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Infantile Hypertrophic Pyloric Stenosis
a report of 3 cases of
false positives in the sonographic diagnoses
高雄市立聯合醫院 小兒外科 李振豐
Case 1
 Age : 2m10d/o Sex: female
 B.H. : G2P2, NSD, full-term, BBW 2600 grams
 PH : Hyperbilirubinemia and phototherapy at our
pediatric ward at age of 7days
 P.I. : Easy postprandial vomiting (non-bilious)
since 20 d/o, persistent postprandial
vomiting in spite of medical treatment,
ultrasonographic diagnosis of infantile
hypertrophic pyloric stenosis (twice) at
OPD, and pediatric surgical consultation
was requested after admission.
Plain Abdomen
Abdominal ultrasonography (1st)(34d/o)
Pylorus - longitudinal axis : 20.4 mm
short axis : 9.6 mm
muscle thickness : 4.3 mm
Abdominal ultrasonography (2nd)(40d/o)
Pylorus – longitudinal axis : 21.0 mm
muscle thickness : 4.3 mm
Laboratory tests
CBC & DC
Biochemistry
Blood gas
P.E. on Consultation
MD and MN
Consc : alert
Conj : not anemic
Sclera : not icteric
Neck : supple, no LAP
Chest : symmetric
H.S.: no murmur
B.S.: no rale
Abdomen : soft and flat
bowel sound: present
*visible gastric peristaltic wave : negative
*palpable olive mass : negative
Upper GI series (by the pediatric surgeon)
no Pyloric Stenosis, Gastroesophageal reflux
Case 2
 Age : 1m5d/o Sex : male
 B.H. : G1P1, C/S, GA 36 weeks, BBW 2640 g
 P.H. : n.p.
 PI : progressive postprandial vomiting for 3-4
days, suspected infantile hypertrophic
pyloric stenosis by abdominal
ultrasonography at OPD, pediatric surgical
consultation was requested after admission
Abdominal ultrasonography
Pylorus - longitudinal axis : 21.9 mm
short axis : 15.2 mm
muscle thickness : 5.13 - 5.53 mm
Laboratory tests
CBC & DC
Biochemistry
Blood gas
Plain Abdomen
P.E. on Consultation
MD and MN, BW 3514 grams
Consc: alert
Conj: not anemic
Sclera: not icteric
Conj: not icteric
Neck: supple, no LAP
Chest: symmetric
H.S.: no murmur
B.S.: no rale
Abd: soft, slightly distended
hyperactive bowel sound
*no visible gastric peristaltic wave
*no palpable olive mass
Upper GI series
gastroesophageal reflux
Case 3
 Age : 29 d/o Sex : male
 B.H. : NSD, full-term, BBW 2635 g (Pojen H.)
