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Fig. 1. The Four Embryolofic Components of The Devoloping Diaphragm (Coran, 7th
Ed)
The precursors of diaphragmatic structure begin to form during the 4th week of gestation
with the appearance of the peritoneal fold from the lateral mesenchymal tissue. At the same time,
the septum transversum forms from the inferior portion of the pericardial cavity. The septum
transversum serves to separate the thoracic from the abdominal cavities and eventually forms the
central tendinous area of the fully developed diaphragm. It defines the rudimentary
pleuroperitoneal canals and allows for the establishment of mesenchymal tissue within these
canals that ultimately results in pulmonary parenchymal development. (Coran, 7th Ed)
Closure of the pleuroperitoneal canals with the formation of a pleuroperitoneal membrane
occurs during the 8th week of gestation. Several theories have been proposed to explain the
formation of this membrane and the subsequent development of a diaphragmatic structure.
Progressive growth of the pleuroperitoneal membrane has been one mechanism proposed for
canal closure. Other researchers have postulated that concurrent hepatic and adrenal
organogenesis is crucial to this process. The involvement of a posthepatic mesenchymal plate in
diaphragmatic formation has been proposed. (Coran, 7th Ed)
The pleuroperitoneal folds extend from the lateral body wall and grow medially and
ventrally until they fuse with the septum transversum and dorsal mesentery of the esophagus
during 6th week of gestational. Complete closure of the canal takes place during 8th week of
gestation. Anatomically, the right side closes before the left. Muscularization of the diaphragm
appears to develop from the innermost muscle layer of the thoracic cavity, although it has been
proposed that the posthepatic mesenchymal plate is a possible source of muscular tissue.
Posterolaterally, at the junction of the lumbar and costal muscle groups, the fibrous lumbocostal
trigone remains as a small remnant of the pleuroperitoneal membrane and relies on the fusion of
the two muscle groups in the final stages of development for its strength. Delay or failure of
muscular fusion leaves this area weak, perhaps predisposing to herniation. (Coran, 7th Ed)
Associated Anomalies
Any newborn with a major congenital anomaly, including infants with CDH, has an
increased incidence of an additional malformation compared with the general population.
Although previously thought to be low, the incidence of associated malformations in infants with
a CDH ranges from 10% to 50%. (Coran, 7th Ed)
A number of syndromes have a CDH as a pathologic finding. (Coran, 7th Ed) CDH has
been associated with over 70 syndromes. (Aschrafts, Ed 6) These include trisomy 21, 18, and
13, and syndromes such as Frey, Beckwith-Weidenmann, Goldenhar, Coffin-Siris, Fryns,
Meacham, and Kabuki. (Coran, 7th Ed) In some cases, the diaphragmatic malformation is the
predominant defect, as in Fryns and DonnaiBarrow syndromes. In other syndromes, CDH only
occurs in a small percentage, but still greater than the general population, as in SimpsonGolabi
Behmel and BeckwithWiedermann syndromes. The inheritance patterns for these syndromes
include dominant and recessive as well as autosomal and X-linked variants. Identifying the
patterns of non-hernia-related anomalies associated with CDH and recognizing genetic
syndromes help determine the prognosis, treatments, counseling, and outcomes. If the antenatal
diagnosis of CDH is made, amniocentesis with karyotype and chromosomal analysis is indicated.
(Aschrafts, Ed 6) Due to this dismal outcome, the emphasis on detailed and accurate prenatal
diagnosis has influenced the management and treatment of CDH. (Aschrafts, Ed 6)
Approximately 50% of CDH are isolated defects with the others associated with
anomalies of the cardiovascular 27.5% according Aschrafts 6th edition (Aschrafts, Ed 6) and
24% according to Coran 7th edition (Coran, 7th Ed); urogenital 17.7%; (Aschrafts, Ed 6)
musculoskeletal 15.7% according to Aschrafts 6th edition (Aschrafts, Ed 6), and 32% according
to Coran 7th edition (Coran, 7th Ed); and central nervous systems (CNS) 9.8%. Many conditions,
such as lung hypoplasia, intestinal malrotation, some cardiac malformations, and patent ductus
arteriosus (PDA) are considered to be consequences of the diaphragmatic defect. Non-CDH-
related defects are estimated to occur in 40-60% of cases and can involve the cardiovascular,
CNS, gastrointestinal, and genitourinary systems, and may be a consequence of an underlying
field defect of unknown etiology. (Aschrafts, Ed 6)
Neural tube defects were abnormalities noted in stillborn group and included
anencephaly, myelomeningocele, hydrocephalus, and encephaloceles. Even in infants who
survive to birth but die shortly thereafter, neural tube defects were common malformations
noted. (Coran, 7th Ed)
Cardiac hypoplasia involving the left ventricle and often associated with hypoplasia of
the aortic arch is frequently described and can be confused with hypoplastic heart syndromes.
