀÎÇÏ¿© žÆÀÇ »ç¸ÁÀ» ÃÊ·¡Çϸç ÀÌ·¯ÇÑ º¯È´Â °¡¿ªÀûÀÓÀÌ µ¿¹° ½ÇÇè¿¡¼ ÀÔÁõµÇ¾ú´Ù(±×¸² 5).
Fig 5-1 Animal Model of CCAM(inflation of balloon) |
Fig 5-2 Animal model of CCAM(deflation of balloon) |
µû¶ó¼ fetal hydrops°¡ ÀÖ´Â CCAMÀÇ °æ¿ì¿¡´Â ÅÂ¾Æ ¼ö¼úÀÇ ´ë»óÀÌ µÇ¸ç ÇöÀç open fetal surgery¸¦ ÀÌ¿ëÇÑ surgical resectionÀÌ ½ÃÇàµÇ°í ÀÖ´Ù(±×¸² 6). ÃÖ±Ù¿¡´Â ħ½ÀÀÌ ÀûÀº radiofrequency ablation¿¡ ´ëÇÑ ¹æ¹ýÀÌ ¿¬±¸µÇ°í ÀÖ´Ù(±×¸² 7).
Fig. 6 Surgical Resection of CCAM |
Fig. 7 Radiofrequency ablation of CCAM |
10. Fetus with Congenital Diaphragmatic Hernia(CDH)
CDHÀÇ °æ¿ì °¡Àå È°¹ßÇÏ°Ô ¿¬±¸µÇ°í ½ÇÁ¦·Î ¸¹Àº caseÀÇ ÅÂ¾Æ ¼ö¼úÀÌ ½ÇÇàµÇ°í ÀÖ´Â ÁúȯÀÌ´Ù. CDH°¡ Àִ žƴ Á¤µµÀÇ Â÷ÀÌ´Â ÀÖÀ¸³ª lung hypoplasia¸¦ µ¿¹ÝÇÏ°Ô µÇ¸ç ½ÉÇÑ °æ¿ì¿¡´Â ÀÌ·Î ÀÎÇÏ¿© Ãâ»ýÈÄ »ç¸ÁÇÏ°Ô µÈ´Ù. CDHÀÇ µ¿¹°¸ðµ¨¿¡¼ CDH¸¦ žƽñ⿡ ±³Á¤ÇÏ¿© ÁÖ¸é lung hypoplasiaÀÇ °¡¿ªÀûÀÎ º¯È°¡ ÀÔÁõµÇ¾ú´Ù(±×¸² 8).
Fig. 8 Animal model of CCAM
Àΰ£ Å¾ƿ¡ ´ëÇÑ Àû¿ë½ÃÀÇ criteriaµµ Á¤¸³µÇ¾î ÀÖÀ¸¸ç ¼ö¼ú¹æ¹ýµµ óÀ½¿¡´Â open repair¸¦ ½ÃµµÇÏ¿´À¸³ª ¼ºÀûÀÌ ÁÁÁö ¾Ê¾Æ ÇöÀç´Â temporary tracheal occlusionÀ» žƳ»½Ã°æ(fetendo)À» ÀÌ¿ëÇÏ¿© ½ÃÇàÇÏ¿© ÁÁÀº ¼ºÀûÀ» °ÅµÎ°í ÀÖ´Ù(Ç¥ 3).
History of Fetal Surgery for Congenital Diaphragmatic Hernia * pathophysiology in fetal lamb model ---> Hypoplasia reversible, surgical repair feasible(1981) * Natural history of untreated human fetus ---> Timing & volume, hidden mortality (1985) * Selection for Rx ----> Liver position (1987), Lung-Head Ratio (1996) * Fetal Rx ----> Open repair (two-step) ----> 1st trial (1991) ----> Temporary tracheal occlusion (1994) -PLUG vs CLIP (1996) -Open vs FETENDO (1997) -Randomized Trial (1999) |
Open Repair
open repair(±×¸² 9)´Â Ãʱ⿡ ½ÃÇàµÇ¾ú´ø ¹æ¹ýÀ¸·Î ÇöÀç´Â žƳ»½Ã°æ(fetendo)À» ÀÌ¿ëÇÑ temporary tracheal occlusionÀÌ ÁÖ·Î ½ÃÇàµÇ°í ÀÖ´Ù.
