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Long-term outcome

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					 Long-term outcome
  in surgically-managed
necrotizing enterocolitis
    in a developing country-

     A high morbidity

     Arnold M, Moore SW, Sidler D, Kirsten GF
     Tygerberg Academic Hospital, Cape Town
       Introduction: NEC
5% premature neonates
Surgery:      30 - 50%
Mortality:    10%      50%
Multiple studies : ↓ longterm outcome
  neurological, cognitive, visual & psychomotor
  impairment
Bell’s stage III disease (surgical treatment):
  ↑ risk neurodevelopmental impairment
                 Methods
128 neonates treated surgically for NEC
Tygerberg Academic Hospital 1992 - 1995
    119 -mortality & surgical morbidity data available
    108 -longterm follow-up & neurodevelopmental
    records available
Retrospective review of data from:
    Paediatric surgery departmental records
    hospital administrative database
    patient files
    High Risk Clinic records
                   Objectives
Demographics
    Gestation, birth weight, % VLBW & ELBW
Mortality
o   Early (30 day post-operative)
o   Late (overall)
Morbidity
o   Surgical problems:
       Strictures, wound dehiscence, intra-abdominal abscesses/
       anastomotic breakdown, adhesive bowel obstruction, small
       bowel fistulae, stitch granulomas
o   Medical:
       Neurological, neurodevelopmental, infectious, cardiac,
       bronchopulmonary dysplasia, non-surgical gastrointestinal,
       visual & auditory
o   Good outcome: incidence
         Demographics
Mean gestational age
   32 weeks (22-40)
Mean birth weight
   1466g (655-3198)
VLBW (<1500g)
   52%
ELBW (<1000g)
   15%
          Early Mortality
<30 days post-
                             Causes of early mortality
operatively                                  extreme prematurity
                         sepsis with

37/119 died            cardiac problem
                           5% (n=2)
                                            (refused ICU) 3% (n=1)


  31% mortality
  69% early survival
                                                  pan-necrosis
                                                   49% (n=18)
                              sepsis with

VLBW and ELBW:                multi-organ
                                 failure

  71% early survival           43% (n=16)


  both subsets
                        Total = 37
              Late deaths
22/119
                                Causes of late mortality
  19 in1styear
  Death at 10 weeks - 36
  months age
                           32% n=7
50% overall survival                 unknown                         n=9
                                                                     40%

                                                          sepsis
VLBW subset
  50% overall survival            intra-
                                ventricular      short
ELBW subset                    haemorrhage
                                                 bowel
  43% overall survival      5% n=1             syndrome

                                              23% n=5              Total = 22
   NEC Post-operative surgical morbidity

       7% stitch         4% Post-operative
    granuloma (n=8)       strictures (n=5)
                                 4% Incisional
                                 hernias (n=5)

                                       5% adhesive
                                     bowel obstruction
    13%                                    (n=6)
short bowel
 syndrome
   (n=15)
                                     6% re-laparotomy
                                      for anastomotic
                                        leak or intra-
               4%           6%           abdominal
          small bowel     w ound       abscess (n=6)
            fistulae    dehiscence
              (n=5)        (n=7)             TOTAL= 57/ 119 patients
          Surgical morbidity
Surgery type
5   - peritoneal drains only
114 - laparotomy
[13 - resection with primary anastomosis]

Strictures (11/119 = 9%)
   6 - primary indication surgery after conservative management
   1 - early presentation at 2 weeks after surgery
   3 - identified & resected at colostomy closure
   1 - late presentation after 2 years 9 months

Adhesive bowel obstruction
   presentation 2 weeks - 10 years post-operatively
Long-term outcome of survivors
60/119 survived longterm (50%)

  19 (16%) lost to follow-up
    6 inadequate data to trace (wrong hospital no, name
    changed etc.)
    5 transferred to secondary hospitals for further management

  41 (68%) followed up at Tygerberg Hospital
    30 followed up >2 years age
        Average follow-up 39 months [4 - 124 months]


  GOOD OUTCOME in 23/60 (56%):
    discharged from follow-up care at various clinics
    (neurodevelopmental, neurological, GIT etc.)
                       Longterm morbidity in 41 survivors
                          of surgically managed NEC

             Foetal Alchol syndrome (5%)      2

                  Visual impairment (10%)         4

                Auditory impairment (12%)             5

                    Cardiac disease (15%)                 6

       Bronchopulmonary dysplasia (15%)                   6

          Severe neurological deficit (20%)                   8

          Gastro-intestinal problems (39%)                        16

                Recurrent infections (39%)                        16

Significant neurodevelopmental delay (49%)                             20
    Long-term outcome cont.
Visual impairment includes:
   Strabism, myopia
Auditory deficit includes:
   Sensory & conductive deafness, with varying degrees of
   associated speech development delay
GIT problems include:
   Constipation, encopresis, GERD, subacute bowel obstruction
   resolving with non-surgical management
Neurodevelopmental outcome
   Improvements by 2 years of age seen in 4 patients
Cardiac disease
   3 PDA’s requiring banding
   1 severe mitral valve prolapse requirng surgery
                                     Discussion
     Systemic review of 821 VLBW children
     with NEC in developed world*:

          2.3x increased odds ratio neurodevelopmental
          impairment in Bell’s stage III NEC

          45% incidence neurodevelopmental
          impairment in NEC overall
         ______________________________________
*
Rees CM , Pierro A, Eaton S. Neurodevelopmental outcomes of neonates with medically and surgically treated
  Rees
               enterocolitis.                                              92(3):F193-
   necrotizing enterocolitis. Arch Dis Child Fetal Neoanatal Ed. 2007 May; 92(3):F193-8.
 NEC increases risk of:
Prolonged          Periventricular
intubation         leukomalacia
Sepsis             Chronic lung
Prolonged          disease
hospitalization    PDA requiring
Impaired           treatment
neurodevelopment   Cognitive
Psychomotor        impairment
impairment         Visual impairment
                   Cerebral palsy
      Surgical morbidity
Stricture, wound dehiscence & intra-
abdominal abscesses:
 comparable rates to other studies


↑ incidence short bowel syndrome
 associated high mortality and morbidity
          Limitations of study
Poor follow-up rates of survivors
  But similar to other retrospective studies
  (68% versus 72 to 78%)
  Limited period follow-up for some
Lack SA cohort/ ↑ spectrum birth weights
No specific measure neurodevelopment
   e.g. Griffiths Developmental Scale; Bayley scores (PDI, MDI)
     Clinical assessment (milestones + gross neurology)
      may miss children at risk
              Conclusions
50% overall survival
despite good (69%) initial survival
in surgically-managed NEC


High long-term morbidity in survivors
  Neurodevelopmental
  Neurological
  Infectious
  Gastrointestinal


50% survivors: good longterm
outcome
       Recommendations
Advise parents
  High risk long-term morbidity
  Need regular, long-term follow-up

Clinician awareness:
  ↑ risk problems
    neurodevelopmental, infectious, gastro-intestinal,
    neurological
  Early detection, longterm follow-up & support

Allocate resources
                                          References
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11.                                                                   weight
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