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Key messages

  • Gastroschisis is characterised by the herniation of bowel and other abdominal contents through an abdominal wall defect, just to the right of the umbilicus.
  • The normal insertion of the umbilical cord into the abdominal wall distinguishes gastroschisis from exomphalos.
  • Specific attention to intravenous (IV) fluid management is required during stablisation.
  • Approximately 20 babies with gastroschisis per year are managed in Victoria.

Gastroschisis is characterised by the herniation of bowel and other abdominal contents through an abdominal wall defect usually located just to the right of the umbilicus. The normal insertion of the umbilical cord into the abdominal wall and the absence of a sac covering the herniated abdominal contents distinguishes gastroschisis from exomphalos, the other common abdominal wall defect.

While parts of this guideline are written primarily for Victorian Level 6 services, the resuscitation, stabilisation and transfer sections are relevant across all levels of health service capability. It covers:

  • management of the protruded abdominal contents
  • fluid management during stabilisation
  • fluid management in the ongoing pre-operative phase.

Abbreviations

MCH: Monash Children's Hospital

RCH: Royal Children's Hospital

RWH: Royal Women's Hospital

MHW: Mercy Hospital for Women

NICU: Neonatal Intensive Care Unit

ANUM: Associate Nurse Unit Manager

IVCs: Intravenous Cannulae

Management

The following document provides guidance for the management of gastroschisis from prenatal diagnosis to post-operative and continuing care.

Antenatal

The principles for management in the antenatal period involve the parents and the multidisciplinary perinatal team.

  • More than 90 per cent of cases are prenatally diagnosed and are managed at MCH, RWH or MHW.
  • Parents receive antenatal multidisciplinary counselling, including consultant paediatric surgical input, so they are generally well prepared.
  • Parents should be offered a broad orientation to the NICU environment antenatally.
  • In contrast to exomphalos, gastroschisis is rarely associated with aneuploidy or other major non-GI congenital abnormalities. Between 10-15 per cent have associated intestinal atresia.
  • In the absence of other obstetric factors, babies with uncomplicated gastroschisis may be safely delivered vaginally. There is a significant incidence of late preterm birth. Many of these relate to induction or semi-elective caesarean section for concern about fetal wellbeing and the wellbeing of the exposed bowel. There is an association between gastroschisis and late fetal death in utero. Median gestation is 36 weeks and median birth weight is 2284 g.
  • Two-thirds of babies require early respiratory support, reflecting the effect of one or more of prematurity, growth restriction, perinatal asphyxia or aspiration of gastric contents.
  • For elective or semi-elective births, the perinatal team should liaise with the MCH or RCH duty neonatologist. Where there is an obstetric imperative to deliver the baby, NICU access will not influence the timing of such births.
  • On the day of anticipated birth (planned or spontaneous), the perinatal team should liaise with the MCH or RCH duty neonatologist or NICU ANUM, who will in turn advise the paediatric surgeons about the impending birth.

Postnatal

The principles of management following birth and during the pre-transfer period address fluid resuscitation, gastric decompression, avoidance of hypothermia and care of the exteriorised abdominal contents.

Birth suite

  • Prepare as for anticipated high-risk birth.
  • Manage airway, breathing and cardiovascular status as per usual practice. In the compromised baby, to minimise gut over-distension, endotracheal intubation should be undertaken rather than prolonged mask ventilation or nasal CPAP.
  • Once the cardiorespiratory status has been stabilised, quickly inspect the bowel, correcting any obvious twists on its pedicle or acute discoloration due to ischaemia. The bowel should then be positioned centrally over the abdomen, supported and wrapped in cling wrap as described below.
  • Insert an 8Fr nasogastric tube and aspirate the stomach. Leave on free drainage.
  • Ensure adequate thermal control.
  • If there is a concern about the appearance of the intestines, the paediatric surgical team must be informed immediately (for example, from the delivery room at MCH).

Management of exposed bowel

There are a number of different methods to manage the exposed bowel. Two methods are described below: one is recommended at Monash Children's, the other is used for babies cared for by PIPER, RWH, MHW and RCH.

