PAINE #PANCE Pearl – Gastrointestinal



Question

Cirrhosis and intrinsic liver disease can cause a significant increase in pressure within the portal system and result in dilation of the caval tributaries. What are the three (3) most common locations these dilations occurs?


Answer

The three (3) most common locations where you can find dilation of portal caval tributaries as a result of portal hypertension are:

  • Esophageal veins
    • Causing esophageal varices
  • Periumbilical veins
    • Causing caput medusae
  • Inferior and middle rectal veins
    • Causing hemorrhoids

Ep-PAINE-nym



Mallory-Weiss Syndrome

Other Known Aliasesgastro-esophageal laceration syndrome

Definitionlongitudinal mucosal lacerations in the distal esophagus and proximal stomach often leading to bleeding from submucosal arteries

Clinical Significance These tears often occurs as a result of forceful vomiting and can present with hematemesis or melena. Risk factors include alcohol abuse, hiatal hernias, and bulemia. In contrast to Boerhaave’s syndrome, this only involves the mucosa and submucosa and therefor, is not a full thickness rupture. Diagnosis is made via endoscopy and treatment depends on how active the bleed is at the time of endoscopy.

HistoryNamed after two physicians, George Kenneth Mallory (1900-1986) and Soma Weiss (1898-1942), from Boston. Dr. Mallory received his medical doctorate from Harvard Medical School in 1926 and followed in his father’s footsteps by working at the Mallory Institute of Pathology at Boston City Hospital. Dr. Weiss studied physiology and biochemistry in Budapest before immigrating to the United States immediately after World War I, when he wualified in medicine in 1923. He started his career at Cornell before moving to Harvard Medical School, and finally becoming physician-in-chief and professor at The Brigham Hospital in 1939. They partnered and co-authored the manuscript with their eponymous name in 1929, where they described 15 cases of severe, painless hemorrhage of the esophagus preceded by vomiting in alcoholics. They followed this up in 1932 with an additional 6 cases.


References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Mallory GK, Weiss A. Hemorrhages from lacerations of the cardiac orifice of the stomach due to vomiting. American Journal of the Medical Sciences. 1929; 178: 506-15
  7. Weiss S, Mallory GK. Lesions of the cardiac orifice of the stomach produced by vomiting. Journal of the American Medical Association; 1932, 98: 1353-1355

#52 – Pyloric Stenosis



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Epidemiology

  • 2-4 per 1000 live births worldwide and 20 per 10,000 live births in the US
  • Higher male to female ratio (4-6:1)
  • Higher incidence (1.5x) in first-born children
  • Highest incidence in caucasian infants
  • Less common in infants of older mothers

Risk Factors and Etiology

The exact mechanisms and etiologies are unclear, but it is hypothesized that it is multifactorial and is a result of both genetic predisposition and environmental triggers.

  • Environmental Factors
    • Maternal smoking (up to 2x increased risk)
    • Bottle feeding
      • Bottle feeding during first 4 months increased risk by 4x
        • Didn’t delineate formula vs breastmilk
  • Genetic Factors
    • Reports of familial aggregation, but there is no clear research association
    • Apolipoprotein A1 (APOA1) gene cluster
      • Hypothesized low plasma cholesterol at birth and increased risk
  • Macrolide Antibiotics
    • Increased risk if given to infants < 2 weeks old
      • Treatment/prophylaxis for pertussis
    • Association with maternal use during first two weeks of life
  • Icteropyloric syndrome
    • Unconjugated hyperbilirubinemia
      • Most commonly from early Gilbert’s Syndrome

History and Physical Examination

  • Class presentation for PANCE
    • < 6 week old with post-prandial, non-bilious projectile vomiting around 10 minutes after cessation of feeding
    • Ravenous feeder even after vomiting
  • May be emaciated and/or dehydrated
    • Though we are diagnosing earlier and infants tend to be healthier
  • Palpable mass in the epigastrium (50-90%)
    • This is also less commonly seen due to healthier infants and ease of obtaining radiologic students
      • 73% in the 1970s to only 30% now
    • Ideally, immediately after vomiting and while the infant is calm
  • Other important assessments
    • Height/weight
    • Mucous membranes and skin turgor
    • Skin and sclerae
    • Genitalia
      • Ambiguous genitalia raises suspicion for congenital adrenal hyperplasia and adrenal crisis

