Duodenal Atresia

Renny Anggraini 0708151236 Supervisor: dr. Dewi Robinar, SpA (K)

Definition
Duodenal atresia is a condition in which the first part of the small bowel (the duodenum) has not developed properly. It is not open and cannot allow the passage of stomach contents.

Embriology

Duodenal atresia is seen in more than 1 in 2500-5000 live births. Approximately 25 -40% of infants with duodenal atresia have Down syndrome. There is no racial and gender predilection on this disease. Duodenal atresia is often associated with other birth defects.

Epidemiology

Classification Type I

Type II

Type III

Pathogenesis

Genetic

No specific teratogen or genetic factor identified as a causal agent of either theoretic mechanism of development. It is certainly conceivable that teratogens or genetic factors could account for an error in the process of recanalization of the intestine or an error of vascular patency or development. Clinical reports have suggested a genetic cause of congenital duodenal atresia with an autosomal recessive inheritance. There also is an association with Downs Syndrome

Gahukamble reported that small intestinal atresia/stenosis generally run in families. This defect has also been connected with deletions of chromosome 22q11 and chromosome 12q24.3 Kannard et al and Fairbanks et al deletion of Fgfr2b and Fgf10

Clinical manifestation

Upper abdominal swelling Early vomiting of large amounts, which may be greenish (containing bile)

Continued vomiting even when infant has not been fed for several hours

Absent bowel movements after first few meconium stools

Plain abdominal X-Rays classic doublebubble sign dilated stomach & duodenal bulb w/ intraluminal air & fluid, no distal air pattern Intestinal gas beyond duodenum incomplete obstruction

Work Up

Gastric decompression (NGT) + correction of fluid & electrolyte USG of the head & urinary tract other anomalies Echocardiography cardiac malformation Genetic consultation for chromosomal analysis trisomy / Down syndr? After resuscitation operative correction

Management

IDENTITY Name: By. M Age : 11 hours Gender : Boy Entry date : 26 August 2013 Medical Record Number : 82-34-74

Case Presentation

ALLOANAMNESIS Given by the patients parents Chief Complaint Greenish vomite

Anamnesis

A 11 hours old baby boy, referred to general hospital from clinical midwife with diagnosis suspect hicsprung disease. Patient was born on August 26th 2013 in clinical midwife in Pekanbaru at 04.45 WIB by spontan

Recent Illness History

Aterm babies, cries spontaneusly, good muscle tone, skin colour red, clear amniotic fluid, history of resuscitation until stimulation, APGAR score 6/8, congenital anomaly (-), delivery trauma (), eye care (+), Neo K inj (+). Patients were taken to join the mother cared for

Recent illness history

One hour after birth, the baby vomite little greenish, by the baby's family (his grandmother), the vomit just being wiped and then his grandmother give formula milk to the patient. -Nine hours after birth the patient vomiting green severely, proyectile vomit (+) and each time when given milk, the baby always vomited. The patient's abdomen looks bloated. Abdominal bloating becomes flat again after vomiting.

Recent Illness History

Twenty four hours after birth, patient was defecate, stool : normal color, no mucus, no blood.

Recent Illness History

G3P2A0H2 mothers age 42 years old, gestation time 36-37 week Hipertension (-), DM(-), Fluor albus (-), fever Alcohol (-), smoking (-), Medication/ drugs(-) Truma (-), get radiation (-) Regular ANC to clinical midwife.

Pregnacy and birth history

DM (-), hipertension (-), same complain (-), anomaly congenital (-)

Family's disease history Nobody have same complain Family history of atresia duodenum (-)

Mother Illness History

General condition: good muscle tone, skin colour redness , warm extremity Awareness: alert Vital signs T : 35,9 C HR :126 x /min RR : 49 x / min

Physical examination:

Develpmental history:
Born weight Instalation weight lenght Head circumference Upper arm circumference Abdominal circumference Chest circumference : 3100 gr : 2955 gr : 49 cm :31 cm :11 cm : 32 cm : 31,5 cm

