GROUP 17

SKENARIO 1: Bayi Koni yang tidak sempurna

Bayi Koni yang berusia 4 hari dibawa ibunya ke Puskesmas karena selalu
muntah setelah menyusu, kadang kadang tersedak sehingga ASI keluar ke hidung. Dari
anamnesis diketahui bahwa sejak lahir bayi tersebut mengeluarkan mekonium sedikit-
sedikit melalui fistel yang terdapat di daerah vulva. Pada pemeriksaan fisik ditemukan
palatoskisis, perut kembung, dan tidak ditemukan adanya lobang anus. Dokter menduga
bayi Koni menderita kelainan bawaan, oleh karena itu dokter merujuk ke RSUP DR. M
Djamil.
Setelah ditindak lanjuti, DPJP menjelaskan kasus bayi Koni kepada mahasiswa.
Dari diskusi yang berlangsung, preseptor mengingatkan kembali mahasiswa tentang
kelainan anatomi yang memungkinkan untuk terjadinya masalah pada bayi Koni.
Preseptor mendemonstrasikan pemeriksaan saluran cerna bayi sejak dari rongga mulut
sampai ke anus. Dari pemeriksaan ditemukan palatoskisis. Untuk mengetahui adanya
kelainan saluran cerna yang lain seperti atresia esophagus dilakukan pemeriksaan foto
rontgen leher-toraks dengan sonde terpasang. Bayi ini dipuasakan untuk menghindarkan
komplikasi aspirasi pneumonia dan diberikan intake nutrisi secara intravena.
Ibu bayi Koni tidak dapat menunggui anaknya dirumah sakit karena nyeri pada
anus, BAB berdarah dan ada benjolan pada anus.
Bagaimana anda menjelaskan kasus di atas?
Identifikasi Masalah
1. Mengapa bayi Koni muntah, tersedak, Asi keluar melalui hidung?
2. Mengapa bayi tersebut mengeluarkan mekonium sedikit demi sedikit melalui fistel
di vulva
3. Apa saja faktor penyebab palatoskisis dan atresia ani?
4. Mengapa bayi koni perutnya kembung dan tidak ditemukan adanya lubang anus?
5. Bagaimana cara pemeriksaan pada saluran cerna pada bayi?
6. Apa saja tanda tanda dari palatoskisis?
7. Mengapa bayi koni harus di puasakan dan diberi nutrisi melalui injeksi intravena?
8. Apa kemungkinan penyakit yang diderita pada ibu koni dengan keluhan nyeri pada
anus, BAB sulit dan benjolan di anus?
9. Apa saja faktor penyebab pad keadaan yang dialami ibu koni?
HIPOTESIS
1. Muntah dapat disebabkan oleh atresia esofagus (tidak terbentuknya esofagus).
Tersedak karena adanya fistula tracheoesofagus, menyebabkan asi masuk ke
trakea. Asi yang keluar bisa juga dikarenakan palatoskisis
2. Jumlah mekonium dapat dipengaruhi oleh stress pada bayi. Mekonium yang
dikeluarkan melalui saluran yang tidak semestinya dapat menyebabkan infeksi
3. Palatoskisis dapat disebabkan oleh obat obat teratogenik yang dikonsumsi selama
masa kehamilan, mutasi gen, dan gangguan pertumbuhan. Sedangkan atresia ani
dikarenakan gangguan pertumbuhan
4. Perut kembung disebabkam oleh banyaknya udara yang masuk pada saat menyusui
karena kesalahan posisi, intoleransi glukosa, sumbatan pada usus bayi.
5. Mulut : mendeteksi palatoskisis
Esofagus : atresia esofagus (tersedak)
Gaster : kateter, monometrik
Duodenum : muntah hijau, buncit, dehidrasi
Anus : memasukkan ujung termometer yang tumpul ke anus
Secara umum bisa melalui anamnesis dan pemeriksaan fisik. Dilanjutkan dengan
pemeriksaan penunjang seperti endoskopi, sigmoidoskopi, kolonoskopi
6.Gejala dan tanda : celah pada palatum, rongga pada bagian hidung, yang menyebabkan
distorsi hidung. Gangguan pernafasan, gangguan berbicara, aspirasi, regurgitasi makanan.
7. Palatoskisis : nutrisi IV
8. Mengalami hemoroid, pelebaran vena hemoradialis, yang dapat terjadi prolaps atau
bengkak
9. Faktor hemoroid : obstruksi vena, mengendan saat bab, posisi bab yang salah, prolaps,
herediter
 CONGENITAL
GASTROINTESTINAL
DISORDER
1. Labioskisis / palatoskisis