Diagnostic Studies

  • Laboratory
    • Hypochloremic metabolic alkalosis
      • 88% PPV if pH > 7.45, chloride < 98, and base excess > +3
    • Assess for dehydration
      • BUN/creatinine > 20:1
    • Liver Function Tests
      • Bilirubin breakdown, AST/ALT, GGT, and ALP
  • Radiography
    • Ultrasound is the test of choice
      • Accuracy is operator dependent, but can reach > 95% sensitivity/specificity
      • “Target” sign on transverse view
      • Normal Measurements (vary with age and used together)
        • Pyloric Muscle Thickness
          • < 3mm
        • Pyloric Muscle Length
          • < 14mm
        • Pyloric Channel Length
          • < 16mm
Target Sign on Transverse View
  • Fluoroscopic Upper Gastrointestinal Series
    • Used if ultrasound is nondiagnostic
    • Classic findings :
      • “string” sign from an elongated pyloric canal
      • “double-track” sign from two thin tracks of barium along the pyloric canal created by compressed pyloric mucosa
      • “beak”sign from a tapered point at the pyloric ending
      • “shoulder” sign from a prepyloric bulge of barium
1) Beak Sign, 2) String Sign, 3) Double Track Sign, 4) Shoulder Sign

Differential Diagnosis

Although pyloric stenosis has a classic presentation, you must entertain the other important causes of vomiting in infancy.


Treatment

  • Definitive management is surgery
  • Timing of surgery depends on the clinical status of the infant
    • If healthy, surgery can be performed on the day of diagnosis
    • If ill, then resuscitation and feeding need to be performed to limit perioperative complications.
  • Technique
    • Ramstedt Pyloromyotomy
      • Longitudinal incision of the pylorus with blunt dissection down to the submucosa
  • Open vs Laparoscopic
    • No difference in operating time, time to full feeding, or length of stay
    • Laparoscopic had lower incidence of emesis and better pain control, but higher incidence of incomplete surgical release
  • Postoperative Management
    • Feeding
      • Resumed within a few hours after surgery
      • Regurgitation is common, but should not delay/stop feedings
    • Breathing
      • Monitor for apnea at least for 24 hours
    • Complications
      • Mucosal perforation (rare)

Follow-Up

  • Surgery is curative in the majority of patients
  • Once normal feeds occur, only routine pediatric care and follow-up is needed
  • Reflux is common and managed conservatively

The Cottage Physician (1893)



