Congenital and Dev Disorders Flashcards
Antenatal imaging procedure to detect gastroschisis?
Prenatal ultrasound
Management options for Gastroschisis?
Primary reduction (Double set-up)
Silastic silo
Gastroschisis is most strongly associated with which anomaly/anomalies?
Bowel abnormalities, atresia or stenoses
Interventions for Gastroschisis patients made prior to transfer?
6 steps
- Wrap bowels in clean kitchen wrap (bowel bag)
- Maintain normothermic
- NPO
- IV Fluid
- NGT
- Explain management to parents
Appropriate management of gastroschisis after primary reduction?
Observation
Long term complications after recovery from gastroschisis?
- Short Bowel Obstruction
- Short Gut Syndrom
- CV catheter-related sepsis
Sac is covered with peritoneum without overlying skin and insertion of distal umbilical cord.
Omphalocele
Management and Treatment for Omphalocele
- Wrap in moist, sterile dressings
- Prompt surgical repair
Omphalocele > 10cm
Giant omphalocele
How can you diagnose omphalocele prenatally?
- Ultrasonography
- Maternal a-fetoprotein (increased)
- Amniocentesis with chromosomal studies (for congenital anomalies)
Patient presents with macroglossia, what syndrome is most likely present?
Beckwith-Wiedemann chromosomal syndrome
(umbilical defect, gigantism, macroglossia, visceromegaly, pancreatic islet cell hyperplasia)
Other coexisting abnormalities commonly seen in patients with omphalocele?
- Cardiac anomalies - VSD, ASD, TOF, Ectopia cordis
- GI anomalies - Duodenal atresia (common in gastroschisis)
- Malignant tumor, neuroblastoma, adrenocortical tumors
- Chromosomal anomalies
Pre-operative interventions for patients with omphalocele
7 steps
- NGT with cont. drainage
- Sterile dressing w/ moist towels
- Plastic foilage
- Lateral position
- IV access
- Transport in heated incubator
- Rectal irrigation - NSS
Treatment options for omphalocele
- Reduce ophalocele, repair abdominal wall
- Immediate operation
- Excision of the sac
- Cylindrical tube (silo)
- Non-operative management
Absence of an anal opening
Imporforate anus
Initial laboratory tests and imaging for patients with imporforate anus?
- Cross-table lateral radiograph (invertogram)
- Xray of spine and chest
- Ultrasonography
- ECG
- MRI
What are the usual associated anomalities in patients with anorectal malformations?
VACTERL association
1. Vertebral/spinal cord abnormalities
2. Anorectal malformations
3. Congenital cardiac disease
4. TEF
5. Esophageal atresia
6. Renal anomalies
7. Limb defects
Diagnostic test/Imaging for Vertebral/spinal abnormalities?
Xray (Babygram), MRI for spinal injury
Diagnostic test/Imaging for Anorectal malformation?
Physical Exam
Diagnostic test/Imaging for Congenital cardiac disease?
2D Echo