• Related with embriology, function and genetic hypoplation of layer mesenkim

playing, not coming in medial section
• Incidence 1 of 75o birth, 1 of 2500 birth (palatum case split) cause: not clear,
• Shared: there is religious with consumption of drugs in pregnancy, complex
syndrome malformation, genetic factor risk 50%
• Increasing boys, asian people, black leather, and if any other difference between
2. ESOPHAGEAL ATRESIA (EA)

• Design of esofagus most frequently discovered,

• Incident 1 in 4000 neonatus 90% related to
trakeoesofagus fistel (fte)
• Early diagnosis and treatment and right
treatment health numbers> 90%
• High death rate if bb <1500 gr
3. HYPERTROPHIC PYLORIC STENOSIS

• Happening of piloros (stenosis) piloros due to

hipertrofi muscle
• Incidence 3 in 1000 baby new birth (united states),
• In indonesia not exists data boy 4x> from woman
• Genetical
• > many on blood type b and o
• Related to other damages like other
trakheoesofagus fistels
4. DUODENAL ATRESIA

• FAILURE FOR ESTABLISHING LUMENS ON WEEK TO 4-

5 MASTERS OF GESTATION
• INCIDENT 1 ON 10,000 BIRTH (25-40% OF ALL
CURRENT CHAIN ATRESIA), 50% PREMATURE BIRTH
 5. HIRSCHSPRUNG DESEASE

• Caused big user interventory inervation

information from spingter anus expand to
proksimal
• No ganglion cell on the wall of the usus
• Incident 1 in 5000 live births
• > boys : girls (4: 1) right on premature baby
• There are genetic controls: the segment long
 6. ATRESIA ANI

• There are 4 molds:

* lower raise stenosis
* making anus establishment
* anus inferforata
* Anus sections are separated with an install points
7. OMFALOKEL

• Caused failure of abdomen rongga tools to return

into rongga abdomen fetal time getting 10 weeks
clinics of protrusi clinics from containers with usus
and visera
• Abdomen through the defect of wall abdomen on
umbilikus
• High death rate when are a great omfalokel.
8. HERNIA INGUINALIS /SKROTALIS

• Insert order abdomen into in the inguinal room even to the

scrotal card (hernia scrotalis)
• Happened by the vaginalist processes that stayed patent
almost 50% happen in the first year of life baby
• Risk factors, prematurity, development of urogenital
channels
• Conditions of relationships related to improve intra
abdomen pressure asites, chronic disease diseases.
• Complications can inkarserata, infark gonad
 ACQUIRED DISORDER OF
GASTROINTESTINAL TRACT
 GASTROESOPHAGEAL
REFLUX DISEASE (GERD)
Is a condition when stomach acid backs up into esophagus. We may feel a burning
pain in the middle of your chest. It often occurs after meals or at night.
While it’s common for people to experience acid reflux and heartburn once in a
while, having symptoms that affect your daily life or occur at least twice each week
could be a sign of GERD.

Gastritis
Gastritis is an inflammation, irritation, or erosion of the lining of the stomach. It can
occur suddenly (acute) or gradually (chronic).
 PEPTIC ULCER
Peptic ulcers are open sores that develop on the inside lining of your stomach and
the upper portion of your small intestine. The most common symptom of a peptic
ulcer is stomach pain.

Peptic ulcers include:

• Gastric ulcers that occur on the inside of the
stomach
• Duodenal ulcers that occur on the inside of the
upper portion of your small intestine (duodenum)
 IRRITABLE BOWEL SYNDROME

Irritable bowel syndrome (also called spastic colon, irritable colon, or nervous
stomach) is a condition in which the colon muscle contracts more often than in
people without IBS. Certain foods, medicines, and emotional stress are some
factors that can trigger IBS.

