Manifestasi Penyakit Sistemik pada Rongga Mulut

Harrison’s GEH chapter 2

Atresia Esofagus

Epidemiologi Kelainan kongenital esophagus paling banyak,

Etiologi
Patogenesis & Patofisiologi
prevalensi 1,7 per 10000 kelahiran, >90% TEF
Defek pemisahan mesenkima antara esofaus dan
trachea, bukan kelainan genetik
The tracheoesophageal septum deviates too far
dorsally, causing the esophagus to end as a closed
tube.

Manifestasi klinis VACTERL (Vertebral anomalies, Anal atresia, Cardiac

anomalies, TEF, Renal anomalies, Limb anomalies)
Diagnosis Anamnesis Mengalami polyhydramnios
Pemeriksaan Mengalami respiratory distress, tachypnea, coughing,
Fisik choking, drooling, mucous and saliva bubbling from
nose and mouth
Pemeriksaan Gagalnya pemasangan pipa nasogastric atau
Penunjang orogastrik
CT scan diperoleh gambaran coiled feeding tube in
esophageal pouch dan/atau air-distended stomach
Tatalaksana Farmakologi Pemberian antibiotic untuk mencegah pneumonia
Non farmakologi Surgical ligation of the TEF and primary end-to-end
anastomosis of the esophagus via right-sided
thoracotomy

Pencegahan & Prognosis The surgically reconstructed esophagus is not normal

and is prone to poor motility, GER, anastomotic
stricture, recurrent fistula, and leakage. The trachea
also is malformed; tracheomalacia and wheezing are
common.
Congenital Diaphragmatic Hernia (Bochdalek)
Epidemiologi Insidensi antara 1/2000 dan 1/5000; 90% pada kasus
hernia masa newborn; female = 2x male; Defek pada
sebelah kiri 85% bilateral <5%; sporadic
Etiologi Largely unknown, but associated malformation.
Patogenesis & Patofisiologi Defek diafragma posterolateral mengakibatkan
viscera abdomen memasuki rongga toraks. The orifice
in diaphragm is caused by delayed or disturbed
separation of thoracic and abdominal compartments
of the body by closure of embryonic pleuroperitoneal
canals effected by growth of the post-hepatic
mesenchymal plate and of the pleuroperitoneal folds.

Manifestasi klinis Hipoplasia paru, mediastinal shift, respiratory distress

Diagnosis Anamnesis Mengalami polyhydramnios
Pemeriksaan Tachypnea, cyanosis, scaphoid abdomen, peningkatan
Fisik diameter toraks
Pemeriksaan Findings on ultrasonography may include polyhy-
dramnios, chest mass, mediastinal shift, gastric bubble or
Penunjang
a liver in the thoracic cavity, and fetal hydrops

Tatalaksana Farmakologi
Non farmakologi
Conventional mechanical ventilation, HFOV, and ECMO
are the 3 main strategies to support respiratory failure in
the newborn with CDH, prolonged mask ventilation must
be avoided.

The ideal time to repair the diaphragmatic defect is under

debate

Pencegahan & Prognosis Penderita yang survive mengalami gangguan

pernapasan, GERD, delayed growth, defek
neurokognitif

Hiatal Hernia (Sliding and Paraesophageal Hernia)

Epidemiologi Insidensi bertambah seiring bertambahnya usia

Etiologi Hiatal hernia terjadi adanya peningkatan tekanan
intra abdomen seperti obesitas, kehamilan
Patogenesis & Patofisiologi Herniation of the stomach through the esophageal
hiatus can occur as a common sliding hernia (type 1),
in which the gastroesophageal junc- tion slides into
the thorax, or it can be paraesophageal (type 2), in
which a portion of the stomach (usually the fundus) is
insinuated next to the esophagus inside the
gastroesophageal junction in the hiatus

Manifestasi klinis Sliding: GERD, acid reflux, regurgitation, epigastric

pain, dysphagia, odynophagia, nausea, bloating,
belching
Paraesophageal: gastric volvulus, regurgitation,
heartburn, dysphagia, chest pain, pulmonary problem,
nausea, hematemesis, iron-deficiency anemia
Diagnosis Anamnesis Fullness after eating and upper abdominal pain

Pemeriksaan
Fisik
Pemeriksaan Classic 24-hour pH test, impedance test, Bravo
Penunjang technique
CT: air-fluidd behind the heart
Tatalaksana Farmakologi Sliding: PPI
Paraesophageal: H2 blocker + PPI
Non farmakologi Surgical, laparoscopy

Pencegahan & Prognosis

Pyloric Stenosis

Epidemiologi 20% male and 10% female dengan ibu yang

mengalami pyloric stenosis
Etiologi Unknown; adanya korelasi penggunaan eritromisin
pada kehamilan usia 2 minggu pertama; kemungkinan
defisiensi NO sintetase

Patogenesis & Patofisiologi

Manifestasi klinis Anak: vomiting, dehidrasi, penurunan berat badan,

asidosis metabolik
Dewasa: nausea, vomiting, satiety, espigastric pain
 after eating, lose weight
Diagnosis Anamnesis

Pemeriksaan Large, dilated stomach

Fisik Anak: classing ‘olive’ might be felt in pyloric area,
strong peristaltic movements observed on inspection
Pemeriksaan Barium: narrowed pylorus
Penunjang USG: classic-e-mm sonolucent “doughnout”
Tatalaksana Farmakologi Anticholinergic therapy + very soft food
Non farmakologi IV fluid and electrolite resuscitation, followed by
Ramstedt pyloromyotomy

Pencegahan & Prognosis Prognosis excellent