However, the clinical significance is limited. (Coran, 7th Ed) Common cardiac defects include
atrial septal defect (ASD), ventricular septal defect (VSD), and other outflow tract anomalies
(transposition of the great vessels, tetralogy of Fallot, double-outlet right ventricle, aortic
coarctation (Aschrafts, Ed 6), and vascular ring (Coran, 7th Ed)). In a review of 4,268 infants
with CDH, approximately 18% of infants had an associated cardiac defect. Major cardiac lesions
(excluding patent foramen ovale, atrial septal defects, PDA) was 8% with an overall survival of
36% compared to infants with minor anomalies (67% survival) and those without cardiac defects
(73% survival). (Aschrafts, Ed 6)
Anatomic anomalies of the tracheobronchial tree have been found in 18% of patients with
CDH and include congenital tracheal stenosis, tracheal bronchus, and trifurcated trachea. (Coran,
7th Ed) Other midline developmental anomalies have also been reported and include esophageal
atresia, omphalocele, and cleft palate. (Coran, 7th Ed)
Twenty percent of prenatally diagnosed CDH infants have chromosomal anomalies, with
70% having an associated structural malformation. In contrast, only 35% of postnatally
diagnosed CDH infants have an associated anomaly. This difference may be the result of lethal
chromosomal anomalies and/or may reflect parental decisions for termination in high-risk infants
with anomalies that portend significant morbidity. (Aschrafts, Ed 6)
The impact of associated anomalies on prognosis and outcome cannot be overstated.
Ninety-five per cent of stillborn infants with CDH have an associated major anomaly. Greater
than 60% of infants who do not survive the immediate neonatal period have associated
anomalies. In contrast, infants that survive preoperative stabilization and come to operative
repair have less than 10% additional anomalies. Although the severity of pulmonary hypoplasia
and hypertension are the major determinants of overall survival, there is a significant survival
advantage for infants with isolated CDH (43.7% vs 7.1%). (Aschrafts, Ed 6)
Late Presentation
Although most CDH infants present in the first 24 hours of life, 1020% of the affected
infants present later. The symptoms and signs of those patients are nonspecific and include
recurrent chest infections, vomiting, abdominal pain, diarrhea, anorexia, failure to thrive, or an
abdominal chest X-ray in an asymptomatic patient. Some children present acutely with volvulus
or strangulation or acute respiratory distress. Chest X-ray with an in situ nasogastric tube is
reliable for the diagnosis. Even if the hernia is asymptomatic, it should be repaired to prevent
complications. (Pediatric Surgery, Springer)
Here, we present 2 cases of CHD antero-medial (Morgagni) who was admitted to Pediatric
Surgery Department - Dr. Kariadi Hospital, Semarang, Middle Java, Indonesia, and treated in
June 2015.
Case Presentation
Case 1
Bayi laki-laki usia 16 bulan, dengan BB 7.9 kg, dan PB 81 cm, datang ke bagian bedah
anak RS Dr. Kariadi Semarang-Indonesia, dengan keluhan utama adanya celah disekat perut-
dada, dengan membawa hasil pemeriksa penunjang berupa hasil laboratorium darah, ECHO,
EEG, x-foto thorax, x-foto Barium Follow Through, MSCT kranio-serebral kontras.
Riwayat Penyakit Sekarang. Sejak usia 1 bln pasien sering batuk-pilek. Setiap batuk-
pilek bayi dibawa berobat oleh orangtuanya ke dokter spesialis anak, lalu diberikan obat untuk
penyakitnya. Bila batu-pilek tidak tampak adanya keluhan gangguan (sulit) saat bernafas, kulit
atau kuku tidak biru. 2 bulan SMRS ke bagian bedah anak RS Dr. Kariadi Semarang-Jawa
Tengah-Indonesia bayi dibawa kembali berobat ke dokter spesialis anak di Pati-Jawa Tengah-
Indonesia. Karena sering mengalami batuk-pilek berulang, pasien kemudian periksa foto rontgen
dada untuk melihat apakah ada infeksi diparu-paru. Dan hasilnya didapatkan gambaran bayangan
putih didaerah rongga dada. Oleh dokter spesialis anak tersebut pasien dikonsulkan ke dokter
spesialis bedah anak RS Dr. Kariadi Semarang-Jawa Tengah-Indonesia. Saat berobat ke spesialis
bedah anak RS Dr. Kariadi Semarang-Jawa Tengah-Indonesia tersebut, bayi diperiksakan
pemeriksaan foto rontgen dengan perut dengan meminum zat kontras, kemudian didapatkan
gambaran usus didalam rongga dada pasien. Lalu bayi disarankan untuk menjalani operasi
perbaikan celah sekat perut-dada.
