12 HAS THE OUTCOME OF EXOMPHALOS IMPROVED IN THE LAST DECADE: A TERTIARY CENTRE EXPERIENCE?
Zain Panjwani, Sewita Nazari, Eric Nicholls, CK Sinha
St Georges University Hospital, London, United Kingdom

Abstract

Aim:

The aim of this study was to find out the current outcomes of Exomphalos major and minor babies.

Methods:

Data of infants treated from July 2007-June 2017 was collected retrospectively. Infants were divided into two groups: Exomphalos minor-where the defect was less than 4cm and contained only intestine; Exomphalos major- where the defect was greater than 4cm and/or with the liver inside the cord.

Results:

Of the 43 infants, 37%(16/43) were Exomphalos(E) major and 63%(27/43) were Exomphalos minor. Incidence of malrotation was 14% in E minor and none in E major. Complications were seen in 7% of E minor and 50 % of E major babies. Other outcomes are summarised in table 1.

Conclusions:

  • Comparing the results from literature, E major babies still do have significant morbidities.
  • Incidence of Beckwith Weiedemann  syndrome was equally common in E major and E minor babies.

Table 1 showing the comparative outcomes of E major and E minor

Parameters

E minor(n=27)

E major(n=16)

Male:Female

15:12

9:7

Gestation

38(28-42) wks

36(30-40) wks

Wt

3.2(0.78- 5.3) kg

2.5(1.24-3.36) kg

Chromosomal anomalies

4% - 1/27(6p deletion)

6% - 1/16(Edward’s)

Beckwith Weiedemann  syndrome

22% (6/27)

25% (4/16)                  

Major cardiac anomalies

15% (4/27)

44% (7/16)             

PPHN

4% (1/27)

19% (3/16)              

Sepsis

26% (7/27)

37.5% (6/16)           

Malrotation

14% (3/21)

0% (0/9)                   

Management

-Primary closure 27

-Median day of

  operation       2 (1-10)

-Prim closure  44% (7/16)

-Surgical silo   25% (4/16)

-Conservative  31% (5/16)

Median(range) days to full feed

5 (1-12)

 9 (5-33)

Hospital stay(days)

6 (2-13)

21 (4-227)

Complications

7%(2/27)

-Wound infection    1/27

-Intest. obstruction 1/27        

31%(5/16)

-Wound infec          2/16

-Incisional hernia    2/16

-Abdomen compartment

syndrome              1/16            

Death

0%(0/27)

19%(3/16)    p value 0.04

[PPHN- primary pulmonary hypertension]

 


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