Management of neural tube defects in a Sub-Saharan African country: The situation in Yaounde, Cameroon

Journal of the Neurological Sciences 275 (2008) 29–32

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Journal of the Neurological Sciences j o u r n a l h o m e p a g e : w w w. e l s ev i e r. c o m / l o c a t e / j n s

Management of neural tube defects in a Sub-Saharan African country: The situation in Yaounde, Cameroon Vincent de Paul Djientcheu a, Alfred Kongnyu Njamnshi b,⁎, Ambroise Wonkam c, Julie Njiki d, Mohamadou Guemse d, Robinson Mbu f, Marie Thérèse Obama e, Samuel Takongmo g, Innocent Kago d, Ekoe Tetanye h, Felix Tietche d a

Neurosurgery Department, Central Hospital Yaounde, Cameroon Neurology Department, Central Hospital Yaounde, Cameroon c Department of Genetic Medicine, Gynaecology Obstetrics and Paediatrics Hospital Yaounde, Cameroon d Paediatric Department, Mother and Child Centre of The Chantal Biya Foundation Yaounde, Cameroon e Paediatric Department, University Hospital Center of Yaounde, Cameroon f Gynaecology and Obstetrics Department, Central Hospital of Yaounde, Cameroon g Surgery Department, University Hospital Center of Yaounde, Cameroon h Paediatric Department, Faculty of Medicine and Biomedical Sciences, University of Yaounde 1, Cameroon b

A R T I C L E

I N F O

Article history: Received 25 January 2008 Received in revised form 30 June 2008 Accepted 3 July 2008 Available online 15 August 2008 Keywords: Neutral tube defects Spina bifida Management Cameroon Sub Sahara Africa

A B S T R A C T Background: Neural tube defect is a serious disabling but preventable congenital malformation with an incidence of 1.99 per 1000 births in Yaounde [A.K. Njamnshi, V. d e P. Djientcheu, A. Lekoubou, M. Guemse, M.T. Obama, R. Mbu, S. Takongmo, I. Kago. Neural tube defects are rare among black Americans but not in Sub-Saharan black Africans: The case of Yaounde – Cameroon. Journal of the Neurological Sciences 2008; 270: 13–17]. The management requires highly qualified personnel and a significant social cost. The aim of this study was to evaluate the management of neural tube defect in a resource-limited developing Sub-Saharan nation like Cameroon. Methods: We reviewed all patients with neural tube defects admitted in the neonatology unit of the Mother and Child Center (Chantal Biya Foundation Yaounde) between January 1st 2000 and December 31st 2006. Results: Sixty-nine (69) patients were enrolled. There was a male predominance (69.57%) in the sample. Myelomeningomecele represented 68.11% of cases, followed by encephalocele (27.54%) and meningocele (4.35%). Antenatal ultrasound examinations were done in 27 cases (32.8%). The prenatal diagnosis was made only in 8 cases. No medical abortion was performed in any of these cases. Medical abortion is illegal in Cameroon (except in certain specific situations) as well as other Sub-Saharan African countries. Hydrocephalus was diagnosed in 40.02% of cases. As most of the patients (62.32%) could not afford modern treatment, only 26.09% of them were operated at birth. The rest sought traditional and other forms of treatment, due to poverty or cultural beliefs. Eight patients (11.59%) died before surgery. Surgery consisted of local closure alone (40%) or local closure associated to CSF shunting (60%). The complications were wound dehiscence (13.69%), shunt infection (1.37%), meningitis (1.37%) and iatrogenic pulmonary oedema (1.37%). Conclusion: Neural tube defects are the most frequent and disabling malformations in neonates in the SubSaharan African paediatric environment. Prenatal management and outcome at birth are limited by poverty and cultural beliefs. Prevention is possible and may be better than palliative care in developing countries. © 2008 Elsevier B.V. All rights reserved.

