Outline of the patients admission to hospital including relevant history, clinical data, diagnosis and clinical progress. This section should also include a short description of the disease area including incidence, pathophysiology and diagnosis, as well as a summary of the drug pharmacology.
CASE SUMMARY-REMOVED
Gastroschisis
Incidence
Gastroschisis is a congenital defect of the anterior abdominal wall located to the right of the umbilicus leading to evisceration of the small and large intestines and in some cases other organs.[1] The condition is diagnosed during pregnancy and thus facilitates planning for the requisite surgery and counselling for parents. The prognosis for most infants affected by gastroschisis is quite good and in 90% of cases they make a full recovery with few long term problems.[2]
Figure 1 Gastroschisis defect with protruding stomach and intestines.[3]
The prevalence rates for gastroschisis vary across regions within the UK from a high of 6.2 per 10,000 births in Wales to a low of 1.6 per 10,000 births in North West Thames. The figure for the Glasgow area is 4.9 per 10,000 births. These figures may differ as a result of the manner in which the data is collected. In Glasgow for instance all data is included, including those cases which led to induced abortions following prenatal diagnosis. This may help to explain the apparent South to North increase in prevalence rates.[4] The average figure has increased significantly over the last ten years from 2.5 to 4.4 per 10,000 births.[5]
Embryogenesis
Understanding the pathogenesis of gastroschisis requires at least a tacit understanding of the normal development of the embryo following conception through to the final stages of gestation leading to birth. At between 3 to 4 weeks of development the gut and the yolk sac become distinct from one another. Following further development at week 6, the midgut elongates a rate which is in excess of that of the embryonic body which leads to the formation of an umbilical hernia. Following on from this at the 10th week of development the herniation is closed after retreat of the midgut to rejoin the embryonic abdominal cavity. It is the failure of this normal physiological development role that is central to gastroschisis, though why it fails to happen is not clear.[6]
Pathophysiology
Genetic
The sporadic occurrence of gastroschisis as an isolated defect suggests that it most likely has multifaceted aetiology. However the occurrence in twins and familial clusters suggest some hereditary involvement and there have been other case reports of possible genetic aetiology in the incidence of the condition.[7, 8]
Environmental
As discussed earlier the increasing prevalence of gastroschisis in births across wide geographical areas and amongst different population sub-types suggest an environmental role in defect occurrence possibly through exposure to teratogens. Medication which acts on the vasculature has been implicated including some herbal and over the counter medications such as pseudoephedrine, paracetamol and aspirin amongst others.[9, 10] Some reports have suggested a link with maternal smoking and gastroschisis with an increase in prevalence reported as well as poorer outcomes for those mothers who smoked.[11] Drinking alcohol, smoking and taking ibuprofen were also implicated in a moderate increase in the likelihood of gastroschisis in another study.[12]
Treatment of Gastroschisis
The management of gastroschisis falls into a number of categories mainly based on the stages of treatment and includes (although is not limited to); pre-surgical management, surgical management/techniques, parenteral nutrition and pain and infection control.[3]
1. Pre-surgical management
Assuming detection at the 20 week scan, management of both the mother and foetus begins almost immediately and will usually begin with a decision on the plan for the mode of delivery. Whilst intuitively one might suspect that an elective Caesarean section is the preferred option for delivery to remove the possibility of trauma to the exposed organs, the clinical data does not suggest a significant difference exists with vaginal delivery.[13] There are a significant number of considerations that must be made in the management of a newborn with gastroschisis. These include amongst others thermoregulation, fluid volume status, gastric distention and intestinal compromise, infection control, respiratory status, and preparation for surgery. Investigation to determine the existence of other anomalies should also be carried out as some patients with gastroschisis may also have other anomalies although their occurrence is less than with other abdominal wall defects such as omphalocele. REFERENCE
The outcome for the infant is best when these considerations have been stabilised before surgery takes place. Following delivery a "bowel bag" is used to protect the infant's trunk and lower extremities and the infant is placed in a warmer to maintain body temperature. The use of a bowel bag also facilitates the reduction in heat loss through the large area of exposed tissues and also provides some protection against infection. The pooling of fluid lost through the exposed tissue also allows for more accurate calculation of the total volume of lost fluid. The loss of large volumes of fluid may mean that the infant displays symptoms of shock and management with isotonic solutions may be required. Once normalisation of urine output occurs or the acid/base balance equilibrates fluid resuscitation may be ceased. The increase loss of fluid volumes through the exposed tissue will mean that the maintenance volumes of fluid required for an infant with gastroschisis will be 2 or 3 times that required for a normal infant. Therefore it is important to monitor the infant's glucose load; multiple infusions with dextrose solutions can lead to hyperglycaemia.