References

  1. Kapoor R, Kancherla V, Cao Y, et al. Prevalence and descriptive epidemiology of infantile hypertrophic pyloric stenosis in the United States: A multistate, population-based retrospective study, 1999-2010. Birth defects research. 2019; 111(3):159-169. [pubmed]
  2. To T, Wajja A, Wales PW, Langer JC. Population demographic indicators associated with incidence of pyloric stenosis. Archives of pediatrics & adolescent medicine. 2005; 159(6):520-5. [pubmed]
  3. Krogh C, Fischer TK, Skotte L, et al. Familial aggregation and heritability of pyloric stenosis. JAMA. 2010; 303(23):2393-9. [pubmed]
  4. Krogh C, Gørtz S, Wohlfahrt J, Biggar RJ, Melbye M, Fischer TK. Pre- and perinatal risk factors for pyloric stenosis and their influence on the male predominance. American journal of epidemiology. 2012; 176(1):24-31. [pubmed]
  5. Svenningsson A, Svensson T, Akre O, Nordenskjöld A. Maternal and pregnancy characteristics and risk of infantile hypertrophic pyloric stenosis. Journal of pediatric surgery. 2014; 49(8):1226-31. [pubmed]
  6. Zhu J, Zhu T, Lin Z, Qu Y, Mu D. Perinatal risk factors for infantile hypertrophic pyloric stenosis: A meta-analysis. Journal of pediatric surgery. 2017; 52(9):1389-1397. [pubmed]
  7. McAteer JP, Ledbetter DJ, Goldin AB. Role of bottle feeding in the etiology of hypertrophic pyloric stenosis. JAMA pediatrics. 2013; 167(12):1143-9. [pubmed]
  8. Sørensen HT, Nørgård B, Pedersen L, Larsen H, Johnsen SP. Maternal smoking and risk of hypertrophic infantile pyloric stenosis: 10 year population based cohort study. BMJ (Clinical research ed.). 2002; 325(7371):1011-2. [pubmed]
  9. Feenstra B, Geller F, Carstensen L, et al. Plasma lipids, genetic variants near APOA1, and the risk of infantile hypertrophic pyloric stenosis. JAMA. 2013; 310(7):714-21. [pubmed]
  10. Eberly MD, Eide MB, Thompson JL, Nylund CM. Azithromycin in early infancy and pyloric stenosis. Pediatrics. 2015; 135(3):483-8. [pubmed]
  11. Honein MA, Paulozzi LJ, Himelright IM, et al. Infantile hypertrophic pyloric stenosis after pertussis prophylaxis with erythromcyin: a case review and cohort study. Lancet (London, England). ; 354(9196):2101-5. [pubmed]
  12. Lund M, Pasternak B, Davidsen RB, et al. Use of macrolides in mother and child and risk of infantile hypertrophic pyloric stenosis: nationwide cohort study. BMJ (Clinical research ed.). 2014; 348:g1908. [pubmed]
  13. Touloukian RJ, Higgins E. The spectrum of serum electrolytes in hypertrophic pyloric stenosis. Journal of pediatric surgery. 1983; 18(4):394-7. [pubmed]
  14. Bakal U, Sarac M, Aydin M, Tartar T, Kazez A. Recent changes in the features of hypertrophic pyloric stenosis. Pediatrics international : official journal of the Japan Pediatric Society. 2016; 58(5):369-71. [pubmed]
  15. Sivitz AB, Tejani C, Cohen SG. Evaluation of hypertrophic pyloric stenosis by pediatric emergency physician sonography. Academic emergency medicine : official journal of the Society for Academic Emergency Medicine. 2013; 20(7):646-51. [pubmed]
  16. Niedzielski J, Kobielski A, Sokal J, Krakós M. Accuracy of sonographic criteria in the decision for surgical treatment in infantile hypertrophic pyloric stenosis. Archives of medical science : AMS. 2011; 7(3):508-11. [pubmed]
  17. Hernanz-Schulman M. Pyloric stenosis: role of imaging. Pediatric radiology. 2009; 39 Suppl 2:S134-9. [pubmed]
  18. Said M, Shaul DB, Fujimoto M, Radner G, Sydorak RM, Applebaum H. Ultrasound measurements in hypertrophic pyloric stenosis: don’t let the numbers fool you. The Permanente journal. 2012; 16(3):25-7. [pubmed]
  19. Iqbal CW, Rivard DC, Mortellaro VE, Sharp SW, St Peter SD. Evaluation of ultrasonographic parameters in the diagnosis of pyloric stenosis relative to patient age and size. Journal of pediatric surgery. 2012; 47(8):1542-7. [pubmed]
  20. St Peter SD, Holcomb GW, Calkins CM, et al. Open versus laparoscopic pyloromyotomy for pyloric stenosis: a prospective, randomized trial. Annals of surgery. 2006; 244(3):363-70. [pubmed]
  21. Hall NJ, Pacilli M, Eaton S, et al. Recovery after open versus laparoscopic pyloromyotomy for pyloric stenosis: a double-blind multicentre randomised controlled trial. Lancet (London, England). 2009; 373(9661):390-8. [pubmed]

Ep-PAINE-nym



Boerhaave’s Syndrome

Other Known Aliasesnone

Definitionesophageal rupture from forceful vomiting

Clinical Significance This rupture occurs as a result of sudden increased intraesophageal pressure with negative intrathoracic pressure and the associated tear is most commonly located on the left posterolateral aspect of the distal esophagus. Gastric contents then invade the mediastinum and cause mediastinitis, necrosis, and severe bacterial infection that can lead to significant mortality.