Crohn’s Disease
Crohn’s disease is part of a group of digestive conditions called inflammatory
bowel disease (IBD). Crohn’s most commonly affects the terminal ileum, which
connects the end of the small bowel and the beginning of the colon, but it can affect
any part of the digestive tract.
 CELIAC DISEASE

Celiac disease is a serious sensitivity to gluten, which is a

protein found in wheat, rye, and barley. Eat gluten, and your
immune system goes on the attack: It damages your villi, the
finger-like protrusions in your small intestines that help you
absorb nutrients from the foods you eat. Symptoms of celiac
disease in children include abdominal pain and bloating,
diarrhea, constipation, vomiting, and weight loss. Symptoms
in adults can also include anemia, fatigue, bone
loss, depression, and seizures.
 COLITIS
There are several types of colitis, conditions that cause an inflammation of the bowel.
These include:
• Infectious colitis
• Ulcerative colitis (cause not known)
• Crohn's disease (cause not known)
• Ischemic colitis (caused by not enough blood going to the colon)
• Radiation colitis (after radiotherapy)

Colitis causes diarrhea, rectal bleeding, abdominal cramps, and

urgency (frequent and immediate need to empty the bowels).
Treatment depends on the diagnosis, which is made by
colonoscopy and biopsy.
 HEMORRHOIDS
Hemorrhoids are swollen blood vessels that line the anal opening.
They are caused by chronic excess pressure from straining during a
bowel movement, persistent diarrhea, or pregnancy.
There are two types of hemorrhoids: internal and external.
• Internal hemorrhoids
Internal hemorrhoids are blood vessels on the inside of the anal
opening. When they fall down into the anus as a result of
straining, they become irritated and start to bleed. Ultimately,
internal hemorrhoids can fall down enough to prolapse (sink or
stick) out of the anus.
• External hemorrhoids
External hemorrhoids are veins that lie just under the skin on
the outside of the anus. Sometimes, after straining, the external
hemorrhoidal veins burst and a blood clot forms under the
skin. This very painful condition is called a pile.
 DIVERTICULAR DISEASE
Diverticulosis is the presence of small outpouchings (diverticula) in the muscular
wall of the large intestine that form in weakened areas of the bowel. They usually
occur in the sigmoid colon, the high-pressure area of the lower large intestine.
Diverticulosis rarely causes symptoms. They include infection or inflammation
(diverticulitis), bleeding, and obstruction.
EPIDEMIOLOGY
Labioschisis or commonly called harelip lips is a congenital defect that becomes
a problem in the community, especially people with weak socioeconomic status. As
a result the surgery is done late and even left to adulthood.
Cleft lip with or without a gap in the ceiling, is the most common congenital
abnormality of the head and neck in the world. Epidemiological studies for the
prevention of cleft lip is still small but surgical techniques to treat it a lot done.
Cleft has different frequencies across cultures and races and countries. It is
estimated that 45% of the population is non-Caucasian. Fogh Andersen in Denmark
reported cases of cleft lip and cleft palate 1.47 / 1000 live births. Similar results
were also reported by Woolf and Broadbent in the United States

Hypertrophy pyloric stenosis (HPS) is the most gastrointestinal disorder

often in young infants. The incidence is 1-2: 1000 live births. This condition is
common occurs in infants aged 2-10 weeks of life.
Epidemiology

• Atresia Ephophagus is a common congenital disorder.

• The most commonly encountered form is the proximal portion of the
esophagus having the tip of a dead end bag, while the distal portion is
associated with the trachea through a narrow channel at the point just above
the branching.
• The incidence rate ranges from 1 in 2500 to 3000 live births.
• Occur 2-3 times more in multiple pregnancies.
• 50% of esophageal atresia is often accompanied by other congenital
abnormalities, such as: cardiac abnormalities, gastrointestinal disorders
(duodeni atresia, atresia ani), bone disorders (hemivertebra).

