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Pediatric Case Management
The Children’s Hospital at Sinai
Joseph Wiley, MD
Cynthia Roldan, MD
November 29, 2005
November Cases-ER 4 month old infant with HIE with severe
neurologic sequelae presented in respiratory arrest
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)
Pediatric Case Management Conference
November 29, 2005
Julia Trintis, D.O.
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
NICU Course
Work up: 1. AXR-gaseous distention of small and large
bowel loops; opacification of rectum and sigmoid colon
2. Barium Enema- no evidence of microcolon; possible rectal web
3. Sigmoidoscopy-normal-no web or stricture
4. CF Studies-ordered (ultimately negative)
Neonatal Assessment FT infant with probable meconium plug syndrome.
Differential Diagnosis: Hirschsprung’s 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
HPI Presentation to Pediatric Floor
Initially doing well BM’s 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
Physical Exam
T:36.5 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
Abdominal X-Ray
B
A
Abnormal Colonic Caliber
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
Points for Discussion Differential Diagnosis of Meconium Plug
Diagnostic Methods for Hirschsprung’s Disease Choice of biopsy: Rectal Suction vs. Full
thickness Anal rectal Manometry
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
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%
Hirschsprung Disease Aganglionic megacolon: lack of intramural
ganglionic cells Occurs in 1:5000 births Associated with Down syndrome Signs: distended abdomen, palpable loops
of bowel, rectal exam without stool in ampulla
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
Meconium Plug Obstruction:Retrospective Case Review21 patients with Large Bowel Obstruction Relieved by Passage of Meconium Plugs
Diagnosis MPS HD SLCS
Number 9 (43%) 8 (38%) 4 (19%)
Mean gestation (wks) 37 39 37
Mean BW (gms) 3369 3363 3403
Abdominal Distention 7 8 4
Bilious emesis 6 8 2
Conclusion: Essential for all babies with MP obstruction to have HD excluded.Burge, D. Meconium Plug obstruction. Pediatric Surg Int(2004) 20:108-110
Diagnosis of Hirschsprung’s Disease: a prospective, comparative accuracy study of common tests
111 Infants suspected of HD
Anal Manometry
83% sensitive
93% specific
Rectal Suction Biopsy
93% sensitive
100% specific
Contrast Enema
76% sensitive
97% 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 Hirschsprung’s disease: a prospective, comparative accuracy study of common tests” J. Pediatrics. 2005, 146 (6): 787-92.
Management Primary pull-through procedure
Soave (endorectal) procedure Swenson procedure Duhamel procedure
Early colostomy with resection of aganglionic segment & Re-establishment of continuity
References1. Diament, M. Emedicine. “Meconium Plug Syndrome.” 3/05.2. Hekmatnia, Ali. Emedicine. “Meconium Ileus.” 7/05.3. De Lorijn, et al. “Diagnosis of Hirschsprung’s disease: a
prospective, comparative accuracy study of common tests” J. Pediatrics. 6/05, 146 (6): 787-92.
4. Gomella, et al. Neonatology: Management, Procedures, On-Call Problems, Diseases, and Drugs. 2004.
5. Behrman, et. Al. Nelson Textbook of Pediatrics: 17 th edition. 2004
6. Lee, Stephen. Emedicine. “Hirschsprung disease.” 8/05.
7. Burge, D. Meconium Plug obstruction. Pediatric Surg Int(2004) 20:108-110
Case Management Conference
Jaime Lanzillotta, DO
November 29, 2005
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°
HPI, cont’d.
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.
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
History, Continued Allergies-NKDA Family History-non-contributory Immunizations: UTD Meds: Poly-vi-sol, Calcium, Phosphorus
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 <3 seconds
Imaging and Laboratory Studies
Lumbar Puncture glucose 93 protein 39 0 WBC /325 RBC Lactate 1.7 Gram stain negative
Urine -, Rota-, RSV-
Nonspecific film: No air fluid levelsDilated loops of bowel present
4.3 23 .3
141 108 15150
N 76 L 16 M 7.5
9.511.6
35421
Assessment/ Plan6 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
Hospital CourseHD#1
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
Hospital Course, cont’d.
OR course: closed loop bowel obstruction large areas of ischemic bowel-no resection abdominal compartment syndrome multiple adhesions-lysed Transferred back to the PICU
PICU course: post opResp:
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
Post-operative film
Multiple
air fluid levels
Dilated loops of bowel
Hospital course, continuedHD # 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
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.
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
Types of Obstruction
1. Simple-blocked in 1 place
2. Closed-loop-blocked in 2 places
3. Strangulated-Decreased blood flow
4. Incarcerated-When obstruction is not relieved and bowel becomes necrotic
Closed Loop Bowel Obstruction
2 sites of bowel obstruction
Obstruction
Accumulation of chyle, salivary,gastric, biliary,pancreatic & intestinal secretions
Peristaltic contractions
There is also:
Impaired perfusion
Ischemia/necrosis
Perforation
Pathophysiology ofSmall Bowel Obstruction
Pathophysiology ofSmall Bowel Obstruction
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)
Diagnostic Work Up Plain abdominal film-flat and upright Upper GI series Ultrasound CT
Labs: CBC, electrolytes, stool guiac
Closed Loop ObstructionDiffuse 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
Diagnosis of Closed Loop Bowel Obstruction with CT19 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
Closed Loop and Strangulating Intestinal Obstruction: CT Signs.Radiology 1992,185:769-775
Conclusion: CT is a promising modality for diagnosis of closed-loop and strangulating small bowel obstruction