Gastro H+I

Question 1

What is Hernia: Congenital diaphragmatic hernia (CDH)?

Answer 1

Congenital defects in the formation of the diaphragm which results in the protrusion of abdominal contents into the thoracic cabity.

Question 2

What is the background of Hernia: Congenital diaphragmatic hernia (CDH)?

Answer 2

1/2k infants.

More common unilateral though may be bilateral. L>R. Bowel or intraabdominal viscera may be herniated. Common for liver and spleen to be herniated, with associated abnormal hepatic vasculatures. Associated with pulmonary tree abnormalities, surfactant defificney and vasculature changes (hypoplastic lung)

· Posterolateral Bochdalek heriaL 90%, left sided, posterolateral

· Morgagni hernia 3%, right sided anterolateral

· Congenital hiatus hernia: rare, stomach through the esophageal hiatus.

Associated w/ cardiac abnormalities, affected siblings, PPH, CRA, malrotation of bowel.

Question 3

What would you find in history exam of Hernia: Congenital diaphragmatic hernia (CDH)?

Answer 3

Hx of polyhydramnios, cyanosis, respiratory distress as a neonate. Bochdalek hernia: poor air entry on L and shift in cardiac sounds to the right of the chest. Smaller defects present later as wheezy child or recurrent chest infection.

Question 4

What investigations would you do for Hernia: Congenital diaphragmatic hernia (CDH)?

Answer 4

Karyotype if appropriate, chromosomal studies

Radiology: CXR with placing of orogastric tube to aid gastric positioning. Cardiac echo for associated abnormalities.

Question 5

What is the management of Hernia: Congenital diaphragmatic hernia (CDH)?

Answer 5

Medical: NGT to decompress, respiratory support via ETT and mechanical ventilation (avoid high peak inspiratory pressures) Avoid bag/mask. UAC/UVC with monitoring of ABG. Surfactant administration after intubation. ?NO therapy and ECMO.

Surgical: if stabilized, open subcostal approach or thoracoscopic/laproscopic approach. Depends on position of hernia and surgeon. Surgical technique involves careful reduction of herniated contents, definition of the posterior rim and repair with non absorbable sutures.

Question 6

What are the complications and prognosis of Hernia: Congenital diaphragmatic hernia (CDH)?

Answer 6

Pulmonary hypoplasia, malrotation, volvulis, perforations, renal hypertrophy

Mortality 25-60%. Associated with degree of pulmonary hypoplasia.

Question 7

What is Hernia: Inguinal hernias (IH)?

Answer 7

Abnormal protrusion of abdominal structures through the inguinal canal and into the inguinal region or scrotum.

Question 8

What is the background of Hernia: Inguinal hernias (IH)?

Answer 8

5% ful term and 30% premature infants. RHS 60%. Bilateral 15%.

Testicle develops retriperitoneally and then descends into the processus vaginalis under the control of hormones and gubernaculum. The processus vaginalis closes after development but rmains patent in some chinden (PPV) allowing the passage of boewl (IH) or fluid (hydrocele) into the canal. In females, the gubernaculum becomes the ovarian ligament and round ligament, with a PPV it etends into the labium and becomes the canal of Nuck. Hernia is most commonly ileum (male) or ovary (female).

Assoc w/ preterm, low BW, FHx, undescended testes, gastroschisis/exomphalos, Ehlers Danlos, CF, CT disorders and anything increasing IAP.

Question 9

What would you find in history exam of Hernia: Inguinal hernias (IH)?

Answer 9

Infant: inguinoscrotal swelling ,First presentation with incarceration. Unable to palpate cord superiorly (Differentiate hydrocele) Reducible unless incarcerated. Non transluminable.

Child: supine and standing position, expansile cough impulse

Incarceration: pain, tender non reducible scrotal mass, erythema, vomiting, abdominal distension

DDX: torsion, hydrocele, retractile testes, indescended, femoral hernia, lymphadenopathy.

