MECONIUM PLUG SYNDROME

Willy Hardy Marpaung

Pembimbing dr. Akhmad Makhmudi, Sp.B, Sp.BA

Pendahuluan
Obstruksi intestinal neonatus : Hirschsprung Malrotasi Volvulus Atresia intestinal Kelainan 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

Pendahuluan

Meconium Plug Syndrome (MPS) obstruksi

kolon distal pada neonatus akibat mekonium abnormal Diteliti oleh Clatworthy (1956) Diperkirakan berkaitan dengan mekonium yang kental Tingkat mortalitas dan morbiditas yang rendah Kejadian 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. 2008 3. Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.

Mekonium

Terdiri dari : air (80%), mukopolisakarida, bilirubin, enzim usus, rambut, sel skuamous. Hijau pigmen bile Peristaltik usus pada janin 8 minggu Absorbsi pada usus 10 12 minggu Mekonium hasil akhir pencernaan janin

Poggi, Sarah. Pathophysiology of meconium passage into the amniotic fluid in Early Human Development. 2009.

Penyebab MPS
Prinsip terjadinya MPS : gangguan motilitas usus dan konsistensi mekonium Kondisi 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. 2008

Cystic Fibrosis

Kelainan genetik autosomal resesif Gangguan elektrolit pada seluruh epitel membran sel Dideteksi dengan sweat test Gangguan sekresi enzim pankreas, elektrolit Mekonium kental 43% penderita MPS

Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 2008

Penyakit 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. 2008 Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.

Penelitian MPS dan Hirschsprung

Peneliti Ellis et al Van Leeuwen et al Burge et al Current series Jumlah Pasien 30 8 21 77 Hirschsprung (+)

% 13,3 25,0 38,1 13,0

Keckler, Scott, et al. Current significance of meconium plug syndrome in Journal of Pediatric Surgery. 2008

Hipermagnesia
Pemberian MgSO4 pada ibu dengan pre eklampsia/eklampsia 4% dari seluruh kejadian MPS Berhubungan 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.

Ibu dengan diabetes mellitus

Obstruksi kolon fungsional Fetus hipoglikemia produksi glikogen yang tinggi penurunan motilitas usus Penurunan motilitas usus reabsrobsi mekonium meningkat MPS

Yun-Yun, et al. Meconium Obstruction in Verly Low Birth Weight Premature Infants in Clinical Neonatology. 2007.

Hipotiroidisme Kongenital
Hipotiroidisme gangguan autoimun Akumulasi mukopolisakarida, asam hialuronat di mukosa saluran cerna edema seluler Edema seluler hipomotilitas usus Reabsorbsi mekonium mekonium kental

Yaylali, Olga, et al. Does hypothyroidism Affect Gastrointestinal Motility in Gastroenterology Research and Practice. 2009.

Displasia usus neuronal

Kondisi patologis persarafan saluran cerna Hiperganglionosis pleksus submukosa dan myentrikus usus akan lebih relaksasi dan hipomotilitas Transit waktu mekonium meningkat Mekonium akan kental Terjadi pada 6 dari 20 MPS

Hutson, JM. Slow transit constipation in children in Journal of Paediatric Child Health. 2001.

Diagnosis MPS
Gejala : mekonium terlambat, kembung, muntah hijau Radiologi : 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

Radiologi

Baucke-Loening, Vera. Failure to pass meconium: diagnosing neonatal intestinal obstruction in Radiologic Decision-Making. 1999

Radiologi

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

Diagnosis MPS vs Mekonium Ileus

MPS: obstruksi pada kolon distal, MI: obstruksi pada ileum terminal MPS : obstruksi ringan, MI : obstruksi total dengan komplikasi MPS: membaik dengan rectal toucher, enema

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 Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.

Penanganan MPS
Rectal Toucher Irigasi Nacl 0,9% Enema barium

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 Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.

Prognosis
Sembuh spontan 29 35% Keberhasilan barium enema 98% Bayi akan segera pulih segera setelah plug dikeluarkan Prognosis baik

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 Cuenca, Alex. Pulling the plug-Management of meconium plug syndrome in neonates in Journal of Surgical Research. 2012.

Kesimpulan
MPS obstruksi neonatus akibat mekonium yang kental pada kolon distal dan merupakan kelainan yang ringan Keberhasilan dengan barium enema sangat tinggi Jarang menyebabkan komplikasi

TERIMA KASIH