Pediatric Case Management

1
Pediatric Case Management

• The Childrens Hospital at Sinai
• Joseph Wiley, MD
• Cynthia Roldan, MD
• November 29, 2005

2
November Cases-ER

• 4 month old infant with HIE with severe
  neurologic sequelae presented in respiratory
  arrest

3
November Cases-NICU

• FT infant with known congenital heart defect,
  with double outlet right ventricle (transfer)
• FT infant with Hydrops transferred for ECMO
  (mortality)
• 24 week ex premature infant with NEC (mortality)

4
Pediatric Case Management Conference

• November 29, 2005
• Julia Trintis, D.O.

5
CC H.W. 9 day old with abdominal distension
and bilious emesis

• Returning 6 days after discharge from NICU
• Passed meconium after first 24 hours
• Evaluation for abdominal distention and bilious
  emesis in FTN
• Transfer to NICU
• Rectal exam-large meconium plug evacuated

6
NICU Course

• Work up
• AXR-gaseous distention of small and large bowel
  loops opacification of rectum and sigmoid colon
• Barium Enema- no evidence of microcolon possible
  rectal web
• Sigmoidoscopy-normal-no web or stricture
• CF Studies-ordered (ultimately negative)

7
Neonatal Assessment

• FT infant with probable meconium plug syndrome.
• Differential Diagnosis Hirschsprungs Disease,
  Cystic Fibrosis, Isolated Meconium Plug
• Monitored for additional 24 hours had normal
  stools and resolution of symptoms
• Plan
• Discharge home
• Follow up with PMD, GI
• Further evaluation including rectal suction
  biopsy for recurrence of symptoms
• F/U CF Genetic studies

8
HPI

• Presentation to Pediatric Floor
• Initially doing well
• BMs occurred every feed until 1 day PTA
• Developed Poor feeding
• Decreased activity
• Watery, mucousy stool
• One episode of bilious emesis
• SHx/FHx/Allergies/Meds-non contributory

9
Physical Exam

• T36.5 HR140 RR40 BP N.O. Ox Sat 98
• General Alert, Awake, in NAD
• Abdomen hyperactive bowel sounds, distended but
  soft, no palpable masses or HSM
• Perianal exam-normally placed anus
• Rectal normal hemoccult negative no narrowing
  or explosive stool
• Neuro intact

10
Abdominal X-Ray
Abnormal Colonic Caliber
B
A
11
Hospital Course

• Normal saline enemas BID
• IV fluids
• Rectal suction biopsy
• Acetylcholinesterase stain
• Discharged home
• Readmitted for full thickness biopsy of sigmoid
  colon
• Scheduled for definitive surgery today

12
Points for Discussion

• Differential Diagnosis of Meconium Plug
• Diagnostic Methods for Hirschsprungs Disease
• Choice of biopsy Rectal Suction vs. Full
  thickness
• Anal rectal Manometry

13
Meconium Plug Syndrome

• Transient disorder of the newborn colon
  characterized by delayed passage of meconium and
  intestinal dilatation
• Epidemiology 1/500
• Incidence increased in premature infants of
  diabetic mothers and in infants whose mothers
  received magnesium sulfate
• Immaturity of myenteric plexus nerve cells or
  their hormonal receptors

14
Clinical Features-MPS

• Abdominal distention
• Failure to pass significant meconium in the first
  24 hours of life
• Bilious vomiting
• Associated with
• Cystic Fibrosis
• Hirschsprung Disease is eventually diagnosed in
  10-30

15
Hirschsprung Disease

• Aganglionic megacolon lack of intramural
  ganglionic cells
• Occurs in 15000 births
• Associated with Down syndrome
• Signs distended abdomen, palpable loops of
  bowel, rectal exam without stool in ampulla

16
Diagnostic Workup/ Dilemmas

• Abdominal X-ray
• Contrast enema- demonstrates the retained
  meconium as a filling defect or plug.
• Must done in an unprepped patient
• MPS diagnosis of exclusion enema findings in
  neonatal Hirschsprung disease can be
  indistinguishable from meconium plug syndrome
• Rectal suction biopsy-risk of perforation,
  bleeding
• Full thickness biopsy

