ENDORECTAL PULL-THROUGH SOAVE MODIFIKASI · 2018. 10. 11. · BOCHDALEK HERNIA Yuliaji Narendra...

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Transcript of ENDORECTAL PULL-THROUGH SOAVE MODIFIKASI · 2018. 10. 11. · BOCHDALEK HERNIA Yuliaji Narendra...

Page 1: ENDORECTAL PULL-THROUGH SOAVE MODIFIKASI · 2018. 10. 11. · BOCHDALEK HERNIA Yuliaji Narendra Putra, Tubagus Odih Rhomdani Wahid, Guntur Surya Alam, Rohadi 40-43 EVALUASI FAKTOR

| Volume 2 | Number 2 | Juli 2018|

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Volume 2 ● Number 2 ● Juli 2018 Jurnal Bedah Nasional

Program Studi Ilmu Bedah FK Universitas Udayana dan IKABI cabang Bali ● ISSN: 2548-5962

DAFTAR ISI

Halaman

BOCHDALEK HERNIA

Yuliaji Narendra Putra, Tubagus Odih Rhomdani Wahid, Guntur Surya Alam,

Rohadi

40-43

EVALUASI FAKTOR RISIKO YANG MEMPENGARUHI LUARAN

OPERASI ENDORECTAL PULL-THROUGH SOAVE MODIFIKASI

SOEWARNO PADA PENYAKIT HIRSCHSPRUNG

Yuliaji Narendra Putra

44-50

CASE REPORT OF A NEONATE WITH CONGENITAL LUMBAR HERNIA

Stephanus Haryanto Hokardi, Neil Angelo S. Sael

51-55

BILATERAL GIANT FIBROADENOMA OF THE BREAST: A CASE

REPORT

Jasmine Stephanie Christian, Putu Anda Tusta Adiputra, INW. Steven Christian

56-59

GOITER MULTINODUL DENGAN PELEBARAN KE

RETROSTERNAL: LAPORAN KASUS

Gede Budhi Setiawan

60-63

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CASE REPORT

40 | Jurnal Bedah Nasional

BOCHDALEK HERNIA

Yuliaji Narendra Putra1, Tubagus Odih Rhomdani Wahid2, Guntur Surya Alam2, Rohadi3

1General Surgery Training Programme, Faculty of Medicine Gadjah Mada University, Dr. Sardjito Hospital.

Correspondence: [email protected]. 2Pediatric Surgery Training Programme, Faculty of Medicine Gadjah Mada University, Dr. Sardjito Hospital.

3Pediatric Surgery Division, Faculty of Medicine Gadjah Mada University, Dr. Sardjito Hospital.

ABSTRACT

Background: Bochdalek hernia is a congenital defect on posterolateral diaphragm with an

abnormal connection between the thoracic cavity and the abdominal cavity. This disease causes

protrusion of abdominal organs into the thoracic cavity. Case: an 8-day-old baby girl admitted to

hospital with shortness of breath 24 hours after delivered. The baby was born spontaneously assisted

by midwife. Upon born, the baby was crying strongly and meconium came out 2 hours after birth. On

physical examination, the abdomen was inspected flat. Darm contour and darm steifung was

observed, and peristaltic sound was heard on left lung. Radiological examination demonstrated a

diaphragmatic hernia with ileus obstruction. The patient underwent laparatomy and stomach, ileum,

transverse colon, and spleen, was found on foramen Bochdalek. Post-surgery chest X Ray showed

favourable result. Ten days after treatment, the patient was discharged in a good condition with no

respiratory or digestive problems. After 1 months the patient’s condition remained good and there

were no respiratory or digestive complaints. Conclusion: In a rare case like Bochadalek hernia,

laparotomy performed as a promising attempt to return the anatomic position of organ.

Keywords: Bochdalek hernia, laparotomy, surgery.

HERNIA BOCHDALEK

Yuliaji Narendra Putra1, Tubagus Odih Rhomdani Wahid2, Guntur Surya Alam2, Rohadi3

1Program Studi Ilmu Bedah, Fakultas Kedokteran Universitas Gadjah Mada, RSUP Dr. Sardjito. Korespondensi:

[email protected].

2Program Studi Ilmu Bedah Anak, Fakultas Kedokteran Universitas Gadjah Mada, RSUP Dr. Sardjito.

3Divisi Bedah Anak, Fakultas Kedokteran Universitas Gadjah Mada, RSUP Dr. Sardjito.

ABSTRAK

Latar belakang: hernia Bochdalek adalah cacat bawaan pada diafragma posterolateral yang

terdapat hubungan antara rongga thoraks dan rongga perut, sehingga terjadi penonjolan organ perut ke

dalam rongga thoraks. Kasus: seorang bayi perempuan umur 8 hari dibawa ke rumah sakit swasta

dengan keluhan sesak nafas 24 jam setelah dilahirkan. Satu minggu yang lalu, pasien lahir spontan

ditolong bidan,menangis kuat, mekonium keluar 2 jam setelah lahir. Pada pemeriksaan fisik

didapatkan abdomen flat,didapatkan darm contour dan darm steifung, Suara peristaltik terdapat pada

auskultasi di hemithoraks kiri. Pada pemeriksaan radiologis terdapat gambaran udara usus pada

hemithoraks kiri. Dari pemeriksaan diatas ditegakkan diagnosis hernia diafragmatika dengan ileus

obstruksi. Dilakukan laparotomi, durante operasi didapatkan gaster, ileum, kolon transversum, dan

lien masuk ke dalam rongga thoraks sinistra, defek ukuran 2x8 cm pada posterior (foramen

Bochdalek). Rontgen thoraks post laparotomy paru-paru mengembang kedua sisi dan diafragma kiri

terlihat intak. Sepuluh hari setelah menjalani perawatan, pasien pulang dengan keadaan baik tidak ada

gangguan pernafasan maupun pencernaan. Evaluasi selama 1 bulan terkhir keadaan pasien tetap baik

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Volume 2 ● Number 2 ● Juli 2018 Bochdalek Hernia

41

dan tidak ada keluhan pernafasan maupun pencernaan. Simpulan: telah dilakukan laparotomi untuk

mengembalikan ke posisi anatomi pada pasien hernia Bochdalek dengan hasil baik.

Kata kunci: hernia Bochdalek, laparotomi, operasi.

INTRODUCTION

Bochdalek hernia is a congenital defect

on posterolateral diaphragm with an

abnormal connection between the thoracic

cavity and the abdominal cavity, resulting

in the protrusion of the abdominal organs

into the thoracic cavity.1 The incidence in

neonates is 1:2000-50002-3 with pulmonary

hypoplasia as the most common

complication.4 Despite advances in the

diagnosis and treatment of congenital

defects, pulmonary hypoplasia still caused

a fairly high mortality rate.5,6

Surgical treatment can be performed

either through the abdominal or thoracic

approach. The abdominal approach has the

advantage of correcting malrotation at the

same time.7 However, closure of the

abdominal wall can result in the difficulty

of organs replacement to abdominal cavity.

For the solution, Charles et al has

recommended a delayed abdominal muscle

closure. In this case, it will be presented

regarding the management of Bochdalek

hernia in children.

CASE REPORT

An 8-day-old baby girl was admitted

taken to a private hospital with shortness

of breath. The patient was born

spontaneously assisted by midwife, crying

strongly, and meconium came out 2 hours

after birth. At 24 hours later, the patient

appeared to have difficulty of breathing,

and referred to a primary hospital. After 6

days of treatment, no improvement was

observed, thus the patient was referred to a

tertiary hospital.

In physical examination, the baby

showed high body temperature, shortness

of breath with respiratory rates 39 times

per minute, but the baby could cry

strongly. Chest x-ray examination showed

multiple cystic shadow at the left

hemithorax accompanied by right

deviation of mediastinum and trachea with

suspicion of diaphragmatic hernia. On the

abdominal x-ray, the distal end of the

nasogastric tube only reached 5th-6th

thoracic vertebra with intestinal air

portrayed on the left chest. Babygram

examination showed that left intestine on

hemithorax was unfilled with contrasts, the

air intestinal image on the left hemithorax

is not filled with contrasts, with left

diaphragmatic hernia image showing

intestines and colon (Figure 1).

Figure 1. Babygram pre-surgery.

Preoperative diagnosis is left

diaphragmatic hernia and laparotomy

surgery via abdominal approach was

decided. A transverse incision of the upper

umbilical (3 cm above of umbilicus),

deepened layer by layer to the peritoneum.

After the peritoneum was opened,

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Yuliaji Narendra Putra Jurnal Bedah Nasional

42

intestinal, gastric, and part of transverse

colon system were seen inside of the

abdomen. The inlet of foramen Bochdalek

was seen released and re-inserted the

content of the hernia consisting ileum and

part of colon to the inside of the abdomen

cavity (Figure 2). The diameter of

foramen Bochdalek was 2x8 cm, then

incision on septum tendinous was made

then 2 layers was sutured. Aspiration of

pleura cavity was performed with

nasogastric tube. Exploration showing

intestinal systems is intact. The surgical

wound was closed layer by layer. The

surgery was completed.

