Gastro D-G Flashcards
What is Exomphalos and gastroschisis?
Congenital anterior wall defects resulting in herniation of abdominal viscera.
What is the background of Exomphalos and gastroschisis?
Exo: 3/10k, Gastrosch: 5/10k.
Embryology: abdominal wall forms after the infolding of caudal and rostral and two lateral embryonic folds. This starts at 6/40 and ends at 12/40, when the intestine undergoes 270deg clockwise rotation and then returns into abdominal cavity.
Exomphalos: failure of migration of bowel back into abdomen, remaining in umbilical cord. Failure of abdominal wall folding.
Gastroschisis: ischaemic injury to developing anterior abdominal wall leading to evisceration of bowel through a defect in the right side of abdominal wall.
What are the associations of Exomphalos and gastroschisis?
General: history of ischaemia in the foetus, may be associated with maternal smoking and drug use, alcohol.
Exomphalos: associated with chromosomal abnormalities (commonly trisomy 18/13). Increased maternal age. Pentalogy of Cantrell: cranial fold defects, exomphalos, diaphragmatic hernias, sternal clefts, pericardial and cardiac defects.
Gastroschisis: Rare associated abnormalities, intestinal atresia, cryptorchidism, low maternal age.
What are the history and exam findings of Exomphalos and gastroschisis?
Antenatal: USS detection in utero, transfer to paediatric surgical centre with antenatal counseling.
Exomphalos: Herniation of the bowel, liver or other organs into umbilical cord. Herniated viscera enveloped in a membrane.
Gastroschisis: Intatct umbilical cord but evisceration through a defect to the right of the cord. No membrane covering. Red matted bowel loops.
What investigations would you do for Exomphalos and gastroschisis?
USS: screening antenatally
Amniocentesis: if associated with chromosomal abnormalities.
What is the management of Exomphalos and gastroschisis?
General: resusc, NGT with frequent aspirations, IV access with dextrose feeding. Intubation and ventilation with surfactant therapy. Prophylactic antibiotics. Cling film therapy to reduce water losses.
Surgical: prompt assessment to make diagnosis and ensure bowel is not compromised. Compromise requires urgent surgery.
Definitive surgical management: Majority of lesions are fixed by primary closure. Larger lesions may require spring loaded silastic silo applied in the NNU. Staged reduction then achieved over two weeks, with eventual complete closure and reduction of intra abdominal pressure / intra gastric pressure.
What is the prognosis and complications of Exomphalos and gastroschisis?
Adhesions, SBS, syndrome related cmx, volvulus antenatally.
Good with antenatal diagnosis, and appropriate surgical management.
What is Encopresis (fecal soiling)?
Voluntary or involuntary passing of faeces in places and moments when inappropriate, in a child of age at which fecal control is expected (>4y).
What is the background of Encopresis (fecal soiling)?
3/100 children, reduces with age.
Retentive encopreis: occurs with chronic constipation: rectal fullness -> distension -> muscle weakness and distension -> loss of normal sensory information and muscle control -> fecal soiling.
Non retentive encopresis: without constipation
Emotional: due to conflict at home, psychological distress, neglect.
Related to surgical treatment for Hirschprungs, anorectal malformations, neuropathy, behavioral problems, low birthweight and enuresis.
What are the history and exam findings of Encopresis (fecal soiling)?
Detialed history of encopresis, neonatal and birth, social history important too.
Abdominal: distension and impacted feces if constipated.
DRE: only by senior doctors, can feel impacted feces if constipation, enel fissures or tears, anal tone and wink, (lax if both retentive of ARM etc related).
What investigations would you do for Encopresis (fecal soiling)?
Anorectal manometry: to measure defecation dynamics, exclusion of ultra short Hirschprings.
Radionuclear transit scintiography: identifies slow colonic transit.
What is the management of Encopresis (fecal soiling)?
Medical: constipation management with diet, activity, routines, enemas, colonic evacuation (disimpaction). Laxative (ie Senna) at night encouraging motion after breakfast.
Behavioral strategies: if psychosocial cause suspect. Scheduled toileting, resolving conflict and stress.
Parental child education: avoiding conflict and anger, positive reinforcement of patterns.
Biofeedback training: to resolve paradoxical constriction of external anal sphincter in children with chronic constipation. Allows EAS relaxation.
Surgical intervention: anterograde continence enema in Hirschprungs, aloowing administration of enema directly into caecum or distal colon.
What is the prognosis and complications of Encopresis (fecal soiling)?
Low self esteem, psychological, fall behind in school.
Depends on cause, unusual to persist after teenage years.
What is Food allergy?
Immune mediated hypersensitivity reaction to food proteins.
What is the background of Food allergy?
6-8% of children. 90% are caused by the big eight: cows milk, peanuts, sesame, kiwi, eggs, wheat, fish, soybean, shellfish, nuts.
Associated with parental atopy and atopic eczema, allergy, allergic rhinitis.
What are the history and exam findings of Food allergy?
Same as CMPA
What investigations would you do for Food allergy?
Same as CMPA
What is the management of Food allergy?
Avoidance: once confirmed, dietitian input is key. Advice on eating out, food labels etc.
Emergency plan: in IgE mediated, training on how to assess severity of reaction. Wearing MedicAlert bracelet. Epipen is indicated in children with proven cardiorespiratory symptoms as a result of contact with allergen. Often indicated in peanut or tree nut allergy, or in teenagers.
What is the prognosis and complications of Food allergy?
Anaphylaxis.
Children often grow out of all, apart form nut/peanut allergies and shellfish.
What is Functional abdominal pain?
Abdominal pain of enough severity to interfere with daily activities but with no proven underlying pathological disease.
What is the background of Functional abdominal pain?
4-25% of school children.
Intermittent or continuous (Rome III criteria). Chronic >2/12. Proposed mechanisms:
· Enteric nervous system ENS: response to noxious or stressful stimuli (inflammatory) psychological (anxiety) or physiological (distension from meal)
· Visceral hyperplasia: low pain threshold to high interluminal pressure, altered sensation in nerves by infection or inflammation.
· Biopsychosocial model: response to biological factors, governed by interaction with environment.
Pthology: marked basal hyperplasia, vascular ectasia and numerous intraepithelial eosinophils / lymphocytes. Lymphoid aggregates , crypt hyperplasia, villous atrophy, increased intraepithelial lymphocytes in duodenum.
What are the history and exam findings of Functional abdominal pain?
Needs to be distinguished from anatomica, infectious, inflammatory or metabolic cuase of pain. May be present with dyspepsia, IBS, migraine of FAPS.
· IBS: pain worse before and relieved after defecating. Stools with excess mucus, bloating, incomplete defecation and constipation.
· Functional dyspepsia: epigastric pain, postprandial vomiting, early satiety and GOR.
· Abdominal migraine: paroxysmal pain with anorexia, N&V, pallor.
· Functional abdominal pain syndrome: FAP without the three above syndromes. (IBS, FD, AM).
What investigations would you do for Functional abdominal pain?
If symptoms and signs indicate organic cause (weight loss, deceleration of growth, GO blood loss, significant vomiting, localized pain, pyrexia, FHx IBD.
Endoscopy with negative biopsy may exclude organic disease. USS for RIF / RUQ pain for gallstones / ovarian cyst / appendix.
What is the management of Functional abdominal pain?
Supportive: positive diagnosis, not by exclusion. Careful explanation, headache parallel, reasonable goals establishment, absence of organic disease.
Psychological: may require therapy to work out stresors, biofeedback, CBT.
Medical: time limited analgesia use. H2 antagonists of PPI.
Dietary modifications: no evidence.