A naso/orogastric tube is also required to prevent gastric distension. The tube should intermittently be placed under suction to keep the bowel and the stomach decompressed. This is important as it facilitates the prevention of total or partial blocking of blood flow and oxygenation of the bowel. The neglecting of this requirement may lead to an increase risk for necrosis of the bowel. Diminution in the infants risk for emesis and thus aspiration is also facilitated by decompression.[14] Positioning of the infant is also important and can help to reduce the risk of bowel compromise. Therefore as a means of increase venous blood return from the gut, infants should be placed on their right side in a lateral decubitus position.
One of the final and perhaps most important considerations needed before surgery is prophylactic antibiotic therapy. Due to the nature of the condition and the fact of surgery by itself infants are broad spectrum antibiotics are administered to reduce the possible risk of infection from the exposed bowel. Typically biochemical and haematological tests are also carried out before surgery.
2. Surgical management/techniques
Some controversy surrounds the different approaches to surgical treatment of gastroschisis. Whilst primary closure of the defect is the preferred approach a staged silo closure may be required if it is deemed necessary by the surgical team. The predominant determinant of this is the size of the gastroschisis. Small and medium sized gastroschisis may permit primary closure while larger gastroschisis will necessitate a staged closure usually over 7-10 days. This procedure involves tucking the exposed bowel into a silastic sheet or silo which is affixed perpendicular to the torso. Periodically the silo is shortened to force the bowel back into the abdominal cavity. Once the entire bowel has been returned to the abdominal cavity the infant's abdomen is then surgically closed Figure 2-5.
Figure 2 Infant with gastroschisis on day 6 following surgical application of a silimed gastroschisis
Figure 3 Infant with gastroschisis on day 8 following surgical application of a silimed gastroschisis
Figure 4 Infant with gastroschisis on day 9 following surgical removal of silo
Figure 5 Closure of the abdominal wall
3. Parenteral nutrition, pain control and infection control
These three concerns play a dominant role in the function of the clinical pharmacist. Infection is a primary concern for infants with gastroschisis. Obviously, the breech in the skin which normally functions as a barrier to infection causes an increase risk to infants with gastroschisis. Those patients who undergo a stage repair may spend a number of days with this continuing risk.
Research has shown that sepsis is the primary cause of mortality in patients with gastroschisis although delay in the closure of the abdominal wall defect showed no significant affect on outcome.[15] There are also other factors which need to be considered in terms of the increased risk for gastroschisis patients. These include the prolonged need and use of a central venous line, long-standing need for TPN and the general immaturity of the patient's immune system.
Following surgical treatment the continuation of the use of broad spectrum antibiotics for a period of 3-7 days is advised and all health care staff are encouraged to be vigilant for any physical signs of infection peripheral to the wound site.
Expand this section to include discussion on EOS and LOS as well TDM in Neonates etc.
Following initial stabilisation the main goal is to provide sufficient nutrition and to manage pain. Since intestinal dysmotility caused by exposure of the intestine to inuterine amniotic fluid is a factor for all patients total parenteral nutrition (TPN) is required. Due to the fact that TPN will be needed fo a number of weeks it usually requires a central line to be administered and is normally started 24 -48 hours post operatively. The TPN requirements post operatively as as a minimum 90 to 100kCal/kg/day, 3g/kg/dy of protein and 3-4 g/kg/day of intravenous lipids and dextrose to maintain euglycaemia. However, due to the surgical stress additional protein in the TPN may be needed. Once gut motility improves enteral feeds should be commenced as soon as possible since there is a positive correlation between time of introduction of enteral feeds and hospital discharge. A number of different feed types have been indicated including expressed human milk, pre-term formula and elemental formula as they are easily digested. Usually at the initiation of feeds the volumes are low around 10-20 ml/kg/day
The control of pain is of crucial importance in dealing with infants with gastroschisis and they should be assessed for pain. The assessment should be carried out following established and validated pain assessment tool and guidelines for pain management should be adhered to in terms of the administration of analgesia.
Expnad this sectiona a little re use on Morphine is neonates.
Pharmacological Treatment
In this case the infant concerned was treated blah blah
Drug Pharmacology? TO BE ADDED Only given Morphine, Antibiotics and anti fungal...maybe TPN as well?
2. Evidence for the treatment of the condition and conclusions
Should include recent published evidence to support or refute the use of particular drugs in the treatment of a patients specific condition. The evidence should link to the management of the patient described in the case summary and should provide critical appraisal of the therapeutic decisions made.
Treatment of Baby L
Baby L was treated in a "textbook" manner. Due to the size of her gastroschisis the abdominal wall was closed by the silo method. NEED TO EXPAND TO DEVELOP CRITICAL APPRAISAL OF THE METHODS USED -OK
3. Patient medication profile and care plan REMOVED
