MECONIUM PLUG SYNDROME

MECONIUM PLUG SYNDROMEWilly Hardy Marpaung

Pembimbingdr. Akhmad Makhmudi, Sp.B, Sp.BA1PendahuluanObstruksi intestinal neonatus :Hirschsprung MalrotasiVolvulusAtresia intestinalKelainan mekonium Meconium Plug Syndrome (MPS)

Caty, Michael. Meconium Disease in Aschrafts Pediatric Surgery 5th edition. 2009.Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 2008

2PendahuluanMeconium Plug Syndrome (MPS) obstruksi kolon distal pada neonatus akibat mekonium abnormalDiteliti oleh Clatworthy (1956)Diperkirakan berkaitan dengan mekonium yang kentalTingkat mortalitas dan morbiditas yang rendahKejadian 1:500 1: 1000 kelahiran hidup

1. Caty, Michael. Meconium Disease in Aschrafts Pediatric Surgery 5th edition. 2009.2. Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 20083. Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.

3MekoniumTerdiri dari : air (80%), mukopolisakarida, bilirubin, enzim usus, rambut, sel skuamous.Hijau pigmen bilePeristaltik usus pada janin 8 mingguAbsorbsi pada usus 10 12 mingguMekonium hasil akhir pencernaan janinPoggi, Sarah. Pathophysiology of meconium passage into the amniotic fluid in Early Human Development. 2009.

4Penyebab MPSPrinsip terjadinya MPS : gangguan motilitas usus dan konsistensi mekoniumKondisi patologi yang berhubungan : Cystic fibrosis, Hirschsprung disease, hipermagnesia, ibu dengan diabetes mellitus, hipotiroidisme, displasia usus neuronal, lain-lain (small left colon syndrome, addiksi narkotik maternal )Caty, Michael. Meconium Disease in Aschrafts Pediatric Surgery 5th edition. 2009.Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 20085Cystic Fibrosis Kelainan genetik autosomal resesifGangguan elektrolit pada seluruh epitel membran selDideteksi dengan sweat testGangguan sekresi enzim pankreas, elektrolitMekonium kental43% penderita MPS

Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 20086Penyakit Hirschsprung Terlambat evakuasi mekonium Hirschsprung?Hubungan yang tinggi dengan MPS (13%)Perlu diagnosis biopsi / enema barium untuk menyingkirkan penyakit Hirschsprung

Caty, Michael. Meconium Disease in Aschrafts Pediatric Surgery 5th edition. 2009.Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 2008Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.

7Penelitian MPS dan HirschsprungPenelitiJumlah PasienHirschsprung (+) %Ellis et alVan Leeuwen et alBurge et alCurrent series308217713,325,038,113,0Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 2008

8HipermagnesiaPemberian MgSO4 pada ibu dengan pre eklampsia/eklampsia4% dari seluruh kejadian MPSBerhubungan dengan relaksasi dari otot polos kolon waktu transit lebih lama reabsorbsi >> mekonium kental

Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.Rajadurai, Victor Samuel. Magnesium Sulphate Infusion for the treatment of persistent pulmonary hypertension of the newborn in Americal Pediatric Society. 1999.9Ibu dengan diabetes mellitusObstruksi kolon fungsionalFetus hipoglikemia produksi glikogen yang tinggi penurunan motilitas ususPenurunan motilitas usus reabsrobsi mekonium meningkat MPSYun-Yun, et al. Meconium Obstruction in Verly Low Birth Weight Premature Infants in Clinical Neonatology. 2007.10Hipotiroidisme KongenitalHipotiroidisme gangguan autoimun Akumulasi mukopolisakarida, asam hialuronat di mukosa saluran cerna edema selulerEdema seluler hipomotilitas ususReabsorbsi mekonium mekonium kental

Yaylali, Olga, et al. Does hypothyroidism Affect Gastrointestinal Motility in Gastroenterology Research and Practice. 2009.11Displasia usus neuronalKondisi patologis persarafan saluran cernaHiperganglionosis pleksus submukosa dan myentrikus usus akan lebih relaksasi dan hipomotilitasTransit waktu mekonium meningkatMekonium akan kentalTerjadi pada 6 dari 20 MPSHutson, JM. Slow transit constipation in children in Journal of Paediatric Child Health. 2001.

12Diagnosis MPSGejala : mekonium terlambat, kembung, muntah hijauRadiologi : dilatasi sistema usus halus dan besar, soap bubble appearance (foto polos), plug mekonium pada colon distal (enema)Pemeriksaan lain : sweat test (cystic fibrosis), biopsi rektal (Hirschsprung)

Caty, Michael. Meconium Disease in Aschrafts Pediatric Surgery 5th edition. 2009.Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 2008

13Radiologi

Baucke-Loening, Vera. Failure to pass meconium: diagnosing neonatal intestinal obstruction in Radiologic Decision-Making. 199914Radiologi

Burge, David. Meconium plug obstruction in Pediatric Surgery International. 2004.

15Diagnosis MPS vs Mekonium IleusMPS: obstruksi pada kolon distal, MI: obstruksi pada ileum terminalMPS : obstruksi ringan, MI : obstruksi total dengan komplikasiMPS: membaik dengan rectal toucher, enemaCaty, Michael. Meconium Disease in Aschrafts Pediatric Surgery 5th edition. 2009.Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 2008Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.

16Penanganan MPSRectal ToucherIrigasi Nacl 0,9%Enema bariumCaty, Michael. Meconium Disease in Aschrafts Pediatric Surgery 5th edition. 2009.Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 2008Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.

17PrognosisSembuh spontan 29 35%Keberhasilan barium enema 98%Bayi akan segera pulih segera setelah plug dikeluarkanPrognosis baikCaty, Michael. Meconium Disease in Aschrafts Pediatric Surgery 5th edition. 2009.Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 2008Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.

18KesimpulanMPS obstruksi neonatus akibat mekonium yang kental pada kolon distal dan merupakan kelainan yang ringanKeberhasilan dengan barium enema sangat tinggiJarang menyebabkan komplikasi19TERIMA KASIH20