  • Following assessment, the exposed bowel should be wrapped with cling wrap (transparent, latex free) for protection and to minimise fluid and heat loss: see Figure 1 and 2
  • Monash procedure:
    • Clean procedure, does not need to be sterile.
    • Construct a roll from a standard flannel nappy and cover the roll with cling wrap.
    • Slide large piece of cling wrap under the baby's buttocks and back.
    • Place exposed organs on baby's abdomen (using sterile latex-free gloves).
    • Ensure the intestine is positioned to minimise tension and optimise blood supply and place the cling wrap covered roll as a 'donut' to support the intestine resting on the abdominal wall.
    • Wrap cling wrap gently around the abdomen and exposed organs.
    • Ensure bowel edges are not exposed to drying air.
    • Avoid compressing the bowel, it should remain mobile but protected.
  • PIPER / RWH / MHW / RCH procedure:
    • Clean procedure, does not need to be sterile.
    • Slide large piece of cling wrap under the baby's buttocks and back.
    • Place exposed organs on baby's abdomen (using sterile latex-free gloves).
    • Wrap cling wrap gently around the abdomen and exposed organs.
    • Ensure bowel edges are not exposed to drying air.
    • Avoid compressing the bowel, it should remain mobile but protected.
  • Monitor the bowel every 15 minutes for dusky or blanching colour changes:
    • remove and rewrap as above if compression, kinking or twisting is suspected.
  • Support the intestines to prevent occlusion of the blood supply where the bowel exits the defect in the abdominal wall:
    • if necessary support the exposed intestines with your hands.
    • where possible, nurse the neonate on their right side, with the wrapped bowel supported perpendicular to the umbilicus using a rolled towel or equivalent.
    • if you have any concerns regarding bowel colour, position or viability, discuss immediately with the paediatric surgical team, and seek senior medical advice.
      Figure 1: gastroschisis
      Figure 1
      Figure 2: 28 week gastroschisis
      Figure 2

       

Perinatal Unit

General

  • The objective in all babies is to stabilise and transfer within four hours of birth.
  • If the baby is delivered at Monash Health Clayton (co-located with MCH) the objective is to close the gastroschisis or achieve silo cover within six hours of birth.
  • Notify PIPER (1300 137 650) when the baby is born (do not wait until stabilisation is complete).
  • Manage the airway, breathing and any cardiovascular instability as per standard practice.
  • Ensure the protruded abdominal contents remain wrapped and supported as per the methods described above.
  • Specifically, reassess the bowel status regularly to ensure it remains supported and not twisted.
  • Establish vascular access. The minimum required will be two peripheral IVCs. Arterial access is not required at this stage unless there is significant respiratory or circulatory compromise. Collect blood sample for baseline gas, electrolytes, glucose and culture when IV inserted.
  • Give intravenous benzylpenicillin and gentamicin.
  • Ensure the baby is clinically assessed for other congenital anomalies.
  • Ensure continued thermal control.

Fluids

  • Insensible losses will be unavoidably high.
  • Commence maintenance fluid 10% Dextrose at 60 ml/kg/day to maintain blood glucose >3 mmol/L.
  • Give a 20 ml/kg fluid bolus of normal saline within an hour of birth.
  • Run an additional 10 ml/kg/hr of normal saline as the default minimum ongoing replacement fluid.
  • Maintain an accurate fluid balance record for all infants, including gastric losses.
  • Review fluid balance and clinical status once a total of 40 ml/kg saline has been administered or as determined by the outcome of repeated assessment of capillary refill, heart rate, blood pressure and acid-base/lactate.
  • At this point, ongoing fluid replacement needs to take account of possible clotting factor dilution and/or the need for colloid as opposed to further crystalloid.
  • Consider adding KCL 10 mmol/L to nasogastric replacement fluid.

Transfer to NICU with paediatric surgical services (MCH or RCH)

For babies not born at Monash Health Clayton, the retrieval team will:

  • treat the referral as time critical
  • leave the gastroschisis wrapped and undisturbed after ensuring it is well supported and the bowel has remained well perfused
  • continue the fluid management regime detailed above.