Figure 2. Clinical picture during surgery.

Chest X-ray after surgery showed

inflation of left lung. The patient was

treated for 10 days and discharged on the

11th day after the surgery. Follow up for 1

months shows patient’s good condition

and neither disturbance was observed in

the digestive system nor respiratory

system.

DISCUSSION

Bochdalek hernia (Figure 3) is a

congenital defect on posterolateral

diaphragm developing an abnormal

connection between the thoracic cavity and

the abdominal cavity. This pleuroparietal

canal normally closed by the pleuroparietal

membrane at the 8th to 10th week of

pregnancy. Failure of canal closure might

result in Bochdalek hernia. Approximately

80-90% cases of Bochdalek hernia occur

on the left side and are very rare in adults.

In this case, a large defect in the left

posterolateral diaphragm (Bochdalek

foramina) was encountered with the

content of ileum and part of colon.4,5

Bochdalek hernia diagnosed from

anamnesis, physical examination,

investigation especially chest x-ray and

OMD.6

Figure 3. Location of Bochdalek Foramen.8

The diagnosis of this patient was made

after physical and radiological examination

because rarity of this disease and the non-

specific diagnosis could lead to delay of

diagnosis. Surgical repair of the defect is

the recommended therapy for Bochdalek

Hernia.7 For this patient, laparotomy

surgery was performed with abdominal

approach.8 A transverse incision of the

upper umbilical (3 cm above of

umbilicus), deepened layer by layer to the

peritoneum. After the peritoneum was

opened, intestinal system, gastric, and part

of transverse colon was seen inside of the

abdomen. Post-surgery showed inflation of

left lung. Long term follows up was also

favourable. This showed that laparotomy

for management of Bochdalek Hernia led

to either short term and long term results.

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Volume 2 ● Number 2 ● Juli 2018 Bochdalek Hernia

43

CONCLUSION

Laparotomy performed as an attempt to

return the anatomic position of patient with

Bochdalek hernia showing good result.

REFERENCES

1. Brunicardi FC, Billiar TR, Dunn DL,

et al. Congenital Diaphragmatic

Hernia. In: Schwartz SI, Fischer JE,

Daly JM, et al., editors. Schwartz’s

Principles of Surgery. 7th Edition.

Asian Student Edition. Singapore:

McGraw-Hill; 1998. p.1691-3.

2. Way LW. Esophagus & Diaphragm.

In: Way LW, Doherty GM, editors.

Current Surgical Diagnosis and

Treatment. 11th Edition. Connecticut:

McGraw-Hill/Appleton & Lange;

2002. p.438-40.

3. Townsend CM, Beauchamp RD,

Mattox KL, et al. Congenital

Diaphragmatic Hernia In: Townsend

CM, Beauchamp RD, Evers BM, et al.,

editors. Sabiston Textbook of Surgery:

The Biological Basis of Modern

Surgical Practice. 16th Edition.

Philadelphia: WB Saunders; 2001.

p.1480-3.

4. Eisenberg RL. Esophagus and

Diaphragmatic Hernia In: Eisenberg

RL, editor. Diagnostic Imaging in

Surgery. New York: McGraw-Hill;

1987. p.275-6.

5. Liechty RD. Respiratory

Diaphragmatic Hernia. In: Niederhuber

JE, editor. Fundamentals of Surgery.

Stamford: Appleton & Lange; 1998.

p.192-3.

6. Reynolds M. Diaphragmatic

Anomalies. In: Raffensperger JG,

editor. Swenson’s Textbook of

Paediatric Surgery. 5th edition. New

York: Appleton & Lange; 1990. p.

721-30.

7. Burki T, Amanullah A, Rehman AU, et

al. Late Presentation of Bochdalek

Hernia with Intestinal Symptoms. J

Ayub Med Coll Abbottabad. 2002;14:

27-8.

8. Nason LK, Walker CM, McNeeley

MF, et al. Imaging of the Diaphragm:

Anatomy and Function.

RadioGraphics. 2012;32:E51-70.

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ORIGINAL ARTICLE

44 | Jurnal Bedah Nasional

EVALUASI FAKTOR RISIKO YANG MEMPENGARUHI LUARAN

OPERASI ENDORECTAL PULL-THROUGH SOAVE MODIFIKASI

SOEWARNO PADA PENYAKIT HIRSCHSPRUNG

Yuliaji Narendra Putra

Program Studi Ilmu Bedah, Fakultas Kedokteran Universitas Gadjah Mada, RSUP Dr. Sardjito. Korespondensi:

[email protected].

ABSTRAK

Tujuan: untuk mengevaluasi luaran operasi endorectal pull-through Soave modifikasi Soewarna

pada pasien Hirschsprung. Metode: penelitian ini adalah penelitian deskriptif pada 48 penderita

Hirschsprung, dimana data dikumpulkan dengan wawancara langsung ke orang tua penderita. Setelah

didapatkan skoring, kemudian dinilai angka keberhasilan penatalaksanaan operasi Soave modifikasi

Soewarno, kemudian dilakukan analisis dengan menggunakan uji statistik Chi-kuadrat. Analisis

antara skor Klotz dengan faktor prognostik, seperti berat badan lahir, status gizi, berat badan saat

operasi, kadar albumin, kadar hemoglobin, kadar kalium, lama perawatan, dan jenis kelamin di

analisis dengan uji Chi-Kuadrat. Hasil: pada penelitian ini, didapatkan pada faktor-faktor risiko yang

mempengaruhi luaran operasi endorectal pull-through Soave modifikasi Soewarno adalah status gizi

p<0,001 dengan RR 28,0 dan albumin p=0,047 dengan RR 1,23. Faktor risiko hemoglobin (p=0,372),

kalium (p=0,256), berat badan lahir (p=0,66), berat badan saat operasi (p=0,0605), lama operasi

(p=0,941), dan lama perawatan (p=0,683) tidak berpengaruh secara signifikan. Simpulan: Status gizi

dan kadar albumin menjadi faktor prognosis signifikan terhadap luaran pasien dengan penyakit

Hirchsprung yang dilakukan tindakan operasi Soave modifikasi Soewarno.

Kata kunci: penyakit Hirschsprung, faktor prognostik, skor Klotz.

EVALUATION OF RISK FACTORS AFFECTING OUTCOME OF SOAVE

ENDORECTAL PULL-THROUGH OPERATION SOEWARNO

MODIFICATION IN HIRSCHSPRUNG’S DISEASE

Yuliaji Narendra Putra

General Surgery Training Programme, Faculty of Medicine Gadjah Mada University, Dr. Sardjito Hospital.

Correspondence: [email protected].

ABSTRACT

Objective: to evaluate the outcome of Soave endorectal pull-through operation with Soewarno

modification in Hirschsprung patients. Methods: this study was a descriptive study of 48

Hirschsprung patients, where data were collected by direct interviews to the patient's parents. After

scoring then assessed the success rate of Soave operation Soewarno modification, then analyzed by

using statistic Chi-square test. The analysis between Klotz score with prognostic factors, such as birth

weight, age of Soave, nutritional status, body weight during surgery, albumin level, hemoglobin level,

potassium level, length of treatment, and sex in analysis with Chi-Square test. Results: the significant

prognostic factors affected the outcome of Soave endorectal pull-through operation with Soewarno

modification in Hirschsprung patients were nutritional status (RR 28; p=0.047) and hemoglobin (RR

1.14; p<0.001). Others factors like hemoglobin (p=0.372), kalium (p=0.256), birth weight (p=0.66),

weight before surgery (p=0.0605), duration of surgery (p=0.941), dan length of hospital stay

(p=0.683). Conclusion: nutritional status and hemoglobin level were the most significant factors that

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Volume 2 ● Number 2 ● Juli 2018 Evaluasi Faktor Risiko yang Mempengaruhi

45

affected the outcome of Hirchsprung’s disease performed Soave operation with Soewarno

modification.

Keywords: Hirschsprung's disease, prognostic factor, Klotz score.

PENDAHULUAN

Penyakit Hirschsprung disebut juga

megakolon kongenital merupakan kelainan

tersering dijumpai sebagai penyebab

obstruksi usus pada neonatus. Pada

penyakit ini, tidak dijumpai pleksus

mienterikus sehingga bagian usus tersebut

tidak dapat mengembang.1,2 Angka

insidensi Hirschprung adalah 1 diantara

5000 kelahiran, maka dengan penduduk

220 juta dan tingkat kelahiran 35 per mil,

diperkirakan akan lahir 1400 bayi setiap

tahunnya dengan penyakit Hirschsprung di

Indonesia.3-6

Pasien dengan penyakit Hirschsprung

harus dikelola segera setelah diagnosis

ditegakkan.3 Prosedur Soave disebut juga

prosedur pull-through ekstramukosa

endorektal dari Soave. Di Subbagian

Bedah Anak FK UGM/RSUP Dr.Sardjito

Yogjakarta, Soave dikerjakan mulai awal

tahun 1990, dalam pelaksanaannya

didapatkan kesulitan dalam pengupasan

mukosa, sehingga diciptakan teknik

prosedur modifikasi Soewarno dan hingga

saat ini dijadikan prosedur tetap untuk

penanganan penderita Hirschsprung di Sub

Bagian Bedah Anak UGM/RS Dr. Sardjito

Yogyakarta. Walaupun begitu, proses

penyembuhan atau keberhasilan setelah

operasi sangat tergantung pada sistem

imun dan kemampuan adaptasi pasien.