Fig. 9-1 Open Repair of CDH |
Fig. 9-2 Gortex pouch to increase the peritoneal cavity |
±× ÀÌÀ¯´Â open repair¸¦ ½ÃÇàÇÒ °æ¿ì ³ôÀº
žƻç¸Á·üÀ» º¸À̴µ¥ ÀÌ´Â °£ÀÌ Èä°û¿¡ À§Ä¡ÇÑ liver-up caseÀÇ °æ¿ì¿¡ °£ÀÌ º¹°³»·Î
Á¤º¹µÇ¸é¼ umbilical veinÀÌ kinkingµÇ¾î žư¡ »ç¸ÁÇÏ°Ô µÇ±â ¶§¹®ÀÌ´Ù (±×¸²
10).
Fig. 10 The Mechanism of High Mortality in Liver-up cases
Temporary Tracheal Occlusion
CDHÀÇ µ¿¹°¸ðµ¨¿¡¼ trachea¸¦ occlusionÇÏ´Â °æ¿ì¿¡´Â Æó°¡ ¼ºÀåÇÏ°í Èä°ûÀ¸·Î µé¾î¿Â º¹°³» Àå±â°¡ ¹ßÀ°µÇ´Â Æó·Î ÀÎÇÏ¿© º¹°³»·Î º¹¿øÇÔÀÌ µ¿¹°¸ðµ¨¿¡¼ Áõ¸íµÇ¾ú´Ù (±×¸² 11).
Fig. 11 Fetal Lamb Model for Temporarily Tracheal Occlusion in CDH
À̸¦ ÀÌ¿ëÇÏ¿© CDH¸¦ °¡Áø Àΰ£ Å¾ƿ¡¼µµ temporary tracheal occlusionÀ» ½ÃÇàÇÏ¿© ÆóÀÇ ¼ºÀåÀ» ÃËÁøÇÏ°í º¹°³» Àå±â°¡ Á¡ÁøÀûÀ¸·Î º¹°³»·Î º¹¿øµÈ ÈÄ lung hypoplasia°¡ È£ÀüµÈ »óÅ¿¡¼ Ãâ»ýÈÄ CDH¸¦ repairÇÏÀÚ´Â °ÍÀÌ CDH¿¡¼ temporay tracheal occlusionÀÇ °³³äÀÌ´Ù(±×¸² 12).
Fig. 12 The First Case of Human Tracheal Occlusion
temporary tracheal occlusionÀÇ ¹æ¹ýÀ¸·Î´Â
PLUG(Plug
the Lung
Until
it Grows)À»
ÀÌ¿ëÇÏ´Â ¹æ¹ý(±×¸² 13), CLIPÀ» ÀÌ¿ëÇÏ´Â ¹æ¹ý(±×¸² 14), BalloonÀ» ÀÌ¿ëÇÏ´Â ¹æ¹ý(±×¸²
15)ÀÌ ÀÖÀ¸¸ç ÀÌµé ¹æ¹ýÀº Ãʱ⿡´Â open procedure·Î ÇàÇÏ¿© Á³À¸³ª ÇöÀç´Â žƳ»½Ã°æ(FETENDO)À»
ÀÌ¿ëÇÏ¿© ½ÃÇàµÇ¾îÁö°í ÀÖ´Ù.
Fig. 13 Foam PLUG |
Fig. 14 Tracheal Clip |
Fig. 15 Detachable Silicon Balloon |
EXIT Procedure
EXIT(EX Utero Intrapartum Treatment)¶õ temporary tracheal occlusionÀ» ½ÃÇàÇÑ Å¾ư¡ Ãâ»ýÇÏ°Ô µÇ¸é žÆÀÇ »óºÎ¸¸À» ÀڱÿܷΠºÐ¸¸½ÃŲ »óÅ¿¡¼ ŹÝÀ» ÅëÇÑ Á¦´ë Ç÷·ù¿¡ ÀÇÁöÇÏ¿© žư¡ ¹«È£ÈíÀÎ »óÅ¿¡¼ tracheal occlusionÀ» Á¦°ÅÇÏ°í bronchoscope, intubation, surfactant Åõ¿©, elective ventilationµîÀÇ procedure¸¦ ½ÃÇàÇÏ´Â ¹æ¹ýÀÌ´Ù. ÀÌ ¹æ¹ýÀº ¿ø·¡ CDH¿¡¼ temporary tracheal occlusionÇÑ Å¾Ƹ¦ À§ÇÏ¿© °³¹ßµÈ ¹æ¹ýÀ̳ª ÇöÀç´Â °æºÎÀÇ cystic hygroma, teratoma, congenital high airway obstruction syndrome(CHAOS)°°Àº air way¸¦ obstructionÇÏ´Â ´Ù¸¥ ¼±Ãµ¼ºÁúȯÀ» °¡Áø žÆÀÇ Ãâ»ê¿¡¼µµ Àû¿ëµÇ°í ÀÖ´Ù. Àϸí OOPS(Operation on Placenta Support)¶ó°íµµ ºÒ¸°´Ù (±×¸² 16).
