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GI VII: Hernias, Congenital & Metabolic disorders Cheat Sheet by

Gastroenterology
hernia     pyloric     stenosis     atresia     megacolon

Hernias

Definition
A protrusion of an organ or structure through the wall that normally contains it. Can entrap the intestines and cause intestinal blockage
Umbilical hernia
Usually congen­ital, appears at birth. Many resolve on their own, but might need surgery.
Diaphr­agmatic or hiatal hernia
Protrusion of stomach through the diaphragm via the esophageal hiatus. Can cause GERD. Acid suppre­ssion might help, but can do surgery.
Incisional hernia
Associated w/ vertical incisions, especially in obese pts. or if they have a wound infx.
Indirect inguinal hernia
More common, passage of intestine through the internal inguinal ring down the inguinal canal. May pass into the scrotum.
Direct inguinal hernia
Passage of intestine through the external inguinal ring at Hessel­bach's triangle. Rarely enters scrotum.
Femoral inguinal hernia
Least common. Passes through the femoral ring.
Treatment (all hernias)
Surgical

Lactose Intole­rance (metab­olic)

General
Lactose normally digested by lactase, which is produced in the SI. For a lot of the world's popula­tion, lactase production doesn't continue after age 12 --> lactose products not digested
Symptoms
Nausea, bloating, flatul­ence, diarrhea, cramping, vomiting
Management
Avoid dairy. Use lactase enzyme tablets.
 

Esophageal Atresia (conge­nital)

Commonly associated with
trache­oes­oph­ageal fistulae
Clinical presen­tation in newborns
Excessive saliva and chokin­g/c­oughing during feeding attempts
Diagnosis
Inability to pass an NG tube
Treatment
Surgical (use suction and withho­lding of oral feedings meanwhile to prevent pulmonary aspira­tion)

Pyloric Stenosis (conge­nital)

Definition
The gastric outlet is obstructed by pyloric hypert­rophy, M>>F
Clinical Features
Progr­essive, non-bi­lious projectile vomiting in a child who remains hungry, 4-6 weeks old. Weight loss + dehydr­ation common. Might feel an olive-­shaped mass adjacent to umbilicus after vomiting.
Lab Findings
U/S, barium swallow will show "­str­ing­-si­gn"
Treatment
Surgery

Phenyl­ket­onuria (metab­olic)

Definition
Rare AR inability to metabolize the protein phenyl­alanine
Conseq­uences
Phenyl­alanyne and its metabo­lites accumulate in the CNS --> causing MR and movement disorders
How is it detect­ed/­managed early on?
Screening at birth
Conseq­uences if not detected by age 3?
Irreve­rsible brain damage
Management
Low-ph­eny­lal­anine diet + tyrosine supple­ment. Strict protein intake for life.
 

Diaphr­agnatic hernia (conge­nital)

Clinical presen­tation in newborn
Immediate respir­atory distress *the affected lung is compressed by pressure from abdominal contents)
Diagnosis
If bowel sounds are heard in the chest
Radiog­raphy
Shows loops of bowel in the involved hemithorax + displa­cement of heart and medias­tinal structures
Treatment
Surgery

Bowel Atresia (conge­nital)

- Occurs in ileum mosts commonly
- Presents with signs of obstru­ction in first few days of life

Hirsch­spr­ung's disease

AKA
Congenital megacolon
Definition
Congenital absence of Meissner's and Auerbach's autonomic plexuses enervating the bowel wall
Symptoms
Consti­pation, obstip­ation, vomiting, failure to thrive
Treatment
Surgical resection of affected bowel

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