Dalam hal ini yang berpengaruh adalah

usia, kadar hemoglobin, kadar kalium

serum, kadar albumin serum, status gizi,

berat badan lahir, berat badan saat operasi,

dan lama perawatan. Tujuan penelitian ini

mengevaluasi luaran operasi endorectal

pull-through Soave modifikasi Soewarno

pada pasien Hirschsprung.

METODE

Penelitian ini dilakukan dengan

rancangan analitik deskriptif cross

sectional pada penderita Hirshsprung yang

ditatalaksana dengan metode Soave

modifikasi Soewarno di RS. Dr. Sardjito

Yogyakarta antara bulan Januari 2005

sampai Februari 2008. Tujuan penelitian

adalah untuk mencari faktor risiko yang

berpengaruh terhadap luaran pasien

Hirschsprung yang ditatalaksana dengan

prosedur Soave modifikasi Soewarno.

Parameter yang dinilai untuk variabel

independen (risiko) meliputi usia, kadar

hemoglobin, kadar kalium serum, kadar

albumin serum, status gizi, berat badan

lahir, berat badan saat operasi, dan lama

perawatan. Variabel dependen atau luaran

hasil operasi dinilai dengan skor Klotz.

Semua parameter didapatkan datanya dari

rekam medis dan wawancara atau

pengukuran langsung. Skor Klotz ≤13

dinyatakan baik/cukup dan dinyatakan

jelek/kurang jika skor Klotz >13.

Pada seluruh subjek penelitian, harus

menyetujui informed consent dari orang

tuanya. Data usia, status gizi, dan berat

badan diperoleh dengan melakukan

alloanamnesis dan pemeriksaan fisik.

Pemeriksaan barium enema dilakukan

untuk mengetahui penyakit Hirschsprung,

seperti adanya penyempitan rektosigmoid,

daerah transisi, daerah dilatasi dan

ketidakteraturan mukosa. Tindakan bedah

definitif dilakukan pada semua pasien

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Yuliaji Narendra Putra Jurnal Bedah Nasional

46

yang menderita penyakit Hirschsprung

yang sudah dikerjakan kolostomi. Jenis

prosedur bedah yang dikerjakan adalah

prosedur pull-through ekstramukosa

endorektal prosedur Soave modifikasi

Soewarno.

Prosedur operasi modifikasi Soewarno

dilakukan dengan irisan transversal pada

dinding depan abdomen mulai 4 cm

sebelah medial SIAS (spina iliaka anterior

superior) kanan melalui garis langer

sampai mencapai lobang kolostomi. Irisan

delanjutkan melengkung ke kraniolateral

secukupnya. Arteri hemorrhoidalis

superior dan arteri sigmoidalis di

identifikasi selanjutnya diikat dan

dipotong. Dilakukan reseksi kolon 3-4 cm

di proksimal kolostomi dan 1-2 cm di

proksimal refleksi peritoneum. Pungtum

proksimal kemudian ditutup, dilakukan

pengupasan mukosa rektum dari lapisan

seromuskuler, dengan cara memegang

mukosa dengan 4 buah klem Allis. Irisan

pertama dilakukan secara tajam

selanjutnya seromuskuler dipegang dengan

4 buah klem Allis, selanjutnya dilakukan

pengupasan secara tumpul. Pengupasan ke

anal sejauh mungkin sehingga mencapai

linea dentata. Selanjutnya dilakukan

pembebasan kolon proksimal yang sehat,

sampai cukup untuk diteroboskan keluar

anus. Pembebasan ini harus hati- hati

sehingga jalinan pembuluh darah tetap

terjamin. Bila sudah dinilai cukup, maka

operasi dilanjutkan lewat peritoneum.

Anus disiapkan, kemudian cerobong

mukosa ditarik dengan jalan memasukkan

sonde khusus dengan ujung berbentuk

kepala yang lebih besar. Mukosa diikat

pada leher sonde tersebut dan ditarik

keluar secara melipat terbalik. Kolon yang

sehat kemudian diteroboskan di dalam

cerobong mukosa. Lapisan mukosa

difiksasi dengan kolon dengan benang

plain catgut, dan dipasang rectal tube di

dalam kolon yang diteroboskan tersebut

sampai melewati sfingter ani. Operasi

dilanjutkan lewat abdominal, vesika

urinaria, dan organ abdomen lain ditata

kembali, cerobong seromuskuler difiksasi

dengan serosa kolon yang diteroboskan

dengan chromic catgut. Dilakukan

appendektomi insidental, rongga abdomen

dicuci dan ditutup lapis demi lapis.

Sepuluh hari setelah dioperasi endorectal

pull-through, telah terjadi perlekatan

antara cerobong seromuskuler dengan

serosa kolon. Dilakukan pemotongan

pungtum kolon yang diteroboskan 1 cm

proksimal linea dentata, dilanjutkan

dengan penjahitan mukosa dengan

mukosa. Selama 3 hari rectal tube terus

dipasang pada rektum yang baru sehingga

gangguan obstruksi akibat edema di daerah

anorektal dapat dihindari.8

Setelah operasi, peneliti mewawancara

orang tua pasien dengan menggunakan

kuesioner berisi tabel skor Klotz (Tabel 1)

terhadap hasil pembedahan operasi

definitif metode Soave modifikasi

Soewarno kemudian dibuat skoring Klotz

tiap pasien berdasarkan kuisioner hasil

wawancara peneliti dengan orang tua

pasien. Hasil skoring Klotz dicari

korelasinya dengan faktor prognostik

disajikan secara tekstular dan tabular dan

diuji statistik dengan Chi-kuadrat.

HASIL

Karakteristik subjek penelitian dari 48

kasus didapatkan jenis kelamin laki-laki

terdapat 35 orang (72,92%) dan

perempuan ada 13 orang (27,08%). Rata-

rata usia 627,32±525,36 hari dengan usia

terendah 1 hari dan maksimum 1980 hari

(5,42 tahun). Berat badan lahir rata-rata

2,93±0,27 kg dengan berat minimal 1,90

kg dan berat maksimal 3,60 kg. Status gizi

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yang baik ada 41 orang (85,4%),

sedangkan status gizi jelek ada 7 orang

(14,6%).

Tabel 1. Tabel Skor Klotz

No Variabel Kondisi Skor

1 Defekasi 1-2 kali sehari 1

2 hari sekali 1

3 – 5 kali sehari 2

3 hari sekali 2

> 4 hari sekali 3

> 5 hari sekali 3

2 Kembung Tidak Pernah 1

Kadang – kadang 2

Terus Menerus 3

3 Konsistensi Normal 1

Lembek 2

Encer 3

4 Perasaan ingin BAB Terasa 1

Tidak terasa 3

5 Soiling / Kecepirit Tidak pernah 1

Bersama Flatus 2

Terus menerus 3

6 Kemampuan menahan

feses yang akan keluar

Lebih dari satu menit 1

Kurang dari satu

menit

2

Tidak bisa 3

7 Komplikasi Lain Tidak ada 1

Minor 2

Mayor 3

Penilaian hasil skoring:

a. Skor 7: sangat baik

b. skor 8-9: baik

c. Skor 10-13: cukup

d. Skor 14-lebih: kurang/ jelek

Hasil analisis Klotz terhadap jenis

kelamin, hemoglobin, dan kalium, secara

statistik tidak bermakna dimana p>0,05.

Skor Klotz bermakna secra statistik pada

status gizi dan albumin, yang mana status

gizi dan albumin yang baik memiliki skor

Klotz yang baik (Tabel 2). Pada Tabel 3,

hasil analisis Klotz terhadap rata-rata

umur, berat badan lahir, berat badan saat

operasi, lama operasi, dan lama perawatan

secara statistik tidak bermakna p>0,05.

DISKUSI

Pasien dengan penyakit Hirschsprung

harus dikelola segera setelah diagnosis

ditegakkan. Pengobatan definitif

aganglionosis kolon adalah pembedahan

dengan membuang semua bagian yang

aganglionik, kemudian membawa usus

(kolon) yang normal persarafannya

(ganglionik) ke anus dengan

memperhatikan kontinensi. Tanpa

penanganan, tingkat mortalitas penyakit ini

80%, di mana pasien penyakit

Hirschsprung akan meninggal pada bulan-

bulan pertama kehidupannya, sebagian

besar pada masa neonatus. Keterlambatan

dan kegagalan tindakan bedah, baik

tindakan bedah sementara maupun bedah

definitif dapat mengakibatkan cacat

bahkan kematian.3

Prosedur Soave merupakan prosedur

pembedahan yang paling sering dilakukan.