• The incidence of duodenal atresia in the United States is 1 per 6000

births. Intrinsic congenital duodenal obstruction is 2/3 of the total congenital
duodenal obstruction (duodenal atresia 40-60%, duodenal web 35-45%, 10-
30% anular pancreas, duodenal stenosis 7-20%). The incidence of congenital
obstruction in Finland (intrinsic, extrinsic, and mixed) is 1 per 3400 live
births. There is no racial and gender predilection in this disease.
Epidemiology
anorectal
The average incidence rate of

malformations worldwide is 1 in 5000 Birth. In general,

anorectal malformations are more common in men than women. Fistula
rektouretra is the most common abnormalities in infant males, followed by
perineal fistula. Whereas in female infants, the type of anorectal malformations
the most common is imperforate anus followed by rektovestibular fistula and
fistulas perineal. The results of Boocock and Donna's research in Manchester
show that malformations low-lying anorectals are more common than high-
altitude anorectal malformations.

Hirschsprung's disease usually occurs in the ratio of 1

out of every 5000 babies born. Opinions and other sources also say that this
disease occurs in the ratio of 1 from 1500 to 7000 newborns. This disease is 5
times more likely to occur in a baby boy and sometimes occurs with other
congenital diseases such as Down Syndrome.
Epidemiology

The incidence of adult intussusception (invagination) is very rare,

according to a reported rate of 0.08% of all abdominal surgery and 3% of intestinal obstruction, another
reported 1% of all intestinal obstruction cases, 5% of all cases invaginations (children and adults),
while the figures depicting the number of events by sex and age have not been reported, whereas the
281 patients reported in the intestinal segment reported by Anderson 281 patients in the small intestine
(Jejunum, Ileum) 7 ileocolica patients, 12 patients cecocolica and 36 colocolica of 336 cases that he
reported. Desai in 667 patients described 53% of the duodenum, jejunum or ileum, 14% lead point on
ileoseccal, 16% colon and 5% including appendic veriformis.

Almost 70% of cases of invagination occur in children younger than 1 year (Bisset et al., 1988) while
Orloff gets 69% of 1814 cases in infants and children younger than 1 year (Cohn 1976). Chairl Ismail
1988 found the highest incidence achieved in children aged between 4 to 9 months. The comparison
between men and women is 2: 1 (Kartono, 1986; Cohn 1976; Chairul Ismail! 988).

The highest incidence of inttususepsiter can be under 2 years (Ellis 1990). Orloof gets 69% of the 1814
cases in children occurring at age less than 1 year (Cohn 1976). In infants and children intussusception
is the cause of approximately 80-90% of cases of obstruction. In adults intussusception is less common
and is thought to account for about 5% of cases of obstruction (Ellis, 1990)
Epidemiology

Patients with the most common hemorrhoids in whites, from high

economic status, and from rural areas. The risk of hemorrhoids increases with age, and
peaks at the age of 45-65 yearsIn the United States, one-third of the 10 million
population suffer from hemorrhoidsThe prevalence of hemorrhoids based on the
National Center of Health Statistics in the United States is about 4.4%In the UK, the
incidence of hemorrhoids occurs in 13% -36% of the population Hemorrhoid in Egypt is
the most common anal disease area with a prevalence of about 50% of proctological
visits in the Colorectal UnitThe prevalence of hemorrhoids in Indonesia is 4% or about
ten million people

The incidence of fissura ani is 1 in 350 people. The frequency of occurrence

of fissure ani is similar between men and women. Fissura ani more likely to occur at a
younger age and middle age
Epidemiology
Hernias in the groyne area account for 75% of all hernia. The inguinal hernia is
divided into indirect and direct inguinal hernias. In indirek inguinal hernia, the hernia
sac enters the internal inguinal annulus and through the external inguinal annulus to the
scrotum. While in the inguinal hernia direk, the hernia sac is located medial of the
internal inguinal annulus. Two-thirds of cases of inguinal hernia are indirect types.
Indirect and direct inguinal hernias can also occur together. Men have 25 times more
risk than women to have inguinal hernias. Indirek inguinal hernia is more common on
the right because a decrease in the right side of the testis occurs more slowly followed
by atrophy of the processus vaginalis.

