Gastroschisis
Key points
1 in 2,250 UK births
Possible failure of migration of right lateral ventral fold
Left sided variant - possible different aetiology - more extra-intestinal abnormalities
Peel - Type 3 collagen ?chemical exposure ?chronic vascular insufficiency
Definitions
Simple: No known intestinal complications
Complex: Any of atresia/perforation/volvulus/ischaemia/bowel loss
Antenatal management
Visible on 20-week USS - differentiate from exomphalos (sometime sac is visible, liver herniation is possiblein gastroschisis but more frequent in exomphalos)
Maternal serum alpha-fetoprotein (AFP) may be raised
Polyhydramnios may suggest atresia
Bowel matting decreased with higher age at delivery
Intra- and extra-abdominal bowel dilatation and gastric dilatation on antenatal USS are associated with increased risk of complex gastroschisis
In closing gastroschisis - may not have prenatal diagnosis, but can have autoanastomosis - true congenital short gut
Timing of delivery
No consensus on timing of delivery
A closing defect may be indicated by increasing intra- and extra-abdominal bowel dilatation, however early delivery (<37 weeks) based on these metrics alone has been shown overall to be detrimental to bowel function (time to full feeds and length of stay) (Carnaghan JPS 2014)
No need for C-section
Primary vs silo closure
2021 NETS2G study (BAPS CASS) - 1268 infants - use of silo for uncomplicated gastroschisis better than primary closure (less perforations, resections), but 40% increase in operation rate
For complicated gastroschisis, no benefit to silo closure over primary closure - possibly due to intention to close in theatre allows for more thorough assessment of condition
Post-operative considerations
NEC in around 1% (lower than historically thought)
GORD
40% undescended testes - 20% at 1 year
Growth often catches up
Standard scenario
Concerns are
1. Simple or complex - based on length + condition of the bowel: ischaemia, atresia, perforation
2. ?Ruptured exomphalos
Ensure resuscitation on NICU with IV fluids, antibiotics, cover with film
If closing ring and ischaemic bowel - use local anaesthetic and make superior midline cut on NICU
Examine for above features, abdomino-visceral disproportion, position of testes
Check antenatal scans for polyhydramnios (atresia)
Consent and counsel for Primary/Silo closure
Risk: Abdominal compartment syndrome
Procedure
Reassess abdomino-visceral disproportion
Reduce bowel if possible
If intra-abdominal testes - push towards internal ring - mark with prolene
Separate muscle and skin
Check ventilation pressure then close
Or
Preformed silo
Reduce over course of week
Other options:
Hand sewn silo
Perforation:
If bowel in good condition and small perforation - close with suture and reduce
If bowel not in good condition/large perforation - reduce as much as possible - and bring out perforation as stoma
If non-reducible - control perforation with catheter through hole in preformed silo
Atresia:
If condition of bowel (minimal peel) and patient favourable - manage as per atresia pathway
If unfavourable - reduce bowel - primary closure or silo, relook in 6 weeks
If atretic segment very dilated - enterotomy, decompress and stoma
Post op
Long line + PN
Median time to full feeds 22 days
If bowel function not present after 4 weeks - UGI contrast follow through for atresias
Follow up
Counsel regarding adhesive obstruction
Paediatrics to monitor growth
Orchidopexy if needed
References
Morris JK, et al. Prevalence of vascular disruption anomalies and association with young maternal age: A EUROCAT study to compare the United Kingdom with other European countries. Birth Defects Res. 2022 Dec 1;114(20):1417-1426. doi: 10.1002/bdr2.2122. Epub 2022 Nov 11. PMID: 36369770; PMCID: PMC10099853.
Sun RC et al. Prenatal ultrasonographic markers for prediction of complex gastroschisis and adverse perinatal outcomes: a systematic review and meta-analysis. Arch Dis Child Fetal Neonatal Ed. 2022 Jul;107(4):371-379. doi: 10.1136/archdischild-2021-322612. Epub 2021 Oct 4. PMID: 34607856.
Carnaghan H, Pereira S, James CP, Charlesworth PB, Ghionzoli M, Mohamed E, Cross KM, Kiely E, Patel S, Desai A, Nicolaides K, Curry JI, Ade-Ajayi N, De Coppi P, Davenport M, David AL, Pierro A, Eaton S. Is early delivery beneficial in gastroschisis? J Pediatr Surg. 2014 Jun;49(6):928-33; discussion 933. doi: 10.1016/j.jpedsurg.2014.01.027. PMID: 24888837.
Stokes, Sarah C, and Fuad Alkhoury. "Gastroschisis." Pediatric Surgery NaT, American Pediatric Surgical Association, 2022. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829060/all/Gastroschisis.
Rentea RM, Gupta V. Gastroschisis. [Updated 2023 Apr 10]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK557894/
Shailinder Singh, Nottingham Children's Hospital teaching series