Prosedur Soave konvensional dilakukan

dengan pemisahan mukosa dan

seromuskuler dengan prokain hidroklorida,

tepat di proksimal masuknya arteri

hemorrhoidalis superior ke lapisan otot

rektum. Pengupasan lapisan seromuskuler

dari lapisan mukosa dengan kasa bulat dan

kecil. Setelah lapisan seromuskuler

terpisah dengan mukosa dilakukan

pemotongan mukosa secara melintang

dilanjutkan dengan pembebasan kolon

proksimal sepanjang 30-50 cm di

proksimal daerah transisi. Di perineum,

mukosa kolon ditarik keluar lubang anus

dan dijahit.7

Untuk lebih memudahkan dengan hasil

luaran yang lebih baik, dilakukan prosedur

operasi modifikasi Soewarno yang

dilakukan dengan irisan transversal pada

dinding depan abdomen mulai 4 cm

sebelah medial SIAS kanan melalui garis

langer sampai mencapai lobang kolostomi.

Irisan dilanjutkan melengkung ke

kraniolateral secukupnya. Arteri

hemorrhoidalis superior dan arteri

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sigmoidalis di identifikasi selanjutnya

diikat dan dipotong.

Tabel 2. Analisis uji Chi-kuadrat antar faktor risiko dengan skor Klotz

Variabel Skor Klotz RR 95% CI p

Jelek Baik

Jenis kelamin (%)

Laki-laki 1 (20,0) 12 (27,9) 0,67 0,08-5,48 0,706

Perempuan 4 (80,9) 31 (72,1)

Status gizi (%)

Kurang baik 4 (80) 2 (4,7) 28,0 3,72-210,5 0,000

Baik 1 (20) 41 (95,3)

Albumin (%)

Jelek (<3,5 g/dL) 0 (0) 21 (48,8) 1,23 1,02-1,47 0,047

Baik 5 (100) 22 (51,2)

Hemoglobin (%)

Jelek (<11 g/dL) 0 (0) 6 (14) 1,14 1,02-1,27 0,372

Baik 5 (100) 37 (86)

Kalium (%)

Jelek (<3,5 mmol/L) 0 (0) 9 (20,9) 1,15 1,02-1,29 0,256

Baik 5 (100) 34 (79,1)

RR: risiko relatif, CI: confidence interval

Tabel 3. Hasil skor Klotz terhadap rata-rata umur, berat badan lahir, berat badan saat operasi,

dan lama operasi, dan lama perawatan

Variabel Skor Klotz (rata-rata ± standar deviasi) p

Jelek Baik

Umur (hari) 439,60 ± 406,11 649,1 ± 537,1 0,405

Berat badan lahir (kg) 2,98 ± 0,265 2,93 ± 281,47 0,660

Berat badan saat operasi (kg) 11,40 ± 1,67 8,1 ± 3,84 0,065

Lama operasi (jam) 2,45 ± 0,447 2,47 ±0,60 0,941

Lama perawatan (hari) 12,6 ± 1,34 13,39 ± 4,26 0,683

Dilakukan reseksi kolon 3-4 cm di

proksimal kolostomi dan 1-2 cm di

proksimal refleksi peritoneum. Pungtum

proksimal kemudian ditutup, dilakukan

pengupasan mukosa rektum dari lapisan

seromuskuler, dengan cara memegang

mukosa dengan 4 buah klem Allis. Irisan

pertama dilakukan secara tajam

selanjutnya seromuskuler dipegang dengan

4 buah klem Allis, selanjutnya dilakukan

pengupasan secara tumpul. Pengupasan ke

anal sejauh mungkin sehingga mencapai

linea dentata. Selanjutnya dilakukan

pembebasan kolon proksimal yang sehat,

sampai cukup untuk diteroboskan keluar

anus. Pembebasan ini harus hati- hati

sehingga arkade pembuluh darah tetap

terjamin. Bila sudah dinilai cukup, maka

operasi dilanjutkan lewat peritoneum.

Anus disiapkan, kemudian cerobong

mukosa ditarik dengan jalan memasukkan

sonde khusus dengan ujung berbentuk

kepala yang lebih besar. Mukosa diikat

pada leher sonde tersebut dan ditarik

keluar secara melipat terbalik. Kolon yang

sehat kemudian diteroboskan di dalam

cerobong mukosa. Lapisan mukosa

difiksasi dengan kolon dengan benang

plain catgut, dan dipasang rectal tube di

dalam kolon yang diteroboskan tersebut

sampai melewati sfingter ani. Operasi

dilanjutkan lewat abdominal, vesika

urinaria, dan organ abdomen lain ditata

kembali, cerobong seromuskuler difiksasi

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49

dengan serosa kolon yang diteroboskan

dengan chromic catgut. Dilakukan

appendektomi insidental, rongga abdomen

dicuci dan ditutp lapis demi lapis.

Sepululuh hari setelah dioperasi endorectal

pull-through, telah terjadi perlekatan

antara cerobong seromuskuler dengan

serosa kolon. Dilakukan pemotongan

pungtum kolon yang diteroboskan 1 cm

proksimal linea dentata, dilanjutkan

dengan penjahitan mukosa dengan

mukosa. Selama 3 hari rektal tube terus

dipasang pada rektum yang baru sehingga

gangguan obstruksi akibat udema di

daerah anorektal dapat dihindari.8

Di Subbagian Bedah Anak FK

UGM/RSUP Dr.Sardjito Yogjakarta,

Soave dikerjakan mulai awal tahun 1990,

dalam melaksanakan tersebut didapatkan

kesulitan dalam pengupasan mukosa,

sehingga diciptakan teknik prosedur

modifikasi Soewarno dan hingga saat ini

dijadikan prosedur tetap untuk penanganan

penderita Hirschsprung di Sub Bagian

Bedah Anak UGM/RS Dr. Sardjito

Yogyakarta. Walaupun begitu, proses

penyembuhan atau keberhasilan setelah

operasi sangat tergantung pada sistem

imun dan kemampuan adaptasi pasien.

Dalam hal ini yang berpengaruh adalah

Usia,kadar hemoglobin, kadar kalium

serum, kadar albumin serum, status gizi,

berat badan lahir, berat badan saat operasi,

dan lama perawatan.

Hasil analisis skor Klotz pada pasien

dengan penyakit Hirchsprung yang

dilakukan tindakan operasi Soave

modifikasi Soewarno dipengaruhi faktor

prognosis berupa status gizi (p=0,000

dengan RR 28,0) dan albumin (p=0,047

dengan RR 1,23) yang secara statistik

signifikan. Sedangkan faktor lain seperti

jenis kelamin, hemoglobin, kalium, umur,

berat badan lahir, berat badan saat operasi,

lama operasi, dan lama perawatan tidak

berpengaruh pada skor Klotz.

SIMPULAN

Teknik operasi endorectal pull-through

Soave modifikasi Soewarno merupakan

pilihan terapi pada penyakit Hirschsprung.

Penyakit Hirchsprung yang dilakukan

tindakan operasi Soave modifikasi

Soewarno dipengaruhi faktor prognosis

berupa status gizi dan albumin, yang dapat

mempengaruhi penyembuhan paska

tindakan.

DAFTAR PUSTAKA

1. Soewarno. Tatalaksana penderita

penyakit Megakolon kongenital pada

bayi dan anak dengan prosedur

Duhamel di RSUP Dr. Sardjito.

Naskah Pertemuan Ilmiah alumnus FK

UGM, HUT FK UGM XI dan HUT

RSUP Dr. Sardjito IV. Yogyakarta: FK

UGM; 1986.

2. Swenson O, Sherman JO. Diagnosis of

Congenital Megacolon: an analysis of

501 patient. J Pediatric Surgery.

1973;8:587-94.

3. Kartono D. Penyakit Hirschsprung,

Perbandingan prosedur Swenson dan

Duhamel modifikasi [disertasi].

Jakarta: Universitas Indonesia; 1993.

4. Yoshida Jr C. Hirschsprung Disease.

Department of Diagnosis Imaging

federal of University of Sao Paulo

(UNIFEST); 2004.

5. Kartono D. Penyakit Hirschsprung.

Jakarta: Sagung Seto; 2004.

6. Holschneider A, Ure BM.

Hirschsprung's disease. Dalam:

Ashcraft KW, Murphy JP, Sharp RJ,

dkk, penyunting. Pediatric Surgery.

Edisi ke-3. Philadelphia: Saunders;

2000. p.453-72.

7. Sieber WK. Hirschsprung’s Disease.

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Yuliaji Narendra Putra Jurnal Bedah Nasional

50

Dalam: Welch KJ, Randolph JG,

Ravitch MM, dkk, penyunting.

Pediatric Surgery. Edisi ke-4.

Chicago: Year Book Medical Publisher

Inc; 1986. p.995-1019.

8. Santos MC, Giacomantonio JM, Lau

HYC. Primary Swenson pull-through

compared with multiple-stage pull-

through in the neonate. Journal of

Pediatric Surgery. 1999;34:1079-81.

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CASE REPORT

Jurnal Bedah Nasional | 51

CASE REPORT OF A NEONATE WITH CONGENITAL SUPERIOR

LUMBAR HERNIA

Stephanus Haryanto Hokardi*, Neil Angelo S. Sael

Department of Surgery, De Los Santos Medical Center, 201 E. Rodriguez Sr. Ave., Quezon City, NCR,

Philippines. *Correspondence: [email protected].