Riwayat penyakit dahulu. Saat usia 4 bulan dilakukan pemeriksaan echo, dan didapatkan
adanya celah sekat ventrikel jantunga, saat itu belum di ambil tindakan apapun. Dan di echo
ulang saat usia 14 bln, hasilnya didapatkan bahwa celah sekat jantungnya tersebut telah tertutup.
Riw. Kejang 1 kali, saat bayi berusia 8 bulan, kejang terjadi <1 menit, seluruh tubuh bergerak,
setelah kejang bayi menangis kuat. Kejang didahului dengan panas tubuh pasien meningkat.
Oleh orangtuanya bayi dibawa berobat ke RS Pati, dan dirawat inap. Selama rawat inap pasien di
CT-Scan/MRI kepala polos, dan tidak didapatkan kelainan apapun di dalam kepala.
Riw. Kehamilan dan persalinan. Bayi lahir dari ibu G1P0A0 berusia 25 th, selama
kehamilan ibu rutin memeriksakan diri dan kandungannya (antenatal) tiap bulan ke dokter
spesialis kandungan di Pati, dan setiap bulan dilakukan pemeriksaan USG. Selama kehamilan,
ibu tidak pernah sakit apapun, dan tidak pernah mengkonsumsi obat-obatan atau jamu-jamuan
selain vitamin yang diberikan oleh dokter kandungan. Dengan status gizi perkembangan saat
kehamilan yang baik. BB ibu sebelum hamil 41 kg dengan tinggi badan 152 cm, status gizi
kurang.
Bayi lahir saat usia kandungan 36 minggu 3 hari, persalinan terjadi secara spontan, ditolong oleh
bidan. Saat lahir bayi langsung menangis kuat dan aktif, tidak berwarna biru. BBL 2400 gram,
dan PBL 45 cm. Tidak didapatkan kelainan fisik yang dapat dilihat dengan mata saat itu.
Riwayat tumbuh kembang anak dan vaksinasi. Tumbuh kembang anak dirasakan kurang
apabila dibandingkan dengan bayi seusianya. Bayi diet ASI eksklusif Sejak lahir hingga usia
bayi 2 bln, lalu sejak usis 2bln hingga usia 8 bln ditambahkan susu formula, lalu saat usia 8 bln
hingga saat ini diet bayi sudah ditambahkan makanan padat. Bayi mendapatkan vaksinasi sesuai
dengan waktu pemberiannya. Tumbuh kembang bayi rutin diperiksakan ke dokter spesialis anak
sejak usia bayi 1 bln.
Riwayat penyakit keluarga. Tidak ada anggota keluarga yang mengalami sakit yang
serupa dengan bayi sebelumnya. Dan, tidak ada anggota keluarga yang mengalami sakit bawaan
lainnya.
Riwayat sosial ekonomi. Bayi lahir dari keluarga yang mampu, ayah bayi memiliki
pendidikan terakhir S1, yang bekerja sebagai pekerja swasta, dan ibu bayi memiliki pendidikan
terakhir S1, yang bekerja sebagai IRT.
Pada pemeriksaan fisik. Keadaan umum sadar, aktif dan menangis kuat. Tanda-tanda
vital: nadi 120 detak/menit (isi dan tegangan cukup), pernafasan 40 kali/menit (abdominotorakal)
suhu 36,8C (rektal), saturasi O2 100%, berat badan 7900 gr, panjang badan 75 cm. ??
Dada
Fig. 3. ????
Fig. 3. ????
Manajemen Sebelum Operasi
Pada kedua kasus kami informed consent. Saat bayi diruang perwatan dihangatkan
dengan menempatkan di inkubator. Dilakukan persiapan untuk repair hernia Morgagni dengan
laparoskopi. Sebelum operasi diberikan dipuasakan 4 jam sebelum operasi, O2 4 LPM dengan
kanul, pemberian antibiotik profilaksis sefalosporin generasi I 30 menit sebelum operasi,
dipersiapkan jalur intravena, pemasangan urin kateter.