17
Meconium Plug ObstructionRetrospective Case
Review
21 patients with Large Bowel Obstruction Relieved
by Passage of Meconium Plugs
Conclusion Essential for all babies with MP
obstruction to have HD excluded.
Burge, D. Meconium Plug obstruction. Pediatric
Surg Int(2004) 20108-110
18
Diagnosis of Hirschsprungs Disease a
prospective, comparative accuracy study of common
tests
111 Infants suspected of HD
Rectal Suction Biopsy 93 sensitive 100 specific
Contrast Enema 76 sensitive 97 specific
Anal Manometry 83 sensitive 93 specific
Conclusion Rectal Suction Biopsy is the most
accurate test for diagnosing HD, with lowest rate
of inconclusive results.

• De Lorijn, et al. Diagnosis of Hirschsprungs
  disease a prospective, comparative accuracy
  study of common tests J. Pediatrics. 2005, 146
  (6) 787-92.

19
Management

• Primary pull-through procedure
• Soave (endorectal) procedure
• Swenson procedure
• Duhamel procedure
• Early colostomy with resection of aganglionic
  segment Re-establishment of continuity

20
References

• Diament, M. Emedicine. Meconium Plug
  Syndrome. 3/05.
• Hekmatnia, Ali. Emedicine. Meconium Ileus.
  7/05.
• De Lorijn, et al. Diagnosis of Hirschsprungs
  disease a prospective, comparative accuracy
  study of common tests J. Pediatrics. 6/05, 146
  (6) 787-92.
• Gomella, et al. Neonatology Management,
  Procedures, On-Call Problems, Diseases, and
  Drugs. 2004.
• Behrman, et. Al. Nelson Textbook of Pediatrics
  17 th edition. 2004
• Lee, Stephen. Emedicine. Hirschsprung
  disease. 8/05.
• Burge, D. Meconium Plug obstruction. Pediatric
  Surg Int(2004) 20108-110

21
Case Management Conference

• Jaime Lanzillotta, DO
• November 29, 2005

22
D.C. 6 month old female with Bilious Emesis

• HPI Ex-23 week premature female
• 1 day h.o. initial nonbilious emesis (4-5
  episodes) (yellow in color occurred after each
  feed)
• Decreased wet diapers, decreased activity
• Normal intake-4 ounces q2-3 hours
• Normal stools
• Temperature 99

23
HPI, contd.

• Admitted 1 week prior with similar symptoms
• Diagnosed with partial small bowel obstruction.
• Decompressed with NGT and feeds were re-started.
• Discharged home on full feeds.

24
Past Medical/Surgical Hx

• 23 weeks premature
• Chronic lung disease-home O2
• Necrotizing enterocolitis-s/p bowel resection,
  ileostomy bowel re-anastomosis
• Retinopathy of prematurity-s/p laser
• Patent ductus arteriosis-s/p ligation

25
History, Continued

• Allergies-NKDA
• Family History-non-contributory
• Immunizations UTD
• Meds Poly-vi-sol, Calcium, Phosphorus

26
Physical Exam

• T 38.3 (ER) 37.3 (peds) P 175 RR 68 Sat 99 on
  0.1L NC
• General Awake, active, no distress
• Lungs Increased upper airway transmitted sounds
  mild subcostal retractions
• Abd Distended, non-tender, bowel sounds,
  reducible ventral hernia, Ø masses
• Ext warm, well perfused, cap refill lt3 seconds

27
Imaging and Laboratory Studies

• Lumbar Puncture
• glucose 93
• protein 39
• 0 WBC /325 RBC
• Lactate 1.7
• Gram stain negative
• Urine -, Rota-, RSV-

141 108 15
150
4.3 23 .3
11.6
Nonspecific film No air fluid levels Dilated
loops of bowel present
9.5
421
35
N 76 L 16 M 7.5
28
Assessment/ Plan

• 6 month old ex 23 week premature female with
  bilious emesis, rule out bowel obstruction
• Plan
• IV Ceftriaxone
• NG Tube decompression
• Surgical consult
• Serial abdominal exam
• NPO
• Guiac stools
• NG tube output replacement