 P.H. : admission at KCGMH for frequent
postprandial vomiting (9 d/o – 29 d/o)
 P.I. : 1. for 自費托嬰
2. still easy postprandial vomiting
3. BW 2965 grams
Abdominal ultrasonography (1st)(45d/o)
Pylorus – longitudinal axis : 2.13 cm
muscle thickness : 0.37 cm
Sonographic diagnosis : IHPS can not be ruled out
Suggestion : upper GI if symptom persists
Abdominal ultrasonography (2nd)(56d/o)
Pylorus – muscle thickness : 0.408 cm
Sonographic diagnosis: IHPS can not ruled out
Suggestion : UGI series if symptom persists
Abdominal ultrasonography (3rd)(66d/o)
Pylorus – longitudinal axis : 1.78 cm
muscle thickness : 0.388cm
Sonographic diagnosis : IHPS can not be ruled out
Pediatric surgery consultation (70d/o)
 C.C. : postprandial vomiting
watery diarrhea with diaper dermatitis and perineal
skin erosion
 B.W. : formula feeding, 150ml / 5hrs
B.W.- 2635 g  5.5 kg
 P.E.: distended abdomen with hyperactive bowel sound
anorectal digital examination – anal stenosis
 DX : 1. GER
2. anal stenosis
 RX : 1. daily anal dilatation for 4-6 weeks
2. conservative treatment, i.e. small frequent feedings,
position change, and medication as necessary
Abdominal ultrasonography (4th)(84d/o)
Pylorus – longitudinal axis : 1.54 cm
muscle thickness : 0.349 cm
Sonographic diagnosis : IHPS cannot be ruled out
Upper GI series (86d/o)
Infantile Hypertrophic Pyloric Stenosis
 Incidence
- 2-4 in 1000 Caucasian
- fewer than 1 in 1000 Asian and African
 Male preponderance
- between 4:1 and 10:1
- firstborn males are accounting for 40-60%
 Age of presentation
- most commonly 3-6th week of age
- (as early as 1st week, and as late as 5th month)
 Family history
- 15-20 folds increase in risk
Clinical Presentation
 Clinical triad
- Projectile vomiting (non-bilious)
- Visible gastric peristaltic wave
- Palpable pyloric (olive) tumor (50-90%)
 Hypochloremic hypokalemic metabolic alkalosis
(serum pH >7.45, chloride <98, and base excess >+3)
 Dehydration
 Constipation
 Coffee-ground flecks if 2° gastritis (15-20%)
 Jaundice (3-5%)(↓ hepatic glucuronyl transferase)
Diagonosis of IHPS
 Clinical diagnosis: 60-90%
History, Lab, S/S,
palpable olive mass (pathognomonic)
 Plain film & barium upper GI series
 Ultrasonography - the gold standard imaging
 Over the past 30 years, increased reliance on
imaging has decreased clinical diagnosis from
up to 90% of cases to as little as 23%.
(J. Paediatric and Child Health 49 (2013) 33–37)
Plain Abdomen in IHPS
Gastric Peristaltic Wave
Note : On NG tube and empty the stomach of content
and then Inflation of air into stomach
Palpable pyloric tumor (olive mass)
Note: On NG tube and empty the stomach of content
and left NG open
and bottle feeding of water
https://survivinginfantsurgery.wordpress.com
test feed
www.diagnostic imaging.com
Upper GI series
string sign
double track sign
shoulder sign
mushroom sign
Diagnostic Criteria
of Ultrasonography
 Pyloric muscle
thickness > 3-4mm
 pyloric muscle
length > 15-20mm
 pyloric diameter
> 10-14mm
Both a high sensitivity (90-99%)
and high specificity (97-100%).
 False positive:
pyloric spasm
 False negative:
prematurity or early disease
ATOTW 276 – Infantile Hypertrophic Pyloric Stenosis,
26th November 2012
Diagnostic ultrasound Fifth Edition Copyright © 2018 by Elsevier, Inc.
Pitfalls in Sonographic Diagnosis
Pitfalls in Sonographic Diagnosis
Diagnostic ultrasound Fifth Edition Copyright © 2018 by Elsevier, Inc.
Treatment of IHPS
 Definitive treatment : Surgery (both safe and effective)
- Ramstedt pyloromyotomy (since 1911 ~)
open vs laparoscopic
 Other approaches (not recommended)
- Atropine, iv or oral (relaxation of pyloric muscle)
- Conservative treatment
(including a trial of continuous nasoduodenal tube feedings)
- Balloon dilatation (endoscopically guided)
https://survivinginfantsurgery.wordpress.com
Preoperative preparation
Preoperative treatment → correcting fluid deficit (dehydration),
acid-base and electrolyte imbalances.