Operasi
Operasi dilakukan dalam 4 jam. Pasien dibius dalam anestesi umum. Diposisikan supine
??. Setelah tindakan asepsis dan antisepsis lapangan operasi dilakukan insisi kulit di regio
infraumbilikal untuk menempatkan kamera, setelah terpasang kembungkan abdomen dengan
CO2 hingga mencapai tekanan yang sesuai. Pada eksplorasi tampak 1 loop colon transversum
masuk ke dalam celah hernia diafragma Margogni ukuran ?? cm yang bisa dibebaskan setelah
abdomen terisi CO2 dengan tekanan yang sesuai, tidak tampak kelainan lain intraabdomen.
Dilakukan juga insisi kulit di regio kiri atas abdomen, kanan atas abdomen, dan epigstrium.
Hingga menembus peritoneum. Masukkan masing-masing dengan alat tambahan. Lakukan repair
hernia diafragma Margogni dengan menjahit secara langsung tepi hernia sehingga tepi hernia
bertemu, menggunakan benang monofilamen non-absorbable. Setelah selesai eksplorasi, tidak
ada kelainan lain. Keluarkan alat laparoskopi dan CO2, jahit luka operasi. Operasi selesai
Hari 1-2
Pasien di knock down. Tanda-tanda vital dalam batas normal. Diberikan antibiotik,
analgetik, jaga kehangatan dengan ditempatkan dalam inkubator, jaga balance cairan, O2 kanul 4
LPM, TPN. Pengawasan tanda-tanda vital. Dipertahankan NGT dan kateter urin.
Hari 3-5
Keadaan umum sadar, aktif. Tanda-tanda vital dalam batas normal. Dilanjutkan
pemberian antibiotik???. analgetik, jaga kehangatan, jaga balance cairan, O2 kanul 4 LPM, TPN.
Pengawasan tanda-tanda vital. Tetap dipertahankan NGT dan kateter urin. Diberikan diet cair
secara bertahap, hingga mencapai diet cair penuh P.O.
Case II
??????
Fig. ?. Clinical Presentation Before Operation
?????
Fig. ?. ????
Pada kedua kasus kami informed consent. Saat bayi diruang perwatan dihangatkan
dengan menempatkan di inkubator. Dilakukan persiapan untuk repair hernia Morgagni dengan
laparoskopi. Sebelum operasi diberikan dipuasakan 4 jam sebelum operasi, O2 4 LPM dengan
kanul, pemberian antibiotik profilaksis sefalosporin generasi I 30 menit sebelum operasi,
dipersiapkan jalur intravena, pemasangan urin kateter.
Operasi
Operasi dilakukan dalam 4 jam. Pasien dibius dalam anestesi umum. Diposisikan supine
??. Setelah tindakan asepsis dan antisepsis lapangan operasi dilakukan insisi kulit di regio
infraumbilikal untuk menempatkan kamera, setelah terpasang kembungkan abdomen dengan
CO2 hingga mencapai tekanan yang sesuai. Pada eksplorasi tampak 1 loop colon transversum
masuk ke dalam celah hernia diafragma Margogni ukuran ?? cm yang bisa dibebaskan setelah
abdomen terisi CO2 dengan tekanan yang sesuai, tidak tampak kelainan lain intraabdomen.
Dilakukan juga insisi kulit di regio kiri atas abdomen, kanan atas abdomen, dan epigstrium.
Hingga menembus peritoneum. Masukkan masing-masing dengan alat tambahan. Lakukan repair
hernia diafragma Margogni dengan menjahit secara langsung tepi hernia sehingga tepi hernia
bertemu, menggunakan benang monofilamen non-absorbable. Setelah selesai eksplorasi, tidak
ada kelainan lain. Keluarkan alat laparoskopi dan CO2, jahit luka operasi. Operasi selesai
Hari 1-2
Pasien di knock down. Tanda-tanda vital dalam batas normal. Diberikan antibiotik,
analgetik, jaga kehangatan dengan ditempatkan dalam inkubator, jaga balance cairan, O2 kanul 4
LPM, TPN. Pengawasan tanda-tanda vital. Dipertahankan NGT dan kateter urin.
Hari 3-5
Keadaan umum sadar, aktif. Tanda-tanda vital dalam batas normal. Dilanjutkan
pemberian antibiotik???. analgetik, jaga kehangatan, jaga balance cairan, O2 kanul 4 LPM, TPN.
Pengawasan tanda-tanda vital. Tetap dipertahankan NGT dan kateter urin. Diberikan diet cair
secara bertahap, hingga mencapai diet cair penuh P.O.
Discussion
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