Fig. 16 EXIT procedure
Prognostic Factors of Prenatal Diagnosed CDH and Selection Criteria for Fetal Surgery
»êÀü Áø´ÜµÈ CDHÀÇ ¿¹Èĸ¦ °áÁ¤ÇÏ´Â ÀÎÀÚ´Â LHR(Lung Head Ratio), Liver position(Liver-up¿©ºÎ), Polyhydroamniosis ¿©ºÎ, CDH°¡ óÀ½ Áø´ÜµÈ ½Ã±â, stomach position, cardiac anomaly ¿©ºÎ µîÀÌ ÀÖ´Ù (±×¸² 17, 18).
Fig. 17 Prognostic Factors in CDH |
Fig. 18 Survival Rate of CDH and Lung-Head Ratio(LHR) |
žƼö¼úÀÇ ´ë»óÀÌ µÇ´Â °æ¿ì´Â ¿¹ÈÄ°¡ ³ª»Ü
°ÍÀ¸·Î ÃßÃøµÇ´Â group¸¸À» ±× ´ë»óÀ¸·Î ÇÏ°í ÀÖ´Ù. Áï ÀӽŠ24ÁÖÀü¿¡ ÃÊÀ½ÆÄ·Î Áø´ÜµÇ°í,
liver°¡ herniation µÇ¾î ÀÖÀ¸¸ç LHRÀÌ 1.4 ¹Ì¸¸ÀΠžư¡ ±× ´ë»óÀÌ µÇ°í ÀÖ´Ù (±×¸²
19)
Fig. 19 Groups of Treatment in Poor Prognosis of CDH
Result of Temporary Tracheal Occlusion for CDH
¿¹ÈÄ°¡ ³ª»Û groupÀ» postnatal repair group, open clip group, fetendo clip groupÀ¸·Î ³ª´©¾î ºñ±³ÇÑ °á°ú¸¦ º¸¸é fetendo clip groupÀÇ »ýÁ¸À²ÀÌ ¿ùµîÈ÷ Çâ»óµÇ¾úÀ½À» ¾Ë ¼ö ÀÖÀ¸¸ç(±×¸² 20) »ê¸ðÀÇ Àç¿øÀϵµ °¨¼ÒµÇ¾ú´Ù.
Fig. 20 Survival Rate of CDH According to Treatment Methods |
FETENDO (FETal ENDOscopy)
FETENDOÀÇ ÀåÁ¡Àº ±×¸² 21°ú °°´Ù.
Fig. 21 Advantages of Fetendo |
FETENDO¸¦ ½ÃÇà½Ã´Â ¼ö¼úÁß ÃÊÀ½ÆķΠŹÝÀÇ À§Ä¡¸¦ È®ÀÎÈÄ ³»½Ã°æÀÇ »ðÀÔºÎÀ§¸¦ °áÁ¤ÇÏ°Ô µÇ¸ç Ư¼öÇÏ°Ô Á¦ÀÛµÈ trochar¸¦ ÀÌ¿ëÇÏ¿© ¾ç¼öÀÇ ´©ÃâÀ» ¹æÁöÇÏ°Ô µÈ´Ù. working mediumÀ¸·Î´Â °ú°Å¿¡´Â gas¿Í fluid¸¦ »ç¿ëÇÏ¿´À¸³ª ÇöÀç´Â fluid¸¦ »ç¿ëÇÏ°í ÀÖÀ¸¸ç fluid¸¦ »ç¿ëÇÒ °æ¿ì ¹®Á¦°¡ µÇ´Â ½Ã¾ßÀÇ È®º¸´Â perfusion pump¸¦ »ç¿ëÇÏ¿© Áö¼ÓÀûÀ¸·Î fluid¸¦ °ü·ù½ÃÅ°´Â ¹æ¹ýÀ¸·Î ÇØ°áÇÏ°í ÀÖ´Ù(±×¸² 22).
Fig. 22 Perfusion Pump for Amniotic Fluid Circulation |
FETENDOÀÇ ´ë»óÁúȯÀº ±×¸² 23°ú °°´Ù.
Fig. 23 Diseases for Operative Fetoscopy |
11. Fetus with Sacrococcygeal Teratoma
¾ÆÁÖ Å« õ¹Ì°ñ ±âÇüÁ¾ÀÌ ÀÖ´Â °æ¿ì¿¡´Â high
output herat failure·Î ÀÎÇÑ fetal hydrops¸¦ ÃÊ·¡ÇÏ°Ô µÇ¾î žư¡ »ç¸ÁÇÏ°Ô µÈ´Ù.
