Case Report

Laparoscopic Repair of Hernia Morgagni (Antero-Medial Diapharghm) in

Dr. Kariadi Hospital-Semarang-Indonesia

Purba S, E. Dion P.* Wardani, Avriani Pety**

* Resident of General Surgery, Medical Faculty of Diponegoro University/Dr.Kariadi

Hospital Semarang-Middle Java-Indonesia

** Consultant of Pediatric Surgery Department, Medical Faculty of Diponegoro

University/Dr.Kariadi Hospital Semarang-Middle Java-Indonesia

Corresponding author

Dr. E. Dion P. Purba S.

Email: purba.dion@gmail.com

Resident of General Surgery

Medical Faculty of Diponegoro University/Dr.Kariadi Hospital Semarang

Jl. Dr. Soetomo No. 16, Semarang, Middle Java,Indonesia

Telp. (+62) 880251565

Background

Congenital diaphragmatic hernia (CDH) remains a major cause of respiratory failure in

the newborn with a high mortality and morbidity rate. The first reported case was an incidental
finding on postmortem evaluation in a 24-year-old man in 1679. (Congenital Malformation
Kumar) The earliest English language description of the gross anatomy and pathophysiology
associated with CDH in a newborn was by McCauley, an associate of Hunter, as reported in the
Proceedings of the Royal College of Physicans, 1754. Cooper, in 1827, and Laennec, in 1834,
not only reported clinical descriptions and gross pathology of CDH but also suggested that
laparotomy might be the proper approach for reduction and correction of the hernia. Bowditch, in
1847, was the first to make the bedside diagnosis of CDH and further emphasized the clinical
criteria for diagnosis. Although Bochdaleks understanding of the embryology was incorrect, this
congenital defect continues to carry his name. He speculated that the hernia resulted from a
posterolateral rupture of the membrane separating the pleuroperitoneal canal into two cavities.
He also incorrectly speculated that the best way to repair the defect was through the bed of the
12th rib. The record is not clear as to whether this was actually attempted. The earliest, although
unsuccessful, efforts to repair CDHs were by Nauman, in 1888, and ODwyer, in 1890. The first
reports of successful repairs were in an adult by Aue, in 1901, and a child by Heidenhain, in
1905. The groundwork for treating CDH in the newborn period was laid by Hedblom, whose
review of the reported cases as of 1925 showed that 75% of 44 infants diagnosed in the newborn
period died. He suggested that earlier intervention might improve survival. Successful repair of
CDH remained rare until 1940, when Ladd and Gross reported 9 of 16 patients surviving
operative repair, the youngest being 40 hours old. It was not until 1946 that Gross reported
survival of the first infant younger than 24 hours old after operative repair of the defect. Until the
1980s, the standard of care remained immediate neonatal surgery followed by postoperative
resuscitative therapy. (Coran, 7th Ed)

Embryology : Diaphragm development

The development of the human diaphragm is a complex, multicellular, multi-tissue
interaction that is poorly understood. Precursors to the diaphragm begin to form during the 4th
week of gestation. Historically, the diaphragm was thought to develop from the fusion of four
embryonic components: (1) anteriorly by the anterior central tendon forms from the septum
transversum, (2) dorsolaterally by the dorsolateral portions form from the pleuroperitoneal
membranes, (3) dorsally by the dorsal crura evolve from the esophageal mesentery, and (4)
posteriorly by the body wall mesoderm (by the muscular portion of the diaphragm). (Aschrafts
Ed 6; Coran , Ed 7)

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)

Epidemiology and Genetics

The reported incidence of CDH is estimated to be between 1 in 2000 to 5000 births. In
the United States, approximately 1000 infants per year are affected with this condition, and in a
recent study from Atlanta the birth prevalence was found to be 2.4 per 10,000 births. (Coran, 7th
Ed; Aschrafts, Ed 6) The incidence in stillborns is less well documented. Approximately one
third of infants with CDH are stillborn, but these deaths are usually the result of associated fatal
congenital anomalies. When stillborns are counted with live births, females appear to be more
commonly afflicted than males according to Coran 7th edition, (Coran, 7th Ed) and infants with
isolated CDH are typically male according to Aschrafts 7th edition. (Aschrafts Ed 7)
Proportions of fetuses are prenatally diagnosed with CDH and will die in utero or shortly after
birth. Thus, many may never been seen or accounted for in a tertiary referral center. Presumed to
be the most severe of all CDH infants, these patients contribute to the hidden mortality of
CDH. (Aschrafts, Ed 6) Defects are more common on the left side, with approximately 80-85%
being left sided and 13-20% right sided. Bilateral CDH defects are rare, around 2%, and have a
high incidence of associated anomalies. (Coran, 7th Ed; Aschrafts, Ed 6) The posterolateral
Bochdalek hernia accounts for 90% of all diaphragmatic hernia cases. The anterior Morgagni
hernia along with defects of the central septum transversum. An anterior diaphragmatic
Morgagni hernia accounts for less than 2% of all CDH. (Aschrafts, Ed 6) The size of the defect
varies from small (2 or 3 cm) to very large, involving most of the hemidiaphragm. (Newborn
Surgey) Infants with isolated CDH are more likely to be premature, macrosomic, and male;
(Coran, 7th Ed) and about one third of affected infants may have associated major defects.
Women who are thin or underweight for their height may have an increased risk of having an
infant with an isolated CDH. (Coran, 7th Ed; Aschrafts, Ed 6)
CDH is thought to represent a sporadic developmental anomaly, although a number of
familial cases have been reported. The expected recurrence risk in a first-degree relative has been
estimated to be 1 in 45, or approximately 2%. Structural chromosomal abnormalities have been
identified in 9% to 34% of CDH infants and include trisomies, deletions, and translocations.
Specific chromosomes with deleted or translocated genes may be candidate loci for CDH
development. The combination of CDH and an abnormal karyotype has been associated with a
poor outcome. (Coran, 7th Ed) In addition, a gene distal to the 15q21 locus has been identified
for the normal development of the diaphragm. (Aschrafts, Ed 6)
The cause of CDH is unknown. As with other embryopathies, there is increasing
evidence that CDH may be due to the exposure of genetically predisposed or susceptible
individuals to environmental factors. Exposure to a number of pharmacologic agents and
environmental hazards has been implicated in its development. These include insecticides and
drugs, such as phenmetrazine, thalidomide, quinine, cadmium, lead, and nitrofen. The clinical
findings of vitamin A deficiency in CDH infants and the effects of vitamin A administration in
nitrofen-induced pulmonary hypoplasia have strengthened the evolving hypothesis that
alterations in retinoid-regulated target genes may be responsible for CDH development. (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)

