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