1. Introduction Neural tube defects have been considered for a long time as a rare malformation in Africa [1]. However, recent studies have revealed incidence rates of 1.99 and 7 for 1000 births respectively in Yaounde and the middle belt Nigeria [2,3]. Spina bifida, anencephaly and encephalocele are the most frequent and severe forms. The place of encepha⁎ Corresponding author. Neurology Department, PO Box 25625, Yaounde, Cameroon. Tel.: +237 77 61 99 64. E-mail address: [email protected] (A.K. Njamnshi). 0022-510X/$ – see front matter © 2008 Elsevier B.V. All rights reserved. doi:10.1016/j.jns.2008.07.003

locele among neural tube defects is still debated [4]. The management of these conditions involves different specialities including paediatrics, neurosurgery, orthopaedics, urology and physical medicine [5]. However, this corrective management is difficult to achieve in resource-limited developing countries. Furthermore, there is no policy to prevent these defects in Sub-Saharan Africa and other developing countries [2,3,6–15]. More and more hospital series are being reported in Sub-Saharan Africa with many underlying difficulties in management [6,8,10,12,13,16]. The aim of this study was to describe neural tube defects in the Neonatology Unit of the Mother and Child Center (Chantal Biya Foundation Yaounde) and to assess its management in a

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V.P. Djientcheu et al. / Journal of the Neurological Sciences 275 (2008) 29-32

Table 1 Prevalence of NTDs in Paediatric Departments of Yaounde

CBF

UHCY

GOPHY

Years

1997

1998

1999

2000

2001

2002

2003

2004

2005

2006

Number of NTD admitted Number of babies admitted Rate of NTD Number of NTD admitted Number of babies admitted Rate of NTD Number of NTD admitted Number of babies admitted Rate of NTD

5 754 0.66

7 999 0.70

8 1137 0.70

14 1230 1.13

6 1122 0.53

8 997 0.80 1 576 0.17

0.66

0.70

0.70

1.13

0.53

0.48

10 1062 0.94 0 496 0.00 1 277 0.36 0.43

10 950 1.05 1 351 0.28 3 558 0.53 0.62

10 1134 0.88 3 468 0.64 5 704 0.71 0.74

11 1160 0.94 4 406 0.98 3 741 0.40 0.77

Mean rate

resource-limited context like our capital city where no structured social system of mutual medical risk-sharing exists. 1.1. Patients and methods This was a retrospective study of all patients admitted in the Neonatology Unit of the Mother and Child Center of Yaounde (Chantal Biya Foundation) from January 1st 2000 to December 31st 2006 with a neural tube defect. The Mother and Child Center (Chantal Biya Foundation) is the Paediatric Hospital associated to the Central Hospital of Yaounde which has the only neurosurgical service of Yaounde. The patients were identified from the service register and the files were then obtained from the archives. For the purpose of comparison, the prevalence rates were obtained from the paediatric registers of the other university teaching hospitals of Yaounde: General Hospital of Yaounde, University Hospital Center of Yaounde and the Gynaecological-Obstetric and Paediatric Hospital of Yaounde. The estimated population of Yaounde is about 2,000,000 inhabitants with a general crude birth rate of 47 births for 1000 inhabitants [17]. The socioeconomic status of patients' parents was assessed by the observation of social workers and nurses based on the ease of the families to buy drugs and food, prepare the patient bed and the number and quality of the patient visitors. They were then classified as of low, middle or high socio-economic status. The diagnosis of neural tube defect and associated hydrocephalus was done clinically. Trans-frontanellar ultrasound was done in neonates suspected of having hydrocephalus. During the study period, surgical management of the patients was done by the two neurosurgeons of the Central Hospital of Yaounde. 2. Results 2.1. Prevalence A total of 69 patients with neural tube defects were admitted in the Neonatology Unit of the Mother and Child Center of Yaounde within the period of the study. This represents a prevalence rate of 0.9% compared to the total number of patients admitted in the paediatric service during this period. During the same period, the admissions register of the 2 other teaching hospitals of Yaounde revealed prevalence rates of 0.52% and 0.39% for the Gynaecological-Obstetric and Paediatric Hospital of Yaounde (GOPHY) and University Hospital Center of Yaounde (UHCY) respectively. These rates increased during the period of study (Table 1). 2.2. Distribution of patients according to sex, birth weight, origin and social status There were 48 males (69.57%) and 21 females (30.43%). The male– female sex ratio was 2.28. The birth weight was below 2000 g in 3 cases of premature babies (4.41%), between 2000 g and 4000 g in 64 cases (92.64%) and above 4000 g in 2 cases (2.94%). Most of the