Rectal prolapse is rarely found even rarely discussed, but the actual number of cases is
unknown because it is rarely reported especially when it occurs in remote areas. More
common in adults and babies. Complete prolapse or rectal prolapse in adults usually
occurs in women. In infants, rectal prolapse usually occurs in infants under three years,
especially in the first year of life
DIAGNOSIS
• Esophageal Atresia
Prenatally, ultrasonographic findings may suggest a diagnosis of isolated esophageal
atresia (EA); however, prenatal EA detection rates are low, and if suspected, the
diagnosis must be confirmed postnatally.
Sonographic evaluation will reveal the first findings suggestive of a congenital anomaly,
but it is not conclusive. Many conditions involve polyhydramnios and a small or absent
stomach bubble at ultrasonography. Visualization of a dilated proximal pouch is
suggestive of esophageal atresia (EA) , but further tests are necessary to confirm the
diagnosis. In addition, sonograms may not give any indication of EA when it is present,
and often, a fistula is not seen.
• Duodenal atresia
Diagnosis of duodenal atresia can be suspected prenatally if there is polyhydramnios,
dilated bowel, ascites, or a combination.
Hirschsprug disease
Diagnosis
Barium enema
Rectal biopsy
Diagnosis of Hirschsprung disease should be made as soon as possible. The longer the
disease goes untreated, the greater the chance of developing Hirschsprung
enterocolitis (toxic megacolon), which may be fulminant and fatal. Most patients can be
diagnosed in early infancy.
Initial approach is typically with barium enema and/or rectal suction biopsy. Barium
enema may show a transition in diameter between the dilated, normally innervated
colon proximal to the narrowed distal segment (which lacks normal innervation).
Barium enema should be done without prior preparation, which can dilate the
abnormal segment, rendering the test nondiagnostic. Because characteristic findings
may not be present in the neonatal period, a 24-h postevacuation x-ray should be
taken; if the colon is still filled with barium, Hirschsprung disease is likely. A rectal
suction biopsy can disclose the absence of ganglion cells. Acetylcholinesterase staining
can be done to highlight the enlarged nerve trunks. Some centers also can do rectal
manometrics, which can reveal lack of relaxation of the internal anal sphincter that is
characteristic of the abnormal innervation.
Hemorrhoids
Diagnosis
• A visual examination of your anus may be enough to diagnose hemorrhoids. To
confirm the diagnosis, do a different examination to check for any abnormalities
within the anus. This check is known as a digital rectal exam . During this exam, your
doctor inserts a gloved and lubricated finger into your rectum. If they feel anything
abnormal, they may order an additional test called a sigmoidoscopy
• A sigmoidoscopy involves your doctor using a small camera to diagnose an internal
hemorrhoid. This small fiber-optic camera, called a sigmoidoscope, fits into a small
tube and then inserts into your rectum. From this test, your doctor gets a clear view
of the inside of your rectum so that they can examine the hemorrhoid up close.
MEDICAL TREATMENT
 ESOPHAGEAL ATRESIA

• Measures should be taken to reduce the risk of

aspiration(continuous suctioning of the upper
pouch, the infant's head should be elevated).
• In infants with respiratory failure, endotracheal
intubation should be performed.
• Transfer to a major tertiary pediatric institution
is best not delayed
 SUMMARY OF
PREOPERATIVE
INVESTIGATIONS

• A plain radiograph
• Renal ultrasonography and echocardiography
are routine preoperative investigations
• Endoscopy or a careful midesophageal contrast
study performed in a tertiary center. In some
centers, bronchoscopy is performed routinely in
all infants with esophageal atresia.
 OPERATIVE REPAIR OF
ESOPHAGEAL ATRESIA

• Surgical repair is delayed (1-2days) in infants with low

birth weight, pneumonia or other major anomalies.
Tracheoesophageal fistula without atresia
(type E).

• Respiratory difficulty after

feedings in a 3-day-old boy.
Barium esophagogram
clearly shows an H-shaped
fistula between the trachea
and the middle segment of
the esophagus (arrowhead).
Barium is filling the bronchi
of the right lower lobe
(arrows).
TRACHEOESOPHAGEAL FISTULA
WITHOUT ATRESIA (TYPE E).