ANTERIOR HERNIAS OF MORGAGNI

 An anterior diaphragmatic Morgagni hernia accounts for less than 2% of all CDH.
(Aschrafts, Ed 6) The defect occurs through the embryologic space of Larrey. (Coran, 7th Ed)
The foramen of Morgagni hernia results from failure of fusion of the crural and sternal portions
of the diaphragm. This can occur on either side at the junction of the septum transversum and
thoracic wall where the superior epigastric artery (internal mammary artery, intrathoracically)
traverses the diaphragm. (Aschrafts, Ed 6) Occasionally, bilateral Morgagni hernias
communicate in the midline, constituting a large anterior diaphragmatic defect extending all the
way across the midline from right to left. (Coran, 7th Ed) Typically, a hernia sac is present,
containing omentum, small intestine, and/or colon, (Aschrafts, Ed 6) Usually discovered to the
right or left of the midline. (Coran, Ed 7) Rarely these hernias will contain liver and/or spleen.
(Aschrafts, Ed 6) Although this defect may be observed in neonates, (Coran, 7th Ed) Morgagni
hernia are much less common and also much less morbid, (Fundamentals of Pediatric Surgery)
the majority of children with a Morgagni hernia are asymptomatic and are rarely diagnosed
during the neonatal period, (Aschrafts, Ed 6) it usually presents more commonly in older
children or adults. (Coran, 7th Ed; Fundamentals of Pediatric Surgery)
Many are discovered incidentally as an anterior chest mass or pneumonia.
(Fundamentals of Pediatric Surgery) Symptoms typically include general epigastric discomfort
or sometimes vomiting and coughing due to intermittent obstruction. An acute presentation may
be due to intestinal ischemia with necrosis and perforation as well as gastric volvulus.
(Aschrafts, Ed 6) Herniation into the pericardium causing tamponade has also been described.
(Aschrafts, Ed 6)
The chest radiograph may exhibit a well-defined airfluid level in the midline of the chest
and visceral herniation in the retrosternal space. (Aschrafts, Ed 6) Small hernias may require a
contrast radiograph or CT scan to confirm the diagnosis. (Aschrafts, Ed 6; Coran, 7th Ed)
Asymptomatic Morgagni hernias in asymptomatic adults can sometimes be observed, but
surgical repair is recommended in children. The surgical approach can be open or minimally
invasive, thoracic or abdominal. Laparoscopic repair is probably the most effective and has
become the standard at many centers. Nearly all have an associated hernia sac, which can often
only be partially removed because it is densely adherent to the pericardium. Primary repair is
usually feasible and can be performed by bringing the anterior and posterior portions of the
defect together with interrupted permanent sutures. (Fundamentals of Pediatric Surgery)
Operative correction is easily performed through an upper transverse abdominal incision. The
diaphragm is sutured to the underside of the posterior rectus sheath at the costal margin after
reduction of the hernia and resection of the sac. (Coran, 7th Ed) Laparoscopic and thoracoscopic
techniques have also been used to repair this defect, but the laparoscopic approach is generally
favored. (Coran, 7th Ed) Although most defects can be repaired primarily, large defects may
require patch closure. The long-term outcome regarding recurrence is yet to be defined.
Associated anomalies may be present and include malrotation, cardiac defects, and
trisomy 21. An anterior midline deficiency in the diaphragm, (Coran, 7th Ed) due to a failure in
the development of the septum transversum, (Aschrafts, Ed 6) with or without the other
elements of the pentalogy of Cantrell, with free pericardial and peritoneal communication may
allow herniation of intestine into the pericardium. (Coran, 7th Ed) An Pentalogy of Cantrell is a
rare cluster of congenital anomalies which includes omphalocele, cardiac defects, ectopic cordis,
and an anterior diaphragmatic defect extending into the pericardium. The cardiac defect is the
most severe problem and is the main cause of mortality. (Aschrafts, Ed 6)