patients were born in the maternities of Yaounde (66.17%) while the others were referred from maternities out of Yaounde (33.83%). Generally, parents of patients were from the low (80%) and middle (20%) socio economic classes. The professions of the patients' mothers were as follows: civil servants (4.4%), students (8%), employees (20.58%), without employment (63.23%) and not stated (3.79%). 2.3. Type of malformation A total of 47 cases (68.11%) of myelomeningocele, 19 cases (27.54%) of encephalocele and 3 cases (4.35%) of meningocele were recorded. Spina bifida was localised in the lumbar region in 32 cases (64%), sacral in 11 cases (22%), lumbosacral in one case (2%) and not indicated in 3 cases (6%). Encephalocele was anterior (fronto-ethmoidal) in 6 cases (31.58%), posterior in 12 cases (63.16%) and not stated in 1 case (5.26%). 2.4. The neurological status of patients with spina bifida The neurological status (Table 4) was normal in 12% of patients. Paraplegia with anal sphincter disorders was present in 52% of patients. Isolated sphincter disorders were present in 12% of patients. Patients with paraplegia presented with either complete or partial neurological involvement below the lesion but information on the sensory level was not available. There was associated hydrocephalus in 42.03% of cases. Orthopaedic talipes (26.08%) and omphalocele (2.04%) were present in some patients (Table 5). 2.5. Management during the antenatal period About a third of mothers started prenatal consultation in the first trimester (35.29%), while the others started in the second (50%) and third trimester (14.71%). The mean number of prenatal consultations was 3.04 per woman. Obstetrical ultra sound was done in 27 cases (32.8%). It was performed in the first trimester for 15 cases, in the second trimester for 6 cases and in the third trimester for 6 cases. The diagnosis of neural tube defect was done through this technique in 8 cases. Anti-anaemic prophylaxis with iron or folic acid was used in 55.88% of cases in the second semester. Maternal history of fever in the

Table 2 Prevalence in other African paediatric departments Region: Year

Number of babies with malformation

Total number of normal babies

Prevalence

Abidjan HUC Cocody (Ivory Coast):1997 [21] Donka (Guinea Conakry): 1999 [10] Chantal Biya Foundation Yaounde (Cameroon): 2006

31

625

4.96%

5

310

1.61%

69

7955

0.90%

V.P. Djientcheu et al. / Journal of the Neurological Sciences 275 (2008) 29–32 Table 3 Post-operative course

Table 5 Associated abnormalities

Complication

Number

Percentage

No complication Wound dehiscence CSF leak Shunt infection Meningitis Dead (iatrogenic pulmonary oedema) Total