 Normal electrolytes with mild or no dehydration
- as usual
 Moderate or severe dehydration
-*surgery delayed and intensive electrolyte and fluid therapy
**• pH ≤7.45 and/or base excess ≤3.5
• Bicarbonate <26 mEq/L
***• Sodium ≥132 mEq/L
***• Potassium ≥3.5 mEq/L
• Chloride ≥100 mEq/L
• Glucose ≥72 mg/dL (4.0 mmol/L)
* : IHPS is a medical emergency, not a surgical emergency
** : correction of alkalosis is essentially to prevent postoperative apnea
*** : severe hypokalemia (serum potassium <2.5 mEq/L) or hyponatremia (serum
sodium <120 mEq/L) are at high risk of postoperative complications.
Ramstedt pyloromyotomy (open)
Incisions for pyloromyotomy
Minimally invasive surgery
Circumumbilical incision
(supraumbilical)
Surgery 136:827-32, 2004
Laparoscpic pyloromyotomy
Complications
 Mucosal perforation
- incidence : 2%, ususally duodenal end.
(<1% unrecognized intraoperatively)
- treatment : close mucosa and reinforced with omental patch
NG tube decompression & NPO for 48 hrs
 Incomplete pyloromyotomy
- rare (0-7%) → re-operation
 Others
- SSI : 4%
- postoperative emesis (44%): transient mucosal edema, GER,
gastric atony.
(incomplete pyloromyotomy).
Misdiagnosis and Negative Laparotomy
 The rate of negative laparotomy was rarely reported (1~4%).
 Reliance upon the ultrasound appearance of the pylorus
without taking into account other important diagnostic
evidence will increase the risk of false-positive diagnoses
and unnecessary laparotomy. (Pyloric Stenosis: Is Over-Reliance on
Ultrasound Scans Leading to Negative Explorations? Eur J Pediatr Surg 1997, 7(6):328-30.)
 If there is no palpable ‘tumor’ on a test feed, criteria for
surgery should include a palpable pyloric mass on
examination under anesthesia (EUA). In the absence of
palpable mass on EUA, upper GI endoscopy may be used
to demonstrate features of IHPS before proceeding with
pyloromyotomy. (Negative exploration for pyloric stenosis – Is it preventable?
BMC Pediatrics 2008, 8:37)
Thank You
1120331-小兒科聯合病例討論會.pdf

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1120331-小兒科聯合病例討論會.pdf

  • 1.
  • 2. Infantile Hypertrophic Pyloric Stenosis a report of 3 cases of false positives in the sonographic diagnoses 高雄市立聯合醫院 小兒外科 李振豐
  • 3. Case 1  Age : 2m10d/o Sex: female  B.H. : G2P2, NSD, full-term, BBW 2600 grams  PH : Hyperbilirubinemia and phototherapy at our pediatric ward at age of 7days  P.I. : Easy postprandial vomiting (non-bilious) since 20 d/o, persistent postprandial vomiting in spite of medical treatment, ultrasonographic diagnosis of infantile hypertrophic pyloric stenosis (twice) at OPD, and pediatric surgical consultation was requested after admission.
  • 5. Abdominal ultrasonography (1st)(34d/o) Pylorus - longitudinal axis : 20.4 mm short axis : 9.6 mm muscle thickness : 4.3 mm
  • 6. Abdominal ultrasonography (2nd)(40d/o) Pylorus – longitudinal axis : 21.0 mm muscle thickness : 4.3 mm
  • 7.
  • 8. Laboratory tests CBC & DC Biochemistry Blood gas
  • 9. P.E. on Consultation MD and MN Consc : alert Conj : not anemic Sclera : not icteric Neck : supple, no LAP Chest : symmetric H.S.: no murmur B.S.: no rale Abdomen : soft and flat bowel sound: present *visible gastric peristaltic wave : negative *palpable olive mass : negative
  • 10. Upper GI series (by the pediatric surgeon) no Pyloric Stenosis, Gastroesophageal reflux
  • 11.