Question 10

What investigations would you do for Hernia: Inguinal hernias (IH)?

Answer 10

USS rarely needed to diagnose.

Question 11

What is the management of Hernia: Inguinal hernias (IH)?

Answer 11

General: herniotomy preformed, Hernia sac located, separated form vas deferens and testicular vessels, transected and ligated. Can be laproscopic with placement of intraperitoneal purse string for closure.

Neonate with reducible hernia: elective repair. Infant: elective repair.

Incarceration: manual reduction under sedation (IV morphine) with repair within 48h.

Question 12

What are the complications of Hernia: Inguinal hernias (IH)?

Answer 12

Inguinal hernia: incarceration (50% within 1y). Henioromy recurrence 1%.

Prognosis excellent with surgical repair.

Question 13

What is Hirschsprung disease?

Answer 13

Aganglionosis of the enteric (Auerbach) and submucoasl (Meissner) plexus. Results in functional obstruction.

Question 14

What is the background of Hirschsprung disease?

Answer 14

1/5k live births, 20% neonatal obstruction.

General: affects anus and variable distance of bowel proximally from it. Classified according to distal bowel affected (ultra short, short, long, total colonic, total intestinal).

EmbryologyL failure of neural crest derived ganglion cells to migrate from proximal to distal bowel during development, leading to lack of PSNS nerves. SNS only in affected bowel leads to hyerptonicity and lack of secretion/ relaxation in response to distension. This leads to stool stasis and narrow lumen.

Pathlogy: increased number of acetylcholinesterase stained nerve endings, and hypertrophied nerve trunks in lamina propria or muscolaris propria.

Question 15

What would you find in history exam of Hirschsprung disease?

Answer 15

NN:

· Failure to pass meconium <24h.

· Acute intestinal obstruction, distension, poor feeding

· Life threatening enterocolitis

Infantile:

· Chronic obdurate constipation with abdominal distension, No soiling.

· Intermittend abdominal pain and fever during episodes

· Failure to thrive

Depends on age of presentation. Typically abdominal distension with dilated bowel. PR exam leads to sudden explosive gush of stool as examination bypasses aganglionic segment, Septic signs if infected

Question 16

What investigations would you do for Hirschsprung disease?

Answer 16

Bloods: high WCC and CRP if infected. Blood cultures. venous gas.

Radiology: AXR shows dilated bowel, exclude atresia as DDX, Gastrogriffin enema may show transition zone.

Suciton rectal biopsy: taken at 2, 3, 4 cm from anal margin. Full thickness biopsy under GA required if insufficient sample with SRB

Anorectal manometry: older children with consitpaiton.

Question 17

What is the management of Hirschsprung disease?

Answer 17

Initial: managed with BS ABx, NGT decompress, rectal washouts. ? colostomy

Surgical: pull through preformed (3-6/12). Can be preformed with laproscopic assistance, take biopsies for investigation of segment length

Question 18

What are the complications and prognosis of Hirschsprung disease?

Answer 18

Enterocolitis, encopresis, constipation, incontinence

Poor if not adequately managed. Enterocolitis mortality 50% if not treated. Postoperatively good outcome but majority will retain intestinal problems.

Question 19

What is Inflammatory bowel disease?

Answer 19

Inflammatory chronic idiopathic condition of the bowel encompassing two related but distinct syndromes: UC and CD.

Question 20

What is the background of Inflammatory bowel disease?

Answer 20

Unknown, thought to be genetic and environmental factors. Genetic component CD>UC. Smoking increases risk of CD but decreases risk of UC.

UC: Diffuse mucosal inflammation of rectum extending proximally, variable length. Subdivision into distal, and extensive diseases.

CD: Patchy transmural inflammation characterized by skip lesions. Defined by anatomical location or pattern of disease (inflammatory, fistulaitng or structuring). Montreal classification.

Intermediate colitis (IC) 10% of children unclassifiable.