À̶§´Â open surgical resectionÀ» ½ÃÇàÇÏ°í ÀÖÀ¸¸ç(±×¸² 24) ÇöÀç´Â ºñħ½ÀÀûÀÎ
radiofrequency ablation ¹æ¹ýÀ» ¸ð»öÇÏ°í ÀÖ´Ù(±×¸² 25).
Fig. 24 Open Resection of Sacrococcygeal Teratoma |
Fig. 25 Laser Ablation of Sacrococcygeal Teratoma |
12. Fetus with Myelomeningocele
myelomeningoceleÀº ºñ·Ï žƸ¦ »ç¸Á¿¡ ÀÌ·ç°Ô
ÇÏÁö´Â ¾ÊÀ¸³ª ¿µ±¸ÀûÀÎ ½Å°æ°è ¼Õ»óÀ¸·Î ÇÏÁöÀÇ ¸¶ºñ, º¯.¿ä½Ç±ÝµîÀ» ÃÊ·¡ÇÑ´Ù.
myelomeningoceleÀÇ µ¿¹°¸ðµ¨ ½ÇÇèÀ» º¸¸é ÀÌ·± ½Å°æ°è ¼Õ»óÀº ½Å°æÁ¶Á÷ÀÌ Àڱó»¿¡¼
³ëÃâµÇ¹Ç·Î ÀÎÇÏ¿© Àڱú®µîÀ¸·Î ºÎÅÍ ¹°¸®Àû ¼Õ»óÀ» ÀÔÀ½À¸·Î½á »ý±ä´Ù°í »ý°¢µÇ¾î
Áö°í ÀÖÀ¸¸ç žƽñ⿡ myelomeningoceleÀ» repairÇÏ´Â °æ¿ì¿¡´Â ½Å°æ¸¶ºñÀÇ Áõ¼¼°¡
¾ø¾îÁüÀÌ È®ÀεǾú´Ù. ÇöÀç Àΰ£ÀÇ Å¾ƿ¡°Ôµµ primary repairÀÇ ¹æ¹ýÀ» »ç¿ëÇÏ¿©
¼º°ø¿¹¸¦ º¸°íÇϱ⠽ÃÀÛÇÏ¿´À¸¸ç ÀϺο¡¼´Â fetendo¸¦ ÀÌ¿ëÇÑ repair¸¦ ½ÃµµÇÏ°í
ÀÖ´Ù(±×¸² 26).
Fig. 26 Fetendo Surgery for Myelomeningocele. |
13. Fetus with Twin Twin Transfusion Syndrome(TTTS)
TTTS´Â °¢°¢ÀÇ amniotic sacÀ» °¡Áø À϶õ¼º
½Ö»ý¾Æ°¡ ÇÑ °³ÀÇ Å¹ÝÀ» °øÀ¯Çϸé¼(±×¸² 27) ÅÂ¹Ý Ç÷°ü»çÀÌ¿¡ ±³ÅëÀÌ ÀÖ¾î(º¸Åë
A-V communication) donor twinÀÇ Ç÷¾×ÀÌ recepient twinÀ¸·Î À̵¿ÇÏ¿© 75%ÀÇ »ç¸Á·üÀ»
º¸ÀÌ´Â ÁúȯÀÌ´Ù(±×¸² 28). À̶§´Â fetendo¸¦ ÀÌ¿ëÇÏ¿© vascular communicationÀ»
laser ablationÇÏ¿© ÁÁÀº °á°ú¸¦ ¾ò°í ÀÖ´Ù.
Fig. 27 Monozygotic twins with tow amnions and one placenta |
Fig. 28 Vascular Communication between Twins |
14.Fetal Treatment Team and Refer System
Fetal Surgery¸¦ À§ÇÑ teamÀº ¼±Ãµ¼º Áúȯ¿¡ ´ëÇÑ Áö½Ä°ú °æÇèÀÌ ¸¹Àº ¼Ò¾Æ¿Ü°ú Àǻ簡 ÁÖÃàÀÌ µÇ±â´Â Çϳª ¹«¾ùº¸´Ùµµ °ü·ÃµÇ´Â °¢ Àü¹®ºÐ¾ß°£ÀÇ »óÈ£ ÇùÁ¶°¡ Áß¿ä½ÃµÈ´Ù. °ü°èµÇ´Â Àü¹®ºÐ¾ß¸¦ »ìÆ캸¸é ¼Ò¾Æ¿Ü°ú, »êºÎÀΰú, ¼Ò¾Æ¹æ»ç¼±ÇÐ, ¸¶Ãë°ú, ½Å»ý¾ÆÇÐ, ¼Ò¾Æºñ´¢±â°ú, ¼Ò¾Æ½Å°æ¿Ü°ú, ¼ö¼ú°£È£ÇÐ µîÀÌ ÀÖÀ¸¸ç À̵鰣ÀÇ ±ä¹ÐÇÑ ÇùÁ¶ÇÏ¿¡ fetal tretment teamÀ» ±¸¼ºÇÏ¿©¾ß ÇÑ´Ù.