57 10 0 1 0 1 69

83.56% 13.69% 0% 1.37% 0% 1.37% 100%

first trimester was recorded in 15 cases (22%). Fever was due to malaria (90%) and pyelonephritis. 2.6. Management at birth Patients presented and were admitted on the first day of life in 44.13% of cases, in the first week of life in 31.88%, between the first and the 4th week or life in 7.25% and after one month in 15.94% of cases. The associated medical conditions were a probable neonatal infection (5%), a possible neonatal infection (29.41%), meningitis (13.23%) and neonatal tetanus (2.94%). All the patients with spina bifida were systematically placed on broad spectrum antibiotics on admission. Hydrocephalus was present in 29 cases and orthopaedic abnormalities in 18 cases. Among the 69 patients, 18 patients (26.09%) were operated, 43 patients (62.32) were discharged and 8 patients (11.59%) died without surgery. Among the patients operated, 60% underwent local repair of the defect associated with ventriculo-peritoneal shunt while 40% underwent only the local repair. The post-operative course was uneventful in 83.56% of cases. The complications were wound dehiscence (13.69%), shunt infection (1.37%), meningitis (1.37%) and iatrogenic pulmonary oedema (1.37%). The wound dehiscence affected only the cutaneous (and subcutaneous) layer and was treated by wound dressing. The patients who were not operated were lost to follow-up. The operated patients were reviewed at 6 weeks and at three months after surgery. The results of post-operative course are shown in Table 3 above. 3. Discussion Neural tube defects are frequent and disabling congenital malformations in Sub-Saharan paediatric hospitals (Tables 2 and 3). They used to be considered as rare pathologies among blacks in general [1,18,19] but recent studies have demonstrated a higher incidence in sub-Saharan black Africans [2,3,14], compared to black Americans [18–20]. The prevalence varies from one service to another[6,8,10,12,13,16] according to the degree of specialisation of the hospital. Few data are available in Sub-Saharan African countries [21] but the epidemiology of neural tube defects is comparable in most series with a male predominance (52 to 70%), a predominance of myelomeningocele (60 to 70%) on other forms like encephalocele and meningocele; the lumbar localisation of spina bifida is more frequent; the occipital form of cephalocele is preponderant compared to the anterior sites. Prenatal diagnosis was done in only 8% of our cases. This may be linked partly to the limited experience of echographers although

Table 4 Neurological status of patients with spina bifida (50 patients) State

Paraplegia and sphincter disorder

Number 26 Percentage 52%

31

Sphincter disorder

Normal No information

Total

12 24%

06 12%

50 100%

06 12%

Associated malformations

Hydrocephalus Orthopaedic talipes

Omphalocele Jaundice Total

Myelomeningocele Meningocele Encephalocele Total number Percentage

16 01 12 29 59.18%

01 00 00 01 2.04%

15 02 01 18 36.74%

01 00 00 01 2.04%

33 03 13 49 100%

prenatal investigation was not performed in many cases. None of the cases diagnosed prenatally underwent pregnancy termination. Medical abortion is illegal except in certain conditions (like malformation not compatible with foetal life or a disease threatening the life of the mother) in Sub-Saharan African countries in general and it is contrary to traditional and religious values. Christianity and Islam are the main religions in Cameroon and other sub-Saharan African countries, to a variable degree. The management of neural tube defects is limited in developing countries in general and sub-Saharan African countries in particular by poverty, ignorance and cultural beliefs [6,8–14,16]. Babies with neural tube defects are considered as a curse or believed to be a disease condition inflicted by witchcraft upon the whole family and are therefore simply neglected in some cases. A general paediatric service (neonatology) was selected for this study rather than the specialised surgical services of neurosurgery in order to limit selection bias. Patients who effectively reached specialised surgical services are those who would be ready both materially and psychologically for surgery. Surgery (surgical closure of the defect and CSF derivation if necessary) is the primary treatment of neural tube defect. The rate of surgically treated babies is very low (Table 6) and ranged from 26.09 to 55% in most African series [6,10,12,13], except in specialised services [8,13] where the system of admission selects candidates for surgery. The patients who could not afford surgery either absconded or were discharged against medical advice and these generally went for native (traditional) treatment. Very rarely did patients leave the hospital not operated because of mystical beliefs. It therefore appears that the main reason for the poor access to surgical treatment is socioeconomic. There is no system of health insurance or social security in our context and to add to this burden, this pathology affects mostly the lower socioeconomic groups of the population. The mortality rate is very high in African series (Table 6). It appears to be due to largely to infections (meningitis) which can be explained by the delay of the surgical closure of the defect. Most deaths did occur before surgery in our current study. The rate of hydrocephalus ranged from 50 to 60%, but some low values [10,17] may be due to unrecognised cases in general surgery units and difficulties with performing and interpreting echographic examinations. After hospitalisation, the patients were generally lost to follow-up. However, some of the operated patients (26.09%) are reviewed at 6 weeks (30%) and 3 months (20%). There is neither a center nor social organisation adapted or equipped for the re-education, the rehabilitation or the social integration of these children in Yaounde.