29
Hospital Course

• HD1
• increasingly irritable
• Mom reported change in activity and behavior to
  staff
• HD 2
• Increased abdominal distension becoming more
  tense
• Poor perfusion
• Repeat x-ray was ordered showed signs of
  obstruction, with air fluid levels
• Transferred to the PICU for presumed obstruction
  and signs of shock
• Intubated and taken to the OR emergently

30
Hospital Course, contd.

• OR course
• closed loop bowel obstruction
• large areas of ischemic bowel-no resection
• abdominal compartment syndrome
• multiple adhesions-lysed
• Transferred back to the PICU

31
PICU course post op

• Resp
• intubated until POD 7.
• Weaned to nasal cannula.
• lasix prn for fluid retention.
• albuterol flovent.
• CV
• Stable Negative echo
• ID
• Broad spectrum antibiotics
• E.coli bacteremia

• Heme
• Anemia, thrombocytopenia
• PRBC, platelet transfusions
• FEN
• TPN x 2 weeks.
• NG feeds 1 wk post-op
• Advanced to full nipple feeds
• Metoclopramide
• Neuro
• sedated for intubation

32
Post-operative film
Dilated loops of bowel
Multiple air fluid levels
33
Hospital course, continued

• HD 26
• Transferred back to pediatric floor
• Hospital course on B3
• Tolerated full feeds
• Intermittent abdominal distension with stable
  x-rays
• Discharged home after 5 days.
• Readmitted 1 week after discharge, with fever,
  r/o SBI

34
Key Points

• Irritability in an infant with changing clinical
  exam warrants further investigation.
• What are the signs/symptoms of a closed loop
  bowel obstruction vs. partial bowel obstruction?
• Could a different diagnostic test have been
  performed to detect closed loop obstruction?
• Repeat examinations by surgical team is
  essential.
• Follow clinical judgement especially with
  changing exam/history.

35
Differential Diagnosis of Small Bowel Obstruction
in Infants

• Intussusception
• Incarcarated hernias
• Malrotation with midgut volvulus
• Postoperative adhesions

• Annular pancreas
• Mesocolic hernia
• Necrotizing enterocolitis
• Cecal volvulus
• Duplication cysts

36
Types of Obstruction

• Simple-blocked in 1 place
• Closed-loop-blocked in 2 places
• Strangulated-Decreased blood flow
• Incarcerated-When obstruction is not relieved and
  bowel becomes necrotic

37
Closed Loop Bowel Obstruction
2 sites of bowel obstruction
38
Pathophysiology ofSmall Bowel Obstruction
Obstruction Accumulation of chyle,
salivary,gastric, biliary,pancreatic intestinal
secretions Peristaltic contractions There is
also Impaired perfusion Ischemia/necrosis Perfora
tion
39
Pathophysiology ofSmall Bowel Obstruction
40
Clinical Features of Bowel Obstruction

• Colicky abdominal pain
• Irritable, fussy or inconsolable
• Decreased activity
• Vomiting (bilious in proximal obstruction,
  feculent in distal obstruction)
• Anorexia
• Diarrhea
• Constipation (complete obstruction)
• Fever (with bowel strangulation/necrosis)

41
Diagnostic Work Up

• Plain abdominal film-flat and upright
• Upper GI series
• Ultrasound
• CT
• Labs CBC, electrolytes, stool guiac

42
Closed Loop Obstruction

• Diffuse abdominal tenderness
• Increased irritablility in an infant
• Absence of bowel sounds
• Fever
• Tachycardia
• Leukocytosis
• Acidosis
• Blood in stool

These clinicial features are non-specific and may
NOT be present even when ischemia and necrosis is
occurring
43
Diagnosis of Closed Loop Bowel Obstruction with CT

• 19 cases of closed loop obstruction imaged with
  CT x-ray
• ABDOMINAL X-RAY CT
• Non-specific findings of SBO Signs of
  closed loop in 8
• in 10 pts
  pts
• Finding specific to closed Signs
  of closed loop
• bowel loop obstruction in 1 pt
  strangulation in 7 pts

Conclusion CT is a promising modality for
diagnosis of closed-loop and strangulating small
bowel obstruction
Closed Loop and Strangulating Intestinal
Obstruction CT Signs.Radiology 1992,185769-775