žƼö¼úÀÇ ÀûÀÀÁúȯÀÌ »êÀü ÃÊÀ½ÆÄ¿¡¼ ¹ß°ßµÇ¸é ȯÀÚÀÇ º´·Â°ú ÃÊÀ½ÆÄ ±â·Ï(video tape)À» ¿ì¼± ¿ìÆíÀ¸·Î Á¢¼ö¹Þ°Ô µÇ¸ç À̸¦ °ËÅäÇϴ ȸÀǸ¦ °ÅÃÄ ÀûÀÀ ´ë»óÀÌ µÈ´Ù°í »ý°¢µÇ¸é ȯÀÚ¸¦ ¹æ¹®ÄÉ ÇÏ¿© ÇÊ¿äÇÑ °Ë»ç¸¦ Ãß°¡·Î ¹Þ°Ô ÇÑ´Ù. ¸ðµç °Ë»ç°¡ ³¡³ª¸é °Ë»ç °á°ú¸¦ Á¾ÇÕÇÏ¿© ȯÀÚ¿ÍÀÇ ¸é´ãÀ» °ÅÄ£ ÈÄ ¼ö¼úÀ» °áÁ¤ÇÏ°Ô µÈ´Ù.
15. ¸Î´Â ¸»
»êÀü Áø´Ü ±â¼úÀÇ ¹ß´Þ·Î ÀÎÇÏ¿© ¿ì¸®´Â °ú°Å¿¡´Â ¾ËÁö ¸øÇÏ¿´´ø ¼±Ãµ¼º ÁúȯÀÇ Áø´ÜÀÌ °¡´ÉÇÏ°Ô µÇ¾úÀ¸¸ç À̵é ÁúȯÀÇ Å¾ƽñâÀÇ º´Å»ý¸®µµ ¾Ë°Ô µÇ¾ú´Ù. ÀÌ·Î ÀÎÇÏ¿© °ú°Å¿¡´Â Ä¡·á°¡ ºÒ°¡´ÉÇÏ´Ù°í »ý°¢µÇ¾îÁ³´ø ¸¹Àº ÁúȯµéÀ» žƽñ⿡ ±³Á¤ÇÏ·Á´Â ½Ãµµ°¡ ÀÖ¾î¿ÔÀ¸¸ç ÀÌÁß Çϳª°¡ žƼö¼úÀÌ´Ù. °ú°Å ¼ö½Ê ³â°£ÀÇ ¿¬±¸¿Í ÀÓ»ó½Ãµµ, ±×¸®°í ±Ù·¡ÀÇ ³»½Ã°æ ¼ö¼úÀÇ ¹ß´Þ¿¡ ÈûÀÔ¾î ¼±Ãµ¼º Ⱦ°æ¸· Å»Àå, twin twin transfusion syndrome°°Àº ÁúȯÀÇ ÅÂ¾Æ ¼ö¼úÀÇ ¼ºÀûÀº °ý¸ñÇÏ°Ô Çâ»óµÇ¾úÀ¸¸ç À̵é Áúȯ¿¡ ´ëÇÑ ±¹³» Àû¿ëµµ °¡´ÉÇÏ´Ù°í »ý°¢µÈ´Ù.
All copyright is reserving for Seok oo Han, M.D., Department of the Pediatric Surgery, Yonsei University College of the Medicine, Seoul, Korea
º» »çÀÌÆ®ÀÇ ³»¿ëÀº ¼Ò¾Æ¿Ü°úÀÇ»ç ÇÑ ¼®ÁÖÀÇ È¨ÆäÀÌÁöÀÇ ³»¿ëÀÔ´Ï´Ù.
µû¶ó¼ º»ÀÎÀÇ Çã¶ô ¾øÀÌ ºÒ¹ýÀ¸·Î º¹»ç »ç¿ëÇÏ´Â °ÍÀº ¹ýÀû Ã¥ÀÓÀ» Áú ¼ö ÀÖ½À´Ï´Ù.