ABSTRACT

Background: congenital lumbar hernias are rare. It constitutes to 20% of all lumbar hernias which

is less than 1.5% of all the abdominal wall hernias. There are no more than 50 cases reported in

literature till date. We report a case of congenital lumbar hernia in a preterm female neonate located

on the superior lumbar triangle. Case: a preterm female neonate was born, presented with a mass at

the right lumbar area with a size of 8x8 cm, round, movable with bluish discoloration, well delineated

border, no visible veins, increases in size when the patient cries, and reduces easily. Ultrasonography

revealed a right posterolateral abdominal mass measuring 4.2x2.88x1.59 cm. CT scan revealed right

posterolateral mid-abdominal wall hernia with protrusion and no intestinal obstruction. The patient

underwent exploratory laparotomy, where hernia defect was about 2 cm in diameter in the right

posterior abdominal wall, pararenal area, and just below the 12th rib. The ascending colon and parts of

the ileum were adherent inside the hernia defect at the right lumbar area. Primary closure of the hernia

defect was done by suturing the psoas major and the transversus abdominis and internal oblique

muscles. The postoperative, patient had good bowel movement, no abdominal distention or vomiting.

Feeding was then started and well tolerated. After two weeks follow-up, there were no signs or

symptoms of intestinal obstruction such as nausea and vomiting. Patient is being fed regularly and

passes bowel movement almost 2-3 times a day. Conclusion: appropriate diagnosis of the extent of

the defect through the advent of CT scan and early detection of other congenital anomalies should be

routine in these cases. Open surgery with primary repair is almost always done but we can consider

laparoscopic approach in the future with uncomplicated lumbar hernias.

Keywords: congenital hernia, superior lumbar hernia, surgery.

LAPORAN KASUS NEONATUS DENGAN HERNIA LUMBAR SUPERIOR

KONGENITAL

Stephanus Haryanto Hokardi*, Neil Angelo S. Sael

Departmen Bedah, De Los Santos Medical Center, 201 E. Rodriguez Sr. Ave., Kota Quezon, NCR, Filipina.

*Korespondensi: [email protected].

ABSTRAK

Latar belakang: hernia lumbal kongenital jarang terjadi. Ini merupakan 20% dari semua hernia

lumbar yang mana kurang dari 1,5% dari semua hernia dinding abdomen. Tidak ada lebih dari 50

kasus yang dilaporkan dalam literatur hingga saat ini. Kami melaporkan kasus hernia lumbar

kongenital pada neonatus perempuan prematur yang terletak di segitiga lumbar superior. Kasus:

neonatus perempuan prematur lahir, terdapat massa di daerah pinggang kanan dengan ukuran 8x8cm,

bulat, dapat digerakkan dengan warna kebiru-biruan, batas baik, tidak ada vena yang terlihat,

peningkatan ukuran ketika pasien menangis, dan mengecil dengan mudah. Ultrasonografi

menunjukkan massa perut posterolateral kanan berukuran 4,2x2,88x1,59 cm. CT scan menunjukkan

hernia dinding abdomen tengah posterolateral kanan dengan protrusi dan tidak ada obstruksi usus.

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Pasien menjalani laparotomi eksplorasi. Lubang hernia sekitar 2 cm dan lubang berada di dinding

perut posterior kanan, daerah pararenal, dan tepat di bawah tulang rusuk ke-12. Kolon asendens dan

bagian ileum melekat di dalam defek hernia di area lumbar kanan. Penutupan primer defek hernia

dilakukan dengan menjahitkan psoas major dan transversus abdominis dan otot oblikus internal. Pada

pasca operasi, pasien mengalami gerakan usus yang baik, tidak ada distensi abdomen atau muntah.

Makan dimulai dan ditoleransi baik. Setelah dua minggu evaluasi, tidak ada tanda-tanda atau gejala

obstruksi usus seperti mual dan muntah. Pasien diberi makan secara teratur dan buang air besar 2-3

kali sehari. Simpulan: diagnosis yang tepat mengenai derajat defek melalui CT scan dan deteksi dini

anomali kongenital lainnya harus rutin dalam kasus ini. Operasi terbuka dengan perbaikan primer

hampir selalu dilakukan tetapi kita dapat mempertimbangkan pendekatan laparoskopi di masa depan

dengan hernia lumbar tanpa komplikasi.

Kata kunci: hernia kongenital, hernia lumbar superior, operasi.

INTRODUCTION

Congenital lumbar hernias are rare

abdominal wall hernias in infants and in

children. Approximately 10% are

congenital and the majority are unilateral.1

They are divided into three types

depending on the site. There are superior,

which occur through the superior lumbar

triangle (Grynfelt-Lesshaft triangle),

inferior, which occur through the inferior

lumbar triangle (Petit), and combined.

Most common location of a congenital

lumbar hernia is in the inferior triangle.1,2

Less than 50 cases of congenital lumbar

hernias are reported in literature till

date.2,3,4 These hernias may contain colon,

small intestine, spleen, or liver and all

have been reported.4,5 CT Scan has

become the imaging modality of choice in

confirming the diagnosis.6 Lumbar hernias

have a 25% risk of incarceration and an

8% risk of strangulation, making surgery a

reasonable option for management.5

Congenital lumbar hernias when

diagnosed, are frequently associated with

other anomalies. Lumbocostovertebral

syndrome is one example wherein there is

a presence of hemivertebra, congenital

absence of ribs, anterior mylomeningocele,

and hypoplasia of anterior abdominal wall

presenting as congenital lumbar hernia.

Other anomalies associated are anorectal

malformations, hydrocephalus, congenital

diaphragmatic hernia, caudal regression

syndrome, pelvoureteric junction

obstruction, cloacal exstrophy, an absent

kidney, or meningomyelocele. Diagnosis

of a congenital lumbar hernia must prompt

one to investigate further for these

anomalies.1,2 We report a case of

congenital lumbar hernia in a preterm

female neonate located on the superior

lumbar triangle.

CASE REPORT

A preterm female neonate was born to a

20-year-old G2P2 (1102) via Cesarean

Section at 36 weeks gestation. She

presented with a mass at the right lumbar

area with a size of 8x8 cm, round, movable

with bluish discoloration, well delineated

border, no visible veins and increases in

size when the patient cries and reduces

easily.

Ultrasonography revealed a right

posterolateral abdominal mass measuring

4.2x2.88x1.59 cm. A dorsal hernia is

considered. There were no signs of bowel

obstruction. Mild pelvocaliectasia was

noted on the right. The liver, gallbladder,

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53

pancreas, spleen, left kidney and urinary

bladder were unremarkable. There was no

hepatobiliary duct ectasia nor

demonstrable suprarenal or pelvic mass.

The scout film of the abdomen was also

done which showed displaced bowel loops

in the right lower quadrant.

CT scan (Figure 1a, 1b) revealed

minimal to moderate hepatomegaly,

normal spleen, multiple surface

calcification, sequel of an intrauterine

granulomatous infection, right

posterolateral mid-abdominal wall hernia

with protrusion, no intestinal obstruction,

small umbilical hernia. There were no

signs of obstruction, no vomiting nor

abdominal distention until on the 5th day of

life, the patient underwent exploratory

laparotomy. Patient was placed in supine

position and a transverse incision was

made on the abdominal right upper

quadrant and carried down to the

peritoneum. There was multiple mucoid

meconium noted on bowels with minute

calcifications. Meconium peritonitis was

considered due to the prolonged standing

of the congenital lumbar hernia. Bowel run

was done and noted no signs of

perforation. The bowels were noted to be

inflamed in different segments.

Adhesiolysis was done with sharp and

blunt dissection. Hernia defect was about 2

cm in diameter and is noted to be in the

right posterior abdominal wall, pararenal

area, and just below the 12th rib. This

marks the superior lumbar triangle. The

ascending colon and parts of the ileum

were adherent inside the hernia defect at

the right lumbar area. Mobilization of the

ascending colon and cecum was done then

the bowels were delivered from the hernia

defect. Primary closure of the hernia defect

was done by suturing the psoas major and

the transversus abdominis and internal

oblique muscles with 6 stitches of Prolene

3-0 simple interrupted suturing. Primary

closure was considered due to the presence

of meconium peritonitis. Interval

appendectomy was done. The rest of the

abdominal wall was closed in layers.

Patient tolerated the procedure well.

Figure 1. (a,b) CT scan imaging of the right

posterolateral abdominal wall hernia. Note only

small bowel loops are located in the hernia defect;

(c,d) pre-operative images of the congenital lumbar

hernia; (e) intraoperative image of the hernia

defect; (f) post-operative image.

The immediate postoperative period

was uneventful. On the second

postoperative day, patient had 3 episodes

of bowel movement. No abdominal

distention or vomiting was noted. Repeat

scout film of the abdomen was done which

revealed non-dilated bowel loops in the

abdominal cavity. No free air noted.