Perioperative care in the surgical NICU

  • Inform the paediatric surgeons of baby's arrival.
  • Ensure continued support of the gastroschisis.
  • Unless there is an immediate need to act otherwise, for example concern about pedicle twist with discoloration/vascular compromise of bowel, wait for the surgeon to arrive before disturbing the wrapped bowel.
  • Discuss ongoing pre-operative fluid management with the duty neonatologist - default is to continue 10 ml/kg/hr of replacement fluid.
  • Consider siting a urinary catheter to decompress the bladder.
  • If nasogastric/orogastric losses are in excess of 20 mL/kg/day, manage according to NICU protocol.
  • Usual NICU admission assessment and documentation.
  • Initial blood sampling should include:
    • Blood gas
    • lactate
    • glucose
    • FBE
    • electrolytes
    • coagulation profile
    • cross match (ASBT).
  • Repeat biochemistry 8-12 hourly or as directed by clinical circumstances.
  • Total Parenteral Nutrition (TPN) ordered for next day and commence when available post-operatively.
  • Continue antibiotics (benzylpenicillin/gentamicin) and add Metronidazole if a silo is required, if there is a bowel perforation or if the bowel appears significantly compromised by ischaemia.
  • Arterial line placement is generally undertaken when the baby is anaesthetised in theatre.
  • Long-term central venous access device (PICC) is usually inserted within the first 6-12 hours post-operatively.
  • Post-operative weaning from mechanical ventilation, along with feed commencement and advancement, are related to the type of closure (primary versus silo), the degree of bowel wall thickening (peel), the presence of intestinal atresias, the tightness of the abdomen following the closure, and potential post-operative complications such as small bowel obstruction or bowel perforation.

Surgical considerations

  • The paediatric surgical team is involved in antenatal counselling.
  • Paediatric surgeons are kept informed by perinatal or RCH/MCH NICU staff of impending planned or unplanned births.
  • Paediatric surgeons should be contacted by perinatal or RCH/MCH NICU staff if there are concerns about the bowel status at any point after birth.
  • It is usually planned to operate within four hours of arrival in the NICU. The site for surgery (NICU vs operating theatre) is determined by patient factors and site logistics. The aim of initial surgery is to close the abdominal wall defect. Management of an associated atresia is generally delayed until a subsequent surgery.
  • The surgical options are reduction of abdominal contents and primary repair of the abdominal wall, or the application of a silo (a transparent bag which covers the bowel contents while they are gradually reduced into the abdominal cavity). In the latter case the aim is to achieve closure within seven days and the surgical team will manage the reduction of the bowel on a daily basis.
  • In more complex cases (perforation, atretic segments) a 'clip and drop' approach may be required with definitive management delayed to a subsequent surgery when the baby is better able to tolerate a prolonged procedure. There are many different surgical options in complex gastroschisis, depending on anatomy and surgical preference.
    Paediatric surgeons and NICU consultants jointly manage feed initiation and advancement. It can take weeks to months for feeding to become established.
  • In babies where initiation or advancement of feeds is significantly delayed (generally more than 4-6 weeks post closure of the abdominal defect) the surgical and neonatal consultants involved will consider the need for contrast imaging and further surgery. The focus will be on considering the likelihood of finding a mechanical explanation (stricture, unanticipated atresia, obstruction due to adhesions) versus functional intestinal dysmotility.

Additional non-acute considerations

  • Anomaly screening:
  • cranial, cardiac and renal USS are performed if there are specific clinical indicators
  • microarray is offered prenatally in selective cases. It is not required postnatally unless significant non-GI anomalies are found.

Nursing considerations

  • On arrival, complete nursing admission assessment and documentation.
  • Nurse neonate on radiant warmer and use servo control for temperature balance and monitoring.
  • Ensure appropriate cardiorespiratory and saturation monitoring is applied.
  • Monitor gastroschisis defect and report abnormalities to medical team. For ongoing management refer to management of exposed bowel.
  • Monitor neonate's central and peripheral perfusion every 15 minutes prior to surgery.
  • Ensure Nasogastric Tube (NGT) is placed on free drainage and aspirated hourly.
  • Consider replacement of NGT losses as per NICU protocol.
  • Document accurate fluid balance and undertake regular assessment of hydration status.
  • Give fluids and medications as prescribed.
  • Monitor intravenous cannula site(s) as per unit protocol.
  • Ensure regular monitoring of electrolytes per medical orders.
  • Undertake pain assessment as per neonatal Pain Assessment Tool.
  • Orientate parents/family to NICU.
  • Provide ongoing explanation of continuing care to parents.

Get in touch

Maternity and Newborn Clinical Network
Safer Care Victoria

Version history

First published: February 2018

Last web update: October 2018

Review by: February 2021

Uncontrolled when downloaded

Page last updated: 21 Nov 2018

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