  • 12. Case 2  Age : 1m5d/o Sex : male  B.H. : G1P1, C/S, GA 36 weeks, BBW 2640 g  P.H. : n.p.  PI : progressive postprandial vomiting for 3-4 days, suspected infantile hypertrophic pyloric stenosis by abdominal ultrasonography at OPD, pediatric surgical consultation was requested after admission
  • 13. Abdominal ultrasonography Pylorus - longitudinal axis : 21.9 mm short axis : 15.2 mm muscle thickness : 5.13 - 5.53 mm
  • 14. Laboratory tests CBC & DC Biochemistry Blood gas
  • 16. P.E. on Consultation MD and MN, BW 3514 grams Consc: alert Conj: not anemic Sclera: not icteric Conj: not icteric Neck: supple, no LAP Chest: symmetric H.S.: no murmur B.S.: no rale Abd: soft, slightly distended hyperactive bowel sound *no visible gastric peristaltic wave *no palpable olive mass
  • 18.
  • 19. Case 3  Age : 29 d/o Sex : male  B.H. : NSD, full-term, BBW 2635 g (Pojen H.)  P.H. : admission at KCGMH for frequent postprandial vomiting (9 d/o – 29 d/o)  P.I. : 1. for 自費托嬰 2. still easy postprandial vomiting 3. BW 2965 grams
  • 20. Abdominal ultrasonography (1st)(45d/o) Pylorus – longitudinal axis : 2.13 cm muscle thickness : 0.37 cm Sonographic diagnosis : IHPS can not be ruled out Suggestion : upper GI if symptom persists
  • 21. Abdominal ultrasonography (2nd)(56d/o) Pylorus – muscle thickness : 0.408 cm Sonographic diagnosis: IHPS can not ruled out Suggestion : UGI series if symptom persists
  • 22. Abdominal ultrasonography (3rd)(66d/o) Pylorus – longitudinal axis : 1.78 cm muscle thickness : 0.388cm Sonographic diagnosis : IHPS can not be ruled out
  • 23. Pediatric surgery consultation (70d/o)  C.C. : postprandial vomiting watery diarrhea with diaper dermatitis and perineal skin erosion  B.W. : formula feeding, 150ml / 5hrs B.W.- 2635 g  5.5 kg  P.E.: distended abdomen with hyperactive bowel sound anorectal digital examination – anal stenosis  DX : 1. GER 2. anal stenosis  RX : 1. daily anal dilatation for 4-6 weeks 2. conservative treatment, i.e. small frequent feedings, position change, and medication as necessary
  • 24. Abdominal ultrasonography (4th)(84d/o) Pylorus – longitudinal axis : 1.54 cm muscle thickness : 0.349 cm Sonographic diagnosis : IHPS cannot be ruled out
  • 25. Upper GI series (86d/o)
  • 26.
  • 27. Infantile Hypertrophic Pyloric Stenosis  Incidence - 2-4 in 1000 Caucasian - fewer than 1 in 1000 Asian and African  Male preponderance - between 4:1 and 10:1 - firstborn males are accounting for 40-60%  Age of presentation - most commonly 3-6th week of age - (as early as 1st week, and as late as 5th month)  Family history - 15-20 folds increase in risk
  • 28. Clinical Presentation  Clinical triad - Projectile vomiting (non-bilious) - Visible gastric peristaltic wave - Palpable pyloric (olive) tumor (50-90%)  Hypochloremic hypokalemic metabolic alkalosis (serum pH >7.45, chloride <98, and base excess >+3)  Dehydration  Constipation  Coffee-ground flecks if 2° gastritis (15-20%)  Jaundice (3-5%)(↓ hepatic glucuronyl transferase)
  • 29.
  • 30.