Table 6 Management of NTD in other African series and our series Series

Mabogunje (Nigeria)

Sanoussi (Niger)

Ouattara (Abidjan)

Alatise (Nigeria)

Our series (Cameroon)

Number of cases Study period Children operated Moved Died Lost to follow-up (1 year)

312 cases 14 years NA 13.7% 17.9% 46.9%

387 cases 10 years 49.35% 40% 20.29% 33%

132 cases 12 years 60% 16.25% NA 79%

106 cases 4 years 91.5% 0% 22.7% 80%

69 cases 6 years 26.09% 62.32% 11.59% 70%

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They are generally abandoned to their families which are lowincome families in most cases, rendering middle and long-term follow-up not possible. Neural tube defects are associated with innate factors (folic acid metabolism, gene mutation) and acquired factors (nutritional, maternal fever, intoxication, irradiation, anti-epileptic and other drugs) which variably interact [18,22–27]. The impact of these factors is not well known in Sub-Saharan African countries where malaria is endemic, auto medication is uncontrolled and consanguineous marriages are frequent in some cultures and where antenatal folic acid supplementation is not the rule. Furthermore, in Sub-Saharan countries and other developing countries, there is no policy on the prevention of neural tube defects [2,3,6–15] although it is well known that peri-conceptional supplementation of folic acid reduces the incidence of the neural tube defects by about 50% or more [18,20–23]. As we have recently suggested [2], in resource-limited and developing countries such as Cameroon, prevention strategies such as folic acid supplementation are an applicable and emergent measure. If 60% of parturient women receive anti-anaemia prophylaxis (iron and folic acid) in the 2nd and 3rd trimester, it is possible to extend this method to all women of reproductive age. Depending on the educational level of couples, the number of undesired or unprogrammed pregnancies, it will be a real challenge to selectively target the peri-conceptional period for this strategy. An accrued sensitisation of the general population, nongovernmental organisations (NGOs), public decision-makers and health personnel is an emergency step in order to curb this disabling and deadly yet preventable condition. Among the various impediments to implementation of the periconceptional prevention strategy, we envision that the absence of an implementation policy, the low awareness on the part of healthcare personnel and the ignorance (presumed) of the target population constitute the major obstacles. It is our hope that the new evidence provided by our previous report [2] and the current paper would serve as an advocacy instrument to convince policy-makers to enact the necessary policy. Furthermore, this information would serve to educate health-care providers on the need to apply the policy when available and to sensitise the general public, especially the target population on the issue. However, there is need to assess the knowledge, attitudes and practices of healthcare personnel, dietary habits and the acceptability of the intervention by the target population, as a prerequisite to the promotion of a peri-conceptional neural tube defect prevention strategy in our country. 4. Conclusion The prevalence of neural tube defects in our paediatric environment, the pattern of lesions, the factors affecting the management and the outcome are comparable with those of Sub-Saharan African countries where data are available. Poverty, cultural beliefs and the insufficiencies of social organizations are limiting factors for management (only 26.09% of patients could afford modern treatment). Effective and efficient prevention policies (peri-conceptional folic acid supplementation) may contribute to reduce the treatment gap between the south and the north. Neural tube defects therefore constitute a major public health problem in sub-Saharan Africa by virtue of their high frequency and mortality rates, the severe

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