Eyes : pupil: isochor +/+, light reflex+/+ Head : simmetric, normal, low set ear (-), mongoloid face (+) CNS : Awareness alert, good muscle tone, seizure (-) Cardiovascular system: HR 126x/i, 1st and 2nd heart sound normal, pathological sound (-) Respiration system: RR 49x/i, shortness of breath (-), retraction (-), cyanosis (-), grunting (-), DS=0

Gastrointestinal system: upper abdominal distended (+), skin colour redness, Abdominal circumference 32cm, normal abdominal sound, anus (+), enlarge organs (-) Genital system : normal, congenital anomaly (-) Extremity : simetrical, warm, CRT <2, jaundiced (-), single transverse palmar crease (+) Delivery trauma (-)

Routine blood examination:

Hb Ht Leu Plt : : : : 14,9/l 46,3% 12. 600/l 168. 000/l

Electrolyt:
Na+ K+ : 147 mmol/L : 3,2 mmol/L

Laboratory Examination

GDS CT BT

: 81 mg/dl : 415 : 215

Abdominal X-Ray

with duodenal atresia + Down syndrome + hypothermia

Aterm neonate SMK BBLC (2775 gram)

Treatment: Keep warm NPO Decompretion OGT

IVFD 100cc/24 hours { Aminosteril (30) & N5+KCl (70)} Meropenem 2x75 mg Amikasin 2x20 mg Consul to specialist pediatric surgery for operation

Working Diagnosis

Surgeon Children consul replied: Prepare for electif operation

August 30th 2013

Duodenoduodenostomy surgery performed on patients.

August 31th 2013 Physical examination :

General condition : mild sick Vital sign : Temperature: 36,9 C HR: 138 x / minute RR: 48 x / minute Abdomen examination :
Inspection: Looks a little convex, stoma (+) redness, pus (-), stool a yellowish color, blood (-) Palpation: supple, tenderness (-), muscular defans (-) Auscultation: BU (+) normal

Follow up

September 1th 2013 Physical examination :

General condition : mild sick Vital sign : Temperature: 37,5 C HR: 132 x / minute RR: 42 x / minute Abdomen examination :
Inspection: Looks a little convex, stoma (+) redness, pus (-), stool a yellowish color, blood (-) Palpation: supple, tender (-), muscular defans (-) Auscultation: BU (+) normal

September 2th 2013 Physical examination :

General condition : mild sick Vital sign : Temperature: 37,5 C HR: 132 x / minute RR: 42 x / minute Abdomen examination :
Inspection: Looks a little convex, stoma (+) redness, pus (-), stool a yellowish color, blood (-) Palpation: supple, tender (-), muscular defans (-) Auscultation: BU (+) normal Cultur I : Sterile

September 6th 2013 Physical examination :

General condition : mild sick Vital sign : Temperature: 37,5 C HR: 132 x / minute RR: 42 x / minute Abdomen examination :
Inspection: Looks a little convex, stoma (+) redness, pus (-), stool a yellowish color, blood (-) Palpation: supple, tender (-), muscular defans (-) Auscultation: BU (+) normal Cultur I : Basillus Suspect contamination

September 10th 2013 Physical examination :

General condition : mild sick Vital sign : Temperature: 37,5 C HR: 132 x / minute RR: 42 x / minute Abdomen examination :
Inspection: Looks a little convex, stoma (+) redness, pus (-), stool a yellowish color, blood (-) Palpation: supple, tender (-), muscular defans (-) Auscultation: BU (+) normal

Echo (+) : hasil ASD

Pasien boleh pulang

A 11 hours old baby boy from clinical midwife diagnosis suspect hicsprung disease Patient was born in clinical midwife on Pekanbaru

Discussion:

RSUD AA
History
Baby always have greenish vomite when feeds milk, abdomen looks enlarged , bloating Down syndrome Upper abdominal distention (+)

Physical examination

Duodenal atresia

Abdominal X-Ray

Double buble sign

Duodenal atresia

Stabilization

Duodeno-duodenostomy

In these patients not found postoperative complications, no signs of bowel leakage that is characterized by peritonitis Surgical wound in patients also found no signs of infection.

Postoperative development

In these patients nutrition, growth and development and immunization must be attention posyandu

Growth and development

Duodenal atresia autosomal recessive

Genetic counseling