  • 31. Diagonosis of IHPS  Clinical diagnosis: 60-90% History, Lab, S/S, palpable olive mass (pathognomonic)  Plain film & barium upper GI series  Ultrasonography - the gold standard imaging  Over the past 30 years, increased reliance on imaging has decreased clinical diagnosis from up to 90% of cases to as little as 23%. (J. Paediatric and Child Health 49 (2013) 33–37)
  • 33. Gastric Peristaltic Wave Note : On NG tube and empty the stomach of content and then Inflation of air into stomach
  • 34. Palpable pyloric tumor (olive mass) Note: On NG tube and empty the stomach of content and left NG open and bottle feeding of water https://survivinginfantsurgery.wordpress.com test feed
  • 36. Upper GI series string sign double track sign shoulder sign mushroom sign
  • 37. Diagnostic Criteria of Ultrasonography  Pyloric muscle thickness > 3-4mm  pyloric muscle length > 15-20mm  pyloric diameter > 10-14mm Both a high sensitivity (90-99%) and high specificity (97-100%).  False positive: pyloric spasm  False negative: prematurity or early disease ATOTW 276 – Infantile Hypertrophic Pyloric Stenosis, 26th November 2012
  • 38.
  • 39. Diagnostic ultrasound Fifth Edition Copyright © 2018 by Elsevier, Inc. Pitfalls in Sonographic Diagnosis
  • 40. Pitfalls in Sonographic Diagnosis Diagnostic ultrasound Fifth Edition Copyright © 2018 by Elsevier, Inc.
  • 41.
  • 42.
  • 43. Treatment of IHPS  Definitive treatment : Surgery (both safe and effective) - Ramstedt pyloromyotomy (since 1911 ~) open vs laparoscopic  Other approaches (not recommended) - Atropine, iv or oral (relaxation of pyloric muscle) - Conservative treatment (including a trial of continuous nasoduodenal tube feedings) - Balloon dilatation (endoscopically guided) https://survivinginfantsurgery.wordpress.com
  • 44. Preoperative preparation Preoperative treatment → correcting fluid deficit (dehydration), acid-base and electrolyte imbalances.  Normal electrolytes with mild or no dehydration - as usual  Moderate or severe dehydration -*surgery delayed and intensive electrolyte and fluid therapy **• pH ≤7.45 and/or base excess ≤3.5 • Bicarbonate <26 mEq/L ***• Sodium ≥132 mEq/L ***• Potassium ≥3.5 mEq/L • Chloride ≥100 mEq/L • Glucose ≥72 mg/dL (4.0 mmol/L) * : IHPS is a medical emergency, not a surgical emergency ** : correction of alkalosis is essentially to prevent postoperative apnea *** : severe hypokalemia (serum potassium <2.5 mEq/L) or hyponatremia (serum sodium <120 mEq/L) are at high risk of postoperative complications.
  • 46. Incisions for pyloromyotomy Minimally invasive surgery Circumumbilical incision (supraumbilical)
  • 48. Complications  Mucosal perforation - incidence : 2%, ususally duodenal end. (<1% unrecognized intraoperatively) - treatment : close mucosa and reinforced with omental patch NG tube decompression & NPO for 48 hrs  Incomplete pyloromyotomy - rare (0-7%) → re-operation  Others - SSI : 4% - postoperative emesis (44%): transient mucosal edema, GER, gastric atony. (incomplete pyloromyotomy).
  • 49.
  • 50. Misdiagnosis and Negative Laparotomy  The rate of negative laparotomy was rarely reported (1~4%).  Reliance upon the ultrasound appearance of the pylorus without taking into account other important diagnostic evidence will increase the risk of false-positive diagnoses and unnecessary laparotomy. (Pyloric Stenosis: Is Over-Reliance on Ultrasound Scans Leading to Negative Explorations? Eur J Pediatr Surg 1997, 7(6):328-30.)  If there is no palpable ‘tumor’ on a test feed, criteria for surgery should include a palpable pyloric mass on examination under anesthesia (EUA). In the absence of palpable mass on EUA, upper GI endoscopy may be used to demonstrate features of IHPS before proceeding with pyloromyotomy. (Negative exploration for pyloric stenosis – Is it preventable? BMC Pediatrics 2008, 8:37)