Authors:

Munyi N 1,2, Poenaru D 1, Bransford R 1, Albright L 1

Affiliation:

1.Bethany Kids at Kijabe Hospital, Kijabe, Kenya.2.School of Medicine, University of Nairobi

ENCEPHALOCELES: THE

KIJABE EXPERIENCE

INTRODUCTION

� Encephaloceles are congenital lesions consisting of a herniationof intracranial contents 1.

� The herniation may consist of :� Meninges only (meningocele),

� Brain matter (encephalocele)

� Meninges + brain matter (meningoencephalocele)

� Meninges + brain matter + ventricle (hydroencephalomeningocele) 1

1. JeffreyV. Rosenfeld and David A.K. Watters, Neurosurgery in the tropics: A practical

approach to common problems, Macmillan Education Ltd, 2000

Are they NTDs?� There has been controversy as to whether they are all truly NTDs.

� Studies have reported that only a minority are / are associated NTDs 2

� Rowland et al 2002 found that with folic acid fortification, prevalence decreased for spina bifida but not significantly for encephaloceles or anencephaly 3.

2. Humphreys R E. Encephaloceles and Dermal Sinuses in: Cheek W R et al, Pediatric

Neurosurgery: Surgery of the Developing Nervous system. 1994

3. Rowland et al Are encephaloceles neural tube defects? Pediatrics 2006; 118:916-923

Epidemiology� Are rare and the incidence varies with geographical location and race. 4

� Overall incidence is 0.8 to 3.0 per 10,000 live births. 5,6,

� Occipital type (85 % of encephaloceles) is most common in N. America and W. Europe with an incidence varying between 1 in 3000 to 1 in 10,000 LB 7,8

� 70% of occipital encephaloceles are in females 7

� 15-20% are associated with NTD

� Anterior encephaloceles are mostly found in SE Asia, Russia and Central Africa . The incidence ranges from 1 in 3500 to 1 in 5000 4,7

4. Suwanwela C and Suwanwela N, A morphological classification of Sincipital

Encephalomeningoceles. J Neurosurg 1972, 36 201-211

5Macfarlane R, Rutka JT, Armstrong D et al Encephaloceles of the anterior cranial fossa, Pediatric

neurosurgery 1995: 23(3) 148-158

6. Simpson DA, David Dj, White J. Cephaloceles treatment, outcome, and antenatal diagnosis.

Neurosurgery. 1984;15:15-21

7. Chapman PH, Swearingen B, Caviness VS. Subtorcular occipital encephaloceles. J. Neurosurg.

1989;71:375-381

8. Mealey Jr J, Dzenitis AJ. The prognosis of encephaloceles. J Neurosurg. 1970; 32: 209-218

CLASSIFICATION

Convexity

� Occipital

� Parietal

� Saggital

� Occipitalcervical

Atretic

Sincipital

� Frontoethmoidal-nasofrontal-nasoethmoidal

-naso-orbital

� Interfrontal

� Craniofacial cleftBasal

� Intranasal

� Spheno-orbital

� Sphenomaxillary

� Spenopharyngeal

Are classified according to the anatomical location of the

skull defect 4

These include:

RATIONALE OF STUDY

� Though encephaloceles are rare congenital malformations, they are associated with severe morbidity and mortality if untreated.

� Most reported series of encephaloceles originate in the West, where resources for their treatment are radically different thanin Africa.

� This study seeks to find out the presentation, management and complications of encephaloceles in an African setting as well as

answer the question: Can encephaloceles be successfully managed in a resource-poor setting?

Design and Methodology� A retrospective study of patients seen and managed at Kijabe hospital between January 1998 and August 2006.

� Inclusion criteria: any patient with an encephalocele.

� Exclusion criteria: any patient who’s records were not available.� Data collected: Biodata, Type of encephalocele, Associated anomalies, US and CT features, Surgical approach used, Intra-OP and Post-OP complications and Follow up outcomes.

� Data Analysis: Calculation of Means, Drawing of frequency charts and distribution graphs done.

RESULTS� Total number of patients: 53

� 23 male ; 30 female.� Charts of seven of the patients were unavailable and hence excluded from the analysis.

� Age Distribution: 1 day- 15 yrs Mean: 24 months

14.016.0

7.05.0

3.0 4.0 4.0

0.0

5.0

10.0

15.0

20.0

< 1 1_6 7_12 13-24 25-60 61-120 >120

age in months

Age distribution

Series1

Num

ber

of

Pat

ien

ts

Types of encephaloceles

distribution0%

2%

4%

33%

61%

basal

complex

missing

sincipital

convexity

Convexity encephaloceles

88%

6%

0%6%

occipital

parietal

sagggital

occipitocervical

detailed distribution

53%

0%

4%

4%

24%

9%2% 0%

4%

occipital

parietal

sagggital

occipitocervical

frontoethmoidal

interfrontal

basal

complex

missing

Associated Anomalies

•Of those with Hydrocephalus (HC) :

•9 had occipital encephaloceles

•3 had sincipital encephaloceles

•1 was not classified

139 6

25

0

5

10

15

20

25

H.C other H.C +

other

none

Associated anomalies

Associated

anomalies

26 px had additional Sx.

17/26(65%) had VPS insertions.

Surgical Approaches

85%

11%4%

direct ext

approach

intracranial

ant & medial

ext

approach16

5

29

1 20

5

10

15

20

25

30

Contents of encephaloceles

Series1

Series1 16 5 29 1 2

brain

tissu

other

tissu

CSF

only

missi

ng

Sx

elsew

0

20

40

Other Surgeries

Series1

Series1 24 19 5 2 3

no 1 Sx 2 Sx >2 miss

Complications(Intra-op, post-op & late)

� 6/49 had intra-operative complications

� 20/49 had post operative complications.� 2 deaths, CSF leak was most common complication 6/20(30%), Others:seizures, wound infection, shunt malfomations, HC

� 10/47 had late post-operative complications� 2 deaths, 3 recurrences, 1CP, 2 HC, 1 infection, 1 frontal swelling

Follow up and outcomes

Follow up outocmes

30

5

1

2

5

2

4

0 10 20 30 40

doing well

dev delay

blind

MR

death

CP and blind

other

Series1

Discussion� Our sample size on of the largest case series in literature (over 8 yr period)

� Missing population based data therefore no conclusion on incidence can be made

� Female preponderance: our study 61% vs other studies:70%

� Contrary to other studies: occipital type is most common in our set up.

� Most of our patients had mainly CSF with little brain tissue: good prognosis

Discussion II

� Age at presentation: earlier presentation than in Thailand study (70% present under 6 months in our study, in Thailand most present between 6-12months)

� Imaging: inaccessible due to low socioeconomic status

� Various surgical approaches have different indications.

� Complications: most common CSF leak- similar to other studies.

Conclusions� Diagnosis can be done on clinical basis.� Encephalocele repair is generally a clean procedure hence no need for routine pre-operative medication.

� External approach is used for occipital but intracranial gives better outcome for frontal

� Most common associated anomaly is HC� Encephaloceles generally have good outcome even in an African setting.

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