Epidemiology
Fhx , smoking, 5/100k. 60% CD, 30% UC, 10% IC. Usual diagnosis teenager or late child.

Question 21

What would you find in history exam of Inflammatory bowel disease?

Answer 21

General: UC has remission and exacerbations, skin manifestations rare, typically bloating, diahrrea and pain. CD is more heterogenous and non specific, triad of diahrrea/abdominal pain/weight loss.

Abdo pain C>U, Diahrrea U>C, rectal bleed U>C, wt loss C>U, lethargy C>U.

Signs o/e: anal fistulae, apthrous ulcers in mouth, clubbing, delayed puberty, perianal abscesses, erythema nodosum or rash, liver disease, toxic megacolon.

Question 22

What investigations would you do for Inflammatory bowel disease?

Answer 22

General: ESPHGAN IBD workup is consensus protocol

Bloods: low Hb, high ESR/CRP, low B12/folate. LFT (may need ERCP/USS if abnormal due to PSC risk).

Specific: limited use of pANCA with UC, ASCA with CD, low sensitivity.

Microbiology: stool sample, C diff toxins.

Radiology: AXR strictures fistulae, small bowel follow through, Tc white cell stain

Endoscopy: eileocolonoscopy and upper GI endoscopy with histology of multiple biopsies from all segments.

Question 23

What is the management of Inflammatory bowel disease?

Answer 23

CD: Exclusive enteral liquid nutrition, corticosteroids, Abx (perianal abscess), aminosalycilates (mesalazine/sulphalazine), budesonide, IV steroids. Second line: azathriopine, parenteral nutrition. Third line: infliximab and surgery (strictures or fistulae). Remission maintenance requires AZA/6MCP.

UC: Induction of remission requires ASA/SLZ if mild and corticosteroids if severe. Maintenance: ASA/MSLZ. Surgery with acute toxic megacolon required.

Question 24

What are the complications and prognosis of Inflammatory bowel disease?

Answer 24

UC: toxic megacolon, perforation, CRC, PSC

CD: Megaloblastic anaemia, gallstones, perianal disease, strictures, obstruction.

Good with early detection and treatment, mortality highest in 2y post diagnosis.

Question 25

What is Intussusception?

Answer 25

Invagination or telescoping of part of the intestine (instussuscepian) into another part of the intestine (intussusceptum).

Question 26

What is the background of Intussusception?

Answer 26

4/1k. 60%<1y, very rare over 2y.

90% idiopathic. 10% identified lead point – Peyers patches after a viral illness, lymph nodes, Meckels diverticulum, adhesions from recent surgery, appendix stump. Related to HSP and FAP.

Most common site of invagination is the terminal ileum into the caecum. Mesenteric constriction -> VR obstruction -> engorgement -> press out arterial supply -> more contraction due to acidosis -> engorgement and odema –> intestinal infarction

Question 27

What would you find in history exam of Intussusception?

Answer 27

Triad of symptoms: vomiting, colic pain, and PR bleeding.

Vomiting is non bilious and then becomes bilious once total obstruction occurs. Pain episodes are severe with leg pulling etc. Redcurrant jelly stools.

Question 28

What investigations would you do for Intussusception?

Answer 28

Bloods (CRP, UE, FBC, GS).

AXR: dilated bowel before site, crescent sign (curvilinear mass on the right, in transverse colon before hepatic flexure).

USS: confirmatory investigation -> donut or target sign.

Question 29

What is the management of Intussusception?

Answer 29

Urgent care: IV access, fluid resusc, NGT, IV Abx, confirmation with USS.

Therapeutic enemaL air, water or contrast enema for reduction. Three attempts then stopped due to risk of perforation.

Surgical: if therapeutic enema fails (in long standing IS) laproscopic or open technique, Right lower transverse incision.

Question 30

What are the complications and prognosis of Intussusception?

Answer 30

Prolonged can lead to shick, perforation, peritonitis.

Highest recurrence risk in the first 24h.