• Esophagogram shows a
fistula (arrow) arising
from the anterior
portion of the
esophagus (e) and
passing cephalad to the
posterior portion of the
trachea (t).
 CLEFT LIP AND PALATE (CLP)
• Before the operation: prepared baby body durability is receive action
operation, sufficient nutrition intake as seen from the balance of weight, and
age. The benchmark used is the usual “rule of ten”

RULE OF TEN :
1. Weight loss at min 10 pounds (4.5 kg)
2. Age at least 10 weeks
3. the levels of Hb > 10gr%
4. number of leukocytes/mm ³ 10,000 <
Besides cleft lip should be glued together using a special plaster is non-alergik to
prevent cleft lip be not far away due to the process of growing flower.If this
occurs the action of correction at the time of surgery will be difficult and the
final result obtained cosmetics not perfect. The plaster non-alergenik last has to
remain bonded to operating time arrived.
LABIOSCHIZIS
• Labioplasty for unilateral labioschizis
• Millard rotation

PALATOSCHIZIS
• Palatoplasty

LABIOPALATOSCHIZIS
• Cheiloraphy
• palatoraphy
 Hemorrhoid
Symptoms of hemorrhoids and discomfort can be removed with good personal
hygiene and avoid pushing during defecation. A diet high in fiber contains fruit is
probably the only one action required; when this action fails, the laxative that
functioning to absorb water as it passes through the gut may help. Soak sitting with
ointment and suppositories containing anesthesia, astrigen (witch hazel) and bed
rest is a possible action reduced enlargement.

There are various types of nonoperative measures for hemorrhoids. Infrared

photocoagulation, bipolar diathermy, and laser therapy are techniques used to
attach the mucosa to the muscle underlying. Injection of sclerosan solution is also
effective for small and bleeding hemorrhoids.
Hemorrhoidectomy or surgical excision may be performed to remove all remaining
tissue that still exists.
 ESOPHAGEAL STRICTURES
Management of benign esophageal
1) Primary therapy is the dilation. There are 2 types of dilators:
• Mechanical dilators
• Balloon dilators/TTs (through the scope)
balloon dilators entered with a guide wire through the endoscope.
After the balloon reaches the strictures, the balloon was developed and
allowed a maximum of 3 minutes. Complications that can occur due to dilation
of the esophagus: perforation, bleeding, and bakteremia.
2) Stents
use of stents as a palliative against the complaint of dysphagia and to prevent
rekurensi strictures
3) Esophageal resection : esofagectomy and esofagogastrectomy
an act of surgical removal of the esophagus with lymph gland. sometimes
accompanied by the adoption of part of the stomach
4) Esophageal replacement
• Management of maligna esophageal
the main therapy was surgical. but there are several other actions
that can be performed such as installation of stents, laser therapy, and
brakiterapi.
 HIRSCHSPRUNG DISEASE

Healing hirschsprung's disease can only be achieved by

surgery in the form of intestinal segment aganglion
Rapture, followed by intestinal continuity returns.
Surgical procedures at hirschsprung's disease is
temporary and definitive surgery.
a) Temporary surgery
the Act of creation with colostomy decompression in
normal colonic berganglion. This action removes the
obstruction of the intestines and prevents the meconium
plugs. colostomy is not done when decompressing Medic
successfully

b) Definitive surgery
1. Procedure duhamel modification
2. Procedure swenson
3. Procedure soave
4. Procedure rehbein
5. Definitive surgical procedures through laparoscopy
 DUODENAL ATRESIA

• A tube is placed to decompress the stomach.

Dehydration and electrolyte abnormalities are
corrected by providing fluids through an
intravenous tube. An evaluation for other
congenital anomalies should be performed.
Surgery to correct the duodenal blockage is
necessary, but is not an emergency. Surgical
treatment for duodenal obstruction includes
some different techniques for the intrinsic
causes, including duodeno-duodenostomy and
duodeno-jejunostomy.
 RECTAL PROLAPSE

• Non-medical

At the beginning, when prolapse is still small, the patient

is given a fibrous diet for facilitate defecation.
Sometimes it is recommended pelvic floor exercises.
Patient instructed to stimulate bowel movements in the
morning and avoid the urge to relieve the rest of the
day because of the fullness they feel is actually the
intussusception of the proximal to the distal rectum.
With time, the urge to defecate will decrease as well as
with intussusception
• Surgery

When prolapse gets bigger and more difficult to reposition, effect

the existence of udem, so that more and more and can not be
entered again because of the stimulation and the mucus dam and
the discharge of blood. Where is sphincter ani becomes loose and
hypotonic resulting in alvi incontinence, handling Rectal prolapse
is performed surgically.