Gastroschisis Dan Omphalocele Lnkppt.ppt 4711j

OMPHALOCELE AND GASTROSHISIS

PEMBIMBING Dr.Muntadhar SpB.SpBA Dr Dian Adi SpBA

OMPHALOCELE Omphalocele  abad 16 (Ambrose Pare) Operasi pertama berhasil 1802 (Hey) Gastroschisis  1894 (Taruffi) Operasi pertama berhasil  1873 (Visick)

EXOMPHALOS (OMPHALOCELE)

CONGENITAL Anterior abdominal wall defect at the base of the umbilical cord with herniation of the abdominal contents Exomphalos and gastroschisis are two different congenital anomalies Overall incidence is approximately 2-4: 10,000 live births

PATHOPHYSIOLOGY Failure of the midgut to return to abdomen by the 10th week of gestation

CLINICAL FINDINGS central defect of the abdominal wall beneath the umbilical ring. Defect may vary from 2-10 cm

Always covered by sac The sac may be intact or ruptured Sac is composed of amnion, Wharton’s jelly and peritoneum The umbilical cord inserts directly into the sac in an apical or occasionally lateral position.

Sac contains intestinal loops, liver, spleen and bladder , testes/ovary >50% have associated defects Prognosis depends on theses associated anomalies Mortality is approximately 40%

ASSOCIATED ANOMALIES 40% have chromosomal abnormalities (Trisomy 13, 18, 21, Turner’s and Klinefelter synd) 60-70% infants have associated malformations  Cardiovascular, genitourinary, CNS  Beckwith-Wiedman syndrome  Pentalogy of Cantrell

Prognosis based on associated anomalies

MANAJEMEN DAN RESUSITASI NEONATUS Evaluasi kardiak (auscultasi, periksa tekanan darah pada ektremitas, periksa nadi) IVFD adekuat dan resusitasi cairan Vital sign (jangan sampai hipotermi) Dressing dengan silver sulfadia

OMPHALOCELE

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Conservative treatment

1. 2.

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Reduction by squeezing the sac or placement of a silo for sequential tightening and staged closure Children with giant omphaloceles or concomitant problems that make them poor anesthetic risks may be treated with topical application of Betadine ointment or silver sulfadiazine to the intact sac. This allows secondary eschar formation and eventual epidermal ingrowth. Residual abdominal wall hernias are then repaired at 1 year of age.

Surgical treatment 1. 2.

Primary closure Staged closure

Pertimbangan pilihan tindakan operasi Ukuran defek Usia gestasional Anomali yang berasosiasi

PRIMARY CLOSURE

OPERATIVE MANAGEMENT

STAGED CLOSURE

POST OPERATIF Feeding  bila NGT nonbilus/ minimal peristaltik (+) AB diberikan selam 48 jam

PASCA OPERASI Gastroesofageal reflux Insufisiensi pulmonar Infeksi paru berulang Asma Failure to thrive gastrostomy

GASTROSCHISIS Congenital defect of the anterior abdominal wall just lateral to the umbilicus

Pada gastroschisis terjadi 1 : 4000 kelahiran USG : Pada minggu ke 20 kehamilan, dengan adanya peningkatan AFP

PATHOPHYSIOLOGY Rapid dissolution of the right umbilical vein after the standard period of organogenesis leaves an area of relative weakness in the mesenchyme through which bowel or abdominal viscera can herniate and eventually rupture.

CLINICAL FINDINGS Defect to the right of an intact umbilical cord allowing extrusion of abdominal content Umbilical cord arises from normal place in abdominal wall Opening  5 cm No covering sac (never has a sac ) Evisceration usually only contains intestinal loops Bowels often thickened, matted and edematous Infants have better prognosis than those with an omphalocele (Mortality is approximately 10% ) 10-15% have associated anomalies (intestinal atresia) 40% are premature

MANAGEMENT ABC Heat Management Sterile wrap or sterile bowel bag Radiant warmer Fluid Management Nutrition NPO and TPN Gastric Distention OG/NG tube Infection Control Ampicillin and Gentamicin Associated Defects Closure of the defect

Surgical Management  Primary Closure  Staged Closure

Staged repair using silo pouch

STAGED CLOSURE/SILO

SILO (1997)

SILO

SILO

PRIMARY CLOSURE VS. STAGED CLOSURE PROBLEMS Primary Closure: abdominal compartment syndrome dengan resiko gagal ginjal dan trauma pada intestinal Staged Closure: Infeksi, yang lebih lama sembuh

Omphalocele Gastroschisis Incidence

1:6,000-10,000

Covering Sac Size of Defect Cord Location Bowel

Present

1:20,00030,000 Absent

Small or large

Small

Onto the sac

On abdominal wall

Normal

Other

Liver often out

Edematous, matted Rare

Omphalocele Gastroschisis Prematurity 10-20% If sac is NEC

50-60%

Associated Anomalies

10-15% most Intestinal atresia

Prognosis

ruptured >50%

20%-70%

18%

70-90%