Feeding was then started and well

tolerated. The rest of the hospital stay was

unremarkable. Antibiotics were completed

and on the 16th day of life, baby was sent

home with stable vital signs, good suck,

and active.

After two weeks follow-up, there were

no signs or symptoms of intestinal

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obstruction such as nausea and vomiting.

Patient is being fed regularly and passes

bowel movement almost 2-3 times a day.

DISCUSSION

The most common site of congenital

lumbar hernias is found in the inferior

lumbar triangle or Petit triangle.7 Acquired

or traumatic lumbar hernias are more

frequently seen in the superior lumbar

triangle because it is the thinnest area in

the lateral and posterior abdominal wall.

Hernias from this location result from

direct trauma, flank incision, or an

abscess.5 In our case, the patient presented

with a congenital lumbar hernia at the

superior lumbar triangle which is rare.8

During embryologic development,

weakening of the area of the aponeuroses

of the layered abdominal muscles that

derive from somatic mesoderm, which

invades the somatopleure, may potentially

lead to lumbar hernias.5

The aim of the surgery is to reduce the

hernia sac, repair the defect and to

strengthen the weakened posterior

abdominal wall. It could be done by

simple anatomical closure, overlapping of

the aponeurosis, or use of prosthetic

meshes or laparoscopic mesh repair in

cases of uncomplicated lumbar hernias.3,6

Elective surgical repair is suggested at

any early age to prevent incarceration and

strangulation.9 Intervention should be done

before 12 months because the hernia defect

may enlarge with growth making primary

direct closure with surrounding tissue

difficult.9 Because of the rarity of

congenital lumbar hernia, appropriate

surgical procedures are still controversial.

Open repair has been performed in most

patients.1,2,5,10 and laparoscopic repair is

preserved with small uncomplicated

hernias. In the present case, the hernial

defect was large, and surrounding

abdominal wall muscles were hypoplastic.

Primary closure was also intended due to

the presence of meconium peritonitis and

mesh repair was not considered.

Aside from primary closure, muscle

grafts could be done by using a fascia lata

graft.9 Currently, laparoscopy is also being

performed in patients with congenital

lumbar hernia. It could be approached

either transabdominally or

extraperitoneally and minimal reports of

recurrence is seen.2,5,6,9,11

CONCLUSION

Congenital lumbar hernia is one of the

rare types of hernias reported in neonates;

and occasionally in older children.

Appropriate diagnosis of the extent of the

defect through the advent of CT scan and

early detection of other congenital

anomalies should be routine in these cases.

Since there is almost to none regarding

Philippine literature on the practice of

congenital lumbar hernia, we can

recommend further studies for the surgical

approaches in this anomaly. Open surgery

with primary repair is almost always done

but we can consider laparoscopic approach

in the future with uncomplicated lumbar

hernias.

REFERENCES

1. Al-Salem AH. Abdominal Wall

Hernias and Hydroceles. In: Al-Salem

AH, editor. An Illustrated Guide to

Pediatric Surgery. Switzerland:

Springer; 2014. p.15-27.

2. Esposito C, Settimi A, De Marco M, et

al. Congenital Lumbar Hernia: Two

Case Reports and a Review of the

Literature. Journal of Pediatric

Surgical Specialties. 2009;3:40-2.

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Volume 2 ● Number 2 ● Juli 2018 Case Report of Neonate with Congenital Superior

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3. Wakhlu A, Wakhlu AK. Congenital

lumbar hernia. Pediatr Surg Int.

2000;16:146-8.

4. Peláez Mata DJ, Alvarez Munoz V,

Fernandez Jimenez I, et al. Congenital

lumbar hernia. Cir Pediatr.

1998;11:126-8.

5. Stamatiou D, Skandalakis JE,

Skandalakis LJ, et al. Lumbar hernia:

surgical anatomy, embryology, and

technique of repair. Am

Surg. 2009;75:202-7.

6. Dakin GF, Kendrick ML. Challenging

Hernia Locations: Flank Hernias. In:

Jacob BP, Ramshaw B, editors. The

SAGES Manual of Hernia Repair. New

York: Springer; 2013. p. 531-40.

7. Pachani AB, et al. A Primary

Idiopathic Superior Lumbar Triangle

Hernia with Congenital Right

Scoliosis: A Rare Clinical Presentation

and Management. Int J Appl Basic

Med Res. 2011;1:60-2.

8. Tavares-de la Paz LA, Martínez-Ordaz

JL. Lumbar hernia. Case report and

literature review. Cir Cir. 2007;75:381-

4.

9. Morita K, Miyano G, Nouso H, et al.

Laparoscopic repair for a congenital

lumbar hernia with free fascia lata graft

reinforcement. J Pediatr Surg Case

Rep. 2014;2:101-3.

10. Sharma A, Pandey A, Rawat J, et al.

Congenital lumbar hernia: 20 years'

single centre experience. J Paediatr

Child Health. 2012;48:1001-3.

11. Moreno-Egea A, Baena EG, Calle MC,

et al. Controversies in the Current

Management of Lumbar Hernias. Arch

Surg. 2007:142:82-8.

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CASE REPORT

56 | Jurnal Bedah Nasional

BILATERAL GIANT FIBROADENOMA OF THE BREAST: A CASE

REPORT

Jasmine Stephanie Christian1, Putu Anda Tusta Adiputra2*, INW Steven Christian2

1General Surgery Training Programme, Faculty of Medicine Udayana University, Sanglah General Hospital,

Denpasar, Bali.

2Surgical Oncology Division, Department of Surgery, Faculty of Medicine Udayana University, Sanglah

General Hospital, Denpasar, Bali. *Correspondence: [email protected].

ABSTRACT

Background: fibroadenoma of the breast is one of the ANDI (Aberration of the Normal

Development and Involution of the Breast) groups. Benign breast disorders are common in females

younger than 30 years, but such masses are not common in juvenile or pre-menarche age groups. The

exact pathological diagnosis can be investigated after surgery. For treatment, surgical procedures are

needed to remove the lump. Case: a 30-year-old girl visited the outpatient clinic with psychological

distress due to the presence of lumps in her both breasts. The lumps have been growing bigger and

bigger in the last 3 years. On the physical examination both breasts are huge. FNAB showed the

interpretation of atypical ductal hyperplasia and excisional biopsy examination confirmed the

presence of bilateral giant fibroadenoma of breast. In this patient, the treatment was carried out in

two-staged surgery due to her initial rejection to undergo total breast lumps and tissue removal.

Several lumps still remained in the right breast which was indicated for the second surgery. The

following surgery was done to remove all of the lumps and breast tissue with reduction breast surgery.

Conclusion: conducting breast surgery on the young girl or un-married is not easy as many physical

and psychology problems should be considered. Therefore, conducting breast surgery, in this case,

should be performed wisely as the recurrence of the lumps was still possible.

Keywords: multiple giant fibroadenoma, surgery, psychology.

FIBROADENOMA RAKSASA BILATERAL PADA PAYUDARA: SEBUAH

LAPORAN KASUS

Jasmine Stephanie Christian1, Putu Anda Tusta Adiputra2*, INW Steven Christian2

1Program Studi Ilmu Bedah, Fakultas Kedokteran Universitas Udayana, Rumah Sakit Umum Pusat Sanglah,

Denpasar, Bali.

2Divisi Bedah Onkologi, Departemen Ilmu Bedah, Fakultas Kedokteran Universitas Udayana, Rumah Sakit

Umum Pusat Sanglah, Denpasar, Bali. *Korespondensi: [email protected].

ABSTRAK

Latar Belakang: fibroadenoma payudara adalah salah satu kelompok ANDI (Aberration of the

Normal Development and Involution of the Breast), yang mencakup spektrum luas dari penyakit

payudara benigna. Gangguan payudara jinak umumnya terjadi pada wanita yang kurang dari 30 tahun,

tetapi massa tersebut tidak umum ditemui pada kelompok usia remaja atau pra-menarche. Giant

fibroadenoma didefinisikan sebagai benjolan berukuran lebih dari 5 cm. Fibroadenoma dapat terjadi

pada satu atau kedua payudara dan tumbuh perlahan. Prosedur bedah diperlukan untuk mengangkat

massa. Diagnosis patologis dapat ditegakkan setelah operasi. Kasus: seorang wanita 30 tahun

mengunjungi klinik rawat jalan dengan tekanan psikologis karena adanya benjolan di kedua

payudaranya. Benjolan tumbuh semakin besar dalam 3 tahun terakhir. Pada pemeriksaan fisik,

ditemukan massa di seluruh kuadran payudara kanan dan kiri dengan berbagai ukuran. Pemeriksaan

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57

FNAB menunjukkan gambaran hiperplasia duktus atipikal, kecurigaan ke arah keganasan.

Pemeriksaan biopsi eksisi mengkonfirmasi adanya fibroadenoma raksasa bilateral pada payudara.

Pada pasien ini, dilakukan operasi dua tahap karena pasien menolak dilakukan operasi total kedua

payudara. Beberapa benjolan masih tersisa di payudara kanan dan direncanakan untuk operasi kedua.