Minimally Invasive Approaches

The respiratory sequelae and other morbidity seen after open CDH repair has prompted
surgeons to adopt minimally invasive surgical approaches. Both thoracoscopic and laparoscopic
repairs have been performed. Data from the CDHR show that laparoscopic and thoracoscopic
strategies are being used worldwide, and have been utilized in 20% of centers since 1995.
Minimally invasive surgical techniques have been used for primary repair as well as prosthetic
patch closure with suggested advantages of less postoperative pain, avoidance of thoracotomy-
associated complications, and an overall reduction of surgical stress. (Aschrafts, Ed 6)
Minimal access surgical techniques are increasingly being applied to the repair of
congenital diaphragmatic hernia. Despite the seemingly straightforward concept of suture repair
or patch replacement of a simple tissue defect, many technical challenges remain. These include:
how best to reduce the hernia contents and keep them from getting in the way during the repair,
how to place the sutures securely and in such a way that tension is distributed evenly, and how
best to place a patch in such a small space without increasing the risk of recurrent herniation. It is
also usually not feasible to perform a minimally invasive operation in the NICU, making it
necessary to transport a potentially labile infant a long distance to the operating room.
(Fundamental of Pediatric Surgery)
The thoracoscopic approach has the theoretical advantage of one not having to work
around the reduced abdominal organs. It is also a larger and cleaner field to work in. Some
believe that the positive pressure generated by CO2 insufflation helps to push the bowel down
into the abdomen, which is true at first, but once the gas fills the abdominal cavity, as it
inevitably does through the hernia defect, the pressure gradient disappears. In addition, gas
insufflation in the chest can compromise ventilation and venous return and if the child is
positioned in a decubitus position, ventilation of the dependent good lung is further limited.
Thoracoscopy is sometimes made more difficult in newborns due to very high end-tidal CO2
levels, which often requires high inspiratory pressures and increased minute ventilation, options
not considered safe in most infants with CDH. Nevertheless, many feel the thoracoscopic
approach is superior and allows them to place multiple sutures that are brought out through the
chest wall before being tied down one by one to allow an even distribution of tension. In the end,
the surgeon must resist the temptation to cut corners or leave even a single stitch that is
inadequate if the operation is not being done with same attention to detail and quality as the
open repair, then it should not be done. (Fundamental of Pediatric Surgery)
The laparoscopic approach is feasible and especially useful for smaller defects, Morgagni
hernias, and when dealing with older children. It is common to require an extra port for retraction
of abdominal contents and proper exposure. A liver retractor is useful to hold the spleen and
stomach down and away from the surgical field. The same principle of tensionfree closure is
paramount. Even with experience, good exposure, and meticulous technique, these operations
can be very long and extremely tedious, making operator fatigue and exposure of a fragile infant
to an excessively long operation potential patient safety concerns that need to be weighed
carefully when deciding on the best approach. This is especially true for large defects that require
patch closure, which should probably be an indication for conversion to open. (Fundamental of
Pediatric Surgery)

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. ??

Pemeriksaan Penunjang. Laboratorium : Hb 12,8 gr%; leukosit 7.400/mm; trombosit

297.000 /mm3; PPT 10,9 detik; PPTK 31,4 detik; ureum 35 mg/dL; Kreatinin 0,42 mg/dL;
albumin 3,7 g/dL; GDS 120 mg/dL; natrium 140 mmol/L; kalium 4,7 mmol/L; klorida 105
mmol/L.
.

Fig. 2. Clinical Presentation Before Operation

X-Foto

Dada

Fig. 3. ????

Barium Follow Through

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

Manajemen Pasca Operasi

Hari 1-5 pasien dirawat di PICU.

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

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 Fig. ?. Clinical Presentation Before Operation

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 Fig. ?. ????

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

Manajemen Pasca Operasi

Hari 1-5 pasien dirawat di PICU.

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

References

1. Furst H, Hartl WH, Lohe F, Schildberg FW. Colon interposition for Esophageal
Replacement : An Alternative Technique Based on the use of the right colon.2000.
Annals of surgery vol 231, No. 2, 173 178.
2. DeMeester TR. Esophageal Replacement With Colon Interposition. Operative
Techniques in Cardiac and Thoracic Surgery, Vol 2, No 1; 1997; pp 73 86.
3. Strutyska M, Karpiska. Esophageal Reconstruction With Large Intestine. 2012.
4. Agha FP, Orringer MB. Colonic Interposition : Radiographic Evaluation. AJR 142:703
708. 1984.
5. Cense HA, Visser MRM, Sandick JWV, De Boer AGEM, Lamme B, Obertop H, et al.
Quality of Life After Colon Interposition by Necessity for Esophageal Cancer
Replacement. J.surg. Oncol. 2004;88:32 38.