HistoryNamed after Herman Boerhaave (1668-1738), who was a Dutch physician and earned his medical doctorate from the University of Harderwijk in 1693. He went on to earn a professorship at the University of Leiden in 1701, speaking on medicine, anatomy, botany, chemistry, and philosophy. It was during his time here that his repuatation as a skilled orator and teacher made international waves and physicians from all over the world came to listen and learn. He first described his eponymous disease in 1724 after witnessing the death of Baron Jan van Wassenaer after a retching following a particularly gluttonous feast.


References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Boerhaave H. Atrocis, nec descripti prius, morbii historia: secundum medicae artis leges conscripta. Leiden, the Netherlands: Lugduni Batavorum Boutesteniana, 1724
  7. Underwood EA. Boerhaave after three hundred years. British medical journal. 1968; 4(5634):820-5. [pubmed]

PAINE #PANCE Pearl – Gastrointestinal



Question

Ischemic bowel disease has two classic presentations: acute and chronic.

  1. What is the typical type of patient (or risk factors) for each?
  2. How does each type classically present?


Answer

  1. The risk factors for each type are a little different, but very important in differentiating the causes of abdominal pain:
    • Acute (from embolism)
      • atrial fibrillation, hypercoaguable states, instrumentation, valvular disease, and ventricular aneurysms
    • Chronic (from decreased perfusion)
      • peripheral vascular disease, atherosclerosis
  2. The presentations for both are also vastly different:
    • Acute
      • sudden onset of severe, periumbilical abdominal pain that is out of proportion to physical examination
      • bloody stools may also occur
    • Chronic
      • post-prandial intestinal pain due to inability to meet the blood flow demands for digestion
      • patients may also lose weight due to “food fear”

Ep-PAINE-nym



Meckel’s Diverticulum

Other Known Aliasesnone

DefinitionVestigial remnant of the omphalomesenteric (vitiline) duct

Clinical Significance It is the most common malformation in the GI tract and is mainly asymptomatic.  When symptoms do occur, it commonly presents as painless, rectal bleeding in children.  The “Rule of 2s” will help you remember the facts of this pathology:

  • Effects 2% of the population
  • 2% of these will be symptomatic by age 2
  • 2 types of heterotopic tissue
  • Boy-to-girl ratio is 2:1
  • Usually 2″ in length
  • 2′ from the ileocecal valve

HistoryNamed after Johann Friedrich Meckel, the Younger (1781-1833), who was a German anatomist and received his medical doctorate from the University of Halle in 1802. He then went on to become full professor of anatomy, surgery, and obstetrics at the University of Halle in 1808 after studying Würzburg, Vienna, and Paris. He made tremendous advancements in the area of anatomy and embryonic development with special attention to birth defects and abnormalities, where he pioneered the early study of teratology. He first published his eponymous finding in 1809 in an article entitled “Über die Divertikel am Darmkanal” in the Halle Archives of Physiology. Of note, he comes from a prestigous medical family, where both his father, grandfather, and brother were physicians….hence the surname “the Younger”.


References

  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Stallion A, Shuck JM.  Meckel’s Diverticulum.  Surgical Treatment: Evidence-Based and Problem-Oriented.  2001 [pubmed]
  7. Blackbourne LH.  Surgical Recall.  6th ed. 2012
  8. J. F. Meckel. Über die Divertikel am Darmkanal. Archiv für die Physiologie, Halle, 1809, 9: 421–453
  9. Klunker R, Göbbel L, Musil A, Tönnies H, Schultka R. Johann Friedrich Meckel the Younger (1781-1833) and modern teratology. Annals of Anatomy. 2002; 184(6):535-40. [pubmed]