Operasi selanjutnya dilakukan untuk mengangkat seluruh masa dan jaringan payudara. Simpulan:

tindakan operasi payudara pada wanita muda yang belum menikah bukanlah hal yang mudah karena

banyak masalah fisik dan psikologis yang harus dipertimbangkan. Oleh karena itu, melakukan operasi

payudara, dalam hal ini, harus dilakukan dengan bijaksana karena kekambuhan masih dapat terjadi.

Kata kunci: fibroadenoma raksasa multipel, operasi, psikologi.

INTRODUCTION

Giant fibroadenoma of the breast is

defined as a fibroadenoma larger than 5

cm1 which is most common find in young

women.2 Fibroadenoma mamma is the one

of the groups of ANDI (Aberration of the

Normal Development and Involution of

the Breast).3 ANDI is an all-encompassing

term that is used to describe a wide

spectrum of Benign Breast Disease.

Fibroadenomas have previously been

regarded as benign neoplasms, but should

now be considered as an aberration of

normal development.4

It may occur during the time of the

normal development of the breast. Most

normal development of both breasts are

range up to 25 years of age. The breast has

reached its major development by 20 years

of age and will usually begin to undergo

atrophic changes in the fifth decade of

life.5 Benign breast disorders are common

in females younger than 30 years, but such

masses are not common in juvenile or

premenarchal age groups. Giant

fibroadenoma may occur in one or both

breast and growing rapidly. Although most

fibroadenomas are benign, the presence of

a mass can cause considerable anxiety

because of the concern for cancer. These

benign tumors arise from the epithelium

and stroma of the terminal duct-lobular

unit. In one consecutive series of patients,

44% of fibroadenomas occurred in

postmenopausal women.6 For treatment,

surgical procedures are needed to remove

the lump. The exact pathological diagnosis

can be investigated after surgery.

CASE REPORT

A 30-year-old un-married girl came

with the psychological distress due to her

both breast growing bigger and bigger in

last 3 years as well as shown on the

picture. Family history was not relevant.

On the physical examination, both breasts

are huge and swelling with multiple lumps

varies in size and involving of the whole

quadrant of the breast. Her right breast

consists of ten lumps involving the whole

quadrant of the breast with the size of the

lumps are varies more than 5 cm. The left

breast consists of nine lumps involving of

the whole quadrant. The lumps were solid,

painless, and moveable. The skin over the

both breasts were hyperpigmented and

showed dilatation of peripheral vascular.

Normal of breast nipple and wide

pigmented areolar of the breast are also

noted. In general, patient is wellbeing. The

blood laboratory test is in normal limit.

Chest x-ray shown normal, no evidence of

lesion. Breast ultrasonography

examination is not provided as well as

mammography. There was no evidence of

both axillary lymphadenopathies.

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Jasmine Stephanie Christian Jurnal Bedah Nasional

58

Fine needle aspiration biopsy (FNAB)

examination showed an interpretation for

atypical ductal hyperplasia suspicious for

malignancy. Incisional biopsy showed the

result of bilateral fibroadenoma of breast.

There was no evidence of malignancy.

Patient underwent two-staged surgery due

to the rejection of taking the whole lumps

for may all breast tissue should be

removed. So, several lumps of the right

breast are still remained (Figure 1).

Majority of normal breast tissue were

removed during excision of the lumps.

Normal breast tissue also being removed

and the breast became flat. The surgery

was stopped because patient dan family

need to be informed. Patient was fully

distressed and rejected any kinds of

surgery later.

Figure 1. Clinical feature in the first presentation.

The patient presented with bilateral giant

fibroadenoma of breast (left) and already get an

operation excisional biopsy on her right breast

(right).

Three months later, she came to the

hospital to make another surgery because

her breast became bigger (Figure 2). The

next surgery is to remove the all the lumps

and breast tissue as well (reduction breast

surgery) in the left breast. But the several

lumps on the right breast still remain.

Figure 2. The lumps are getting bigger and worse

after 3 months and next surgery is removed the all

the lumps and breast tissue as well (reduction

breast surgery) in the left breast.

DISCUSSION

The diagnosis and treatment of giant

fibroadenoma can be a challenge for

physicians. As fibroadenoma is considered

a benign lesion without tendency to

malignant degeneration, surgical treatment

is generally not considered necessary to

treat fibroadenoma. However, because of

their size, giant fibroadenoma could result

in local problems including breast

asymmetry or deformity. It can become

cosmetic problem for the affected patient

with serious psychological effects. In fact,

patient is often extremely concerned about

potential cosmetic changes to the

appearance of the breast after surgery. In

the serious case, while some authors

recommend reduction mammaplasty with

inverted-incision technique to resect large

fibroadenomas, others recommend using a

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Volume 2 ● Number 2 ● Juli 2018 Bilateral Giant Fibroadenoma of the Breast

59

more cosmetically appealing incision.7,8

The surgical management of giant

fibroadenoma is still a matter of

controversial debate in the literature. The

necessity for excision is not disputed in

patients presenting with breast deformity

or suffering from local problems such as

venous congestion, pressure necrosis and

even occasionally ulcer.

CONCLUSION

Many problems should be considered

before performing breast surgery in un-

married young girl. Although radical

mastectomy will reduce the recurrence, the

patient’s satisfaction and psychological

problems can be surfaced.

REFERENCES

1. Hille-Betz H, Klapdor R, Henseler H,

et al. Treatment of Giant

Fibroadenoma in Young Women:

Results after Tumor Excision without

Recontructive Surgery. Geburtshilfe

Frauenheilkd. 2015;75:929-34.

2. Chang DS, McGrath MH.

Management of benign tumors of the

adolescent breast. Plast Reconstr Surg.

2007;120:13e-19e.

3. Robert E. Mansel. Management of

Breast Pain. In: Harris JR, Lippman

ME, Morrow M, et al. editor. Disease

of The Breast. 5th Edition.

Philadelphia: Wolters Kluwer Health;

2014. p.51-7.

4. Purushotham AD, Britton P, Bobrow

L. In: Borgen PI, Hill ADK, editors.

Benign Breast Disease. Texas: Landes

Bioscience; 2000. p.35-40.

5. Osborne MP, Boolbol SK. Breast

Anatomy and Development. In: Harris

JR, Lippman ME, Morrow M, et al.

editor. Disease of The Breast. 5th

Edition. Philadelphia: Wolters Kluwer

Health; 2014. p.3-14.

6. Bleicher RJ. Management of the

Palpable Breast Mass. In: Harris JR,

Lippman ME, Morrow M, et al. editor.

Disease of The Breast. 5th Edition.

Philadelphia: Wolters Kluwer Health;

2014. p.29-37.

7. Biggers BD, Lamont JP, Etufugh CN,

et al. Inframammary approach for

removal of giant juvenile

fibroadenomas. J Am Coll Surg.

2009:208:e1-4.

8. Jacob MM. Application of reduction

mammaplasty in treatment of giant

breast tumour. Br J Plast Surg.

2000;53:265-6.

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CASE REPORT

60 | Jurnal Bedah Nasional

GOITER MULTINODUL DENGAN PELEBARAN KE RETROSTERNAL:

LAPORAN KASUS

Gede Budhi Setiawan

Divisi Bedah Onkologi, Departemen Ilmu Bedah, Fakultas Kedokteran Universitas Udayana, Rumah Sakit

Umum Pusat Sanglah, Denpasar, Bali. Korespondensi: [email protected].

ABSTRAK

Latar belakang: goiter multinodul retrosternal merupakan massa tiroid yang meluas sampai

sternum hingga mengisi ruang inlet torakal. Dapat disebut retrosternal multinodul tiroid apabila massa

tiroid lebih dari separuhnya berada di dalam inlet torakal. Pembedahan merupakan pilihan utama.

Pilihan pembedahan yaitu dengan insisi collar dan sternotomi, clamshell insisi, bahkan sampai

thorakotomi. Kasus: dilaporkan kasus pasien wanita 62 tahun dengan goiter multinodul hingga

retrosternal. Hasil USG menunjukkan struma diffusa kiri dengan kista multipel kompleks pada tiroid

kanan. CT Scan didapatkan massa tiroid multipel kiri dengan gambaran nekrosis sentral dan

kalsifikasi egg shell, dan infiltrasi toraks. Dari foto toraks didapatkan massa di leher kiri yang meluas

hingga rongga toraks yang tampak mendesak trakea ke kanan. Telah dilakukan total tiroidektomi,

sternotomi, dan trakeostomi. Simpulan: Operasi pengangkatan goiter substernal dapat dilakukan

dengan pendekatan leher di sebagian besar pasien tetapi dalam beberapa kondisi, pasien melakukan

pendekatan leher dan sternotomi.

Kata kunci: goiter multinodul retrosternal, tiroidektomi, sternotomi.

MULTINODULAR GOITER WITH RETROSTERNAL EXTENSION: CASE

REPORT

Gede Budhi Setiawan

Surgical Oncology Division, Department of Surgery, Faculty of Medicine Udayana University, Sanglah General

Hospital, Denpasar, Bali. Correspondence: [email protected].

ABSTRACT

Background: retrosternal goiter refers to the thyroid mass grows along dermal sternum from the

neck to the substernal portion, descending below the thoracic inlet. The current accepted definition of

retrosternal goiter is thyroid gland with more than 50% of its mass located below the thoracic inlet.

Surgery is the treatment choice of retrosternal goiter. Nowadays, several surgical options have been

used for the surgical resection of this type of goiters, which is related to the size of the gland and the

location, such as collar neck incision or/and sternotomy, clamshell incision or even thoracotomy.

Case: we reported female 62-year-old with multinodular goiter with retrosternal extension.

Ultrasonography showed diffuse mass of left thyroid and multiple complex cyst of right thyroid. CT

Scan examination revealed multiple mass of left thyroid with central necrotic and egg shell

calcification, and infiltrated thorax. Thorax x-ray showed mass derived from left neck to intra thoracic

cavity and there is deviation of trachea to the right. Fine needle aspiration biopsy shows colloid

nodule and follicular neoplasm. We perform total thyroidectomy, sternotomy, and tracheostomy.

Conclusion: Surgical removal of substernal goiters can be performed by a cervical approach in the

majority of patients but in some condition, patient performed cervical and sternotomy approach.

Keywords: retrosternal multinodular goiter, total thyroidectomy, sternotomy.

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Volume 2 ● Number 2 ● Juli 2018 Multinodular Goiter with Retrosternal Extension

61

PENDAHULUAN

Goiter retrosternal mengacu pada massa

tiroid yang tumbuh sepanjang sternum dari

leher ke bagian substernal, turun di bawah

inlet torakal. Tiroid retrosternal mencakup

kelenjar tiroid dengan lebih dari 50% dari

massa yang terletak di bawah inlet torakal.

Pembedahan adalah pengobatan pilihan

untuk tiroid retrosternal. Saat ini, pilihan

operasi yang berbeda telah digunakan

untuk reseksi jenis goiter ini, yang

berkaitan dengan ukuran kelenjar dan

lokasinya, seperti sayatan leher collar atau

dengan sternotomi, sayatan clamshell, atau

bahkan torakotomi.1

Meskipun sebagian besar goiter

retrosternal adalah ekstensi dari leher,

goiter intra torakal murni, mungkin terjadi.

Goiter retrosternal lebih cenderung di sisi

kiri dan sangat jarang meninggalkan sisi

kiri kemudian turun ke sisi kanan toraks

yang disebut "crossed substernal goiter".

Dengan beberapa pengecualian, goiter

yang besar mungkin dioperasi dengan

pendekatan servikal tetapi sekitar kurang

dari 2% pasien membutuhkan pendekatan

servikal dan sternotomi.1 Dalam artikel ini

kami melaporkan kasus langka wanita

berusia 62 tahun yang memiliki goiter

multinodular dengan ekstensi retrosternal

yang sangat besar. Tumor diangkat melalui

pembedahan dengan pendekatan servikal

dan sternotomi.

LAPORAN KASUS

Seorang wanita 62 tahun datang ke

pusat kanker kami dengan keluhan utama

adanya massa leher yang besar sejak 20

tahun yang lalu. Pemeriksaan lokal

mengungkapkan pembengkakan kelenjar

tiroid yang difusa (Gambar 1).

Pemeriksaan radiologis dilakukan

(Gambar 2) dan ultrasonografi

menunjukkan adanya massa difusa tiroid

kiri dan kista kompleks multipel tiroid

kanan. Computed tomography (CT) scan

leher menunjukkan adanya lesi multipel

padat pada tiroid kiri berukuran 5,6 x 5,6

cm dengan peningkatan kontras dan

nekrosis sentral. Lesi lain berukuran 6,0 x

4,6 cm dengan kalsifikasi egg shell dan

peningkatan kontras. Nodul multipel kanan

dengan peningkatan kontras, diameter 1,7

cm. Massa memanjang ke inlet torakal dan

menggeser trakea ke kanan. Tidak ada

limfadenopati servikal.

Gambar 1. Gambaran klinis

Gambar 2. Pemeriksaan ultrasonografi dan CT

scan leher.

X-ray toraks (Gambar 3) menunjukkan

massa yang berasal dari leher kiri ke intra

toraks dan ada pergeseran trakea ke kanan.

Hasil biopsi aspirasi jarum halus

menunjukkan nodul koloid dan neoplasma

folikel. Uji fungsi tiroid (FT4 dan TSH)

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Gede Budhi Setiawan Jurnal Bedah Nasional

62

dilakukan, yang mana FT4 2,00 ng/dL dan

TSH 0,03 µIU/mL.

Gambar 3. X-ray toraks.

Gambar 4. Posisi kelenjar tiroid.

Pembedahan dilakukan dengan anestesi

umum dan sayatan collar dilakukan 2 cm

di atas sternum. Otot strap kemudian

dipisahkan sampai tampak kelenjar tiroid.

Kelenjar tiroid kiri dimobilisasi lebih

dahulu kemudian diikuti dengan kelenjar

tiroid kanan. Kedua saraf laringeal rekuren

diidentifikasi dan diamankan (Gambar 4).

Setelah tiroid kiri dibebaskan, dilanjutkan

dengan tiroid kanan. Sternotomi dilakukan

untuk enukleasi massa retrosternal.

Kelenjar tiroid bersama dengan massa

retrosternal dipotong (Gambar 5).

Trakeostomi dilakukan untuk menjaga

patensi saluran napas. Pada kasus ini

pasien keluar rumah sakit setelah

menjalani perawatan 21 hari.

Gambar 5. Kelenjar tiroid dan massa retrosternal

sudah dieksisi.

DISKUSI

Pembedahan adalah pengobatan pilihan

untuk goiter retrosternal dengan atau tanpa

gejala klinis. Definisi gioter retrosternal

tidak seragam dan sering bervariasi di

antara penulis. Menurut Katlic dan rekan-

rekan goiter retrosternal adalah ketika

lebih dari 50% dari massa terletak jauh ke

outlet toraks. Pasien sering mengeluh

pertumbuhan massa yang lambat dan

pertumbuhan massa progresif biasa terjadi

pada dekade kehidupan ke-5 atau ke-6.2

Goiter retrosternal dapat menyebabkan

gangguan pernapasan, disfagia, kompresi

vaskular, dan bahkan menimbulkan

kematian mendadak.3-5

Kondisi tersebut tidak jarang dan risiko

keganasan antara 3-21%. Operasi

pengangkatan goiter substernal dapat

dilakukan dengan pendekatan servikal

pada sebagian besar pasien. Telah

dilaporkan bahwa ahli bedah yang ahli,

dengan pengalaman operasi tiroid yang

baik, perlu melakukan pendekatan ekstra

servikal pada 2-5% tiroidektomi untuk

goiter substernal, tetapi beberapa penulis

telah melaporkan kejadian sternotomi

terjadi pada 29% pasien. CT scan telah

menjadi standar emas investigasi radiologi

pra operasi untuk penilaian massa tiroid

dengan ekstensi substernal dan

hubungannya dengan struktur yang

berdekatan, dan juga dapat digunakan

dalam menentukan pasien yang

kemungkinan akan memerlukan

pendekatan toraks. Beberapa orang telah

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meneliti faktor-faktor yang meningkatkan

kemungkinan sternotomi.4

Flati dkk menyatakan bahwa sternotomi

tidak dapat dihindari ketika gondok lebih

dari 70% berada di dalam mediastinum.

Beberapa penulis telah menyatakan operasi

revisi sebagai indikasi yang mungkin

untuk sternotomi.5 Sternotomi hanya boleh

digunakan ketika goiter tidak dapat

diekstraksi dari toraks dengan "manuver

lembut", juga dalam kasus semua gondok

yang berulang dan menyimpang.4 Di sisi

lain sternotomi diperlukan ketika traksi

yang berlebihan diperlukan selama

operasi, ketika perluasan yang paling

inferior dari nodul dapat dipalpasi, dalam

kasus operasi revisi, adanya kompresi

trakea akut, obstruksi vena yang parah,

keganasan, dan diagnosis preoperatif yang

tidak jelas. Banyak rekomendasi

sternotomi untuk nodul substernal ganas,

goiter mediastinum posterior dengan

ekstensi kontralateral, goiter mediastinum

dengan suplai darah dari mediastinum,

gondok yang menyebabkan sindrom vena

cava superior, kasus operasi revisi, jika

eksisi massa toraks yang sulit, perdarahan

yang signifikan, dan ketika diameter nodul

mediastinum secara signifikan melebihi

diameter inlet torakal.4

SIMPULAN

Goiter retrosternal cukup umum dan

dapat diagnosis secara klinis dan

radiologis yang jelas. Pembedahan

merupakan pilihan utama. Operasi

pengangkatan goiter substernal dapat

dilakukan dengan pendekatan servikal di

sebagian besar pasien tetapi dalam

beberapa kondisi pasien melakukan

pendekatan servikal dan sternotomi. CT

scan dapat digunakan dalam menentukan

pasien yang kemungkinan akan

membutuhkan pendekatan toraks.

DAFTAR PUSTAKA

1. Mohammad A, Rajnish N, Shirsendu

R, et al. Huge Nodular Goitre with

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