Craniofacial anomalies amongst births at two hospitals in Nairobi, Kenya

Int. J. Oral Maxillofac. Surg. 2012; 41: 596–603 doi:10.1016/j.ijom.2012.01.009, available online at http://www.sciencedirect.com

Clinical Study Craniofacial Anomalies

Craniofacial anomalies amongst births at two hospitals in § Nairobi, Kenya

A. Odhiambo, E. C. Rotich, M. L. Chindia, F. G. Macigo, M. Ndavi, F. Were University of Nairobi, School of Dental Sciences, Nairobi, Kenya

A. OdhiamboE.C. Rotich, M.L. Chindia, F.G. Macigo, M. Ndavi, F. Were: Craniofacial anomalies amongst births at two hospitals in Nairobi, Kenya. Int. J. Oral Maxillofac. Surg. 2012; 41: 596–603. # 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. The pattern of congenital oral and craniofacial anomalies (CFAs) in the Kenyan population remains unknown. The objective of this study was to describe the pattern of occurrence of CFAs at two hospitals in Nairobi. A descriptive crosssectional study at the Kenyatta National Hospital and Pumwani Maternity Hospital was carried out from November 2006 to March 2007. Mothers who delivered at the hospitals consented to an interview and physical examination of their babies within 48 h of delivery. The anomalies were classified for type and magnitude. Data were analysed to determine the association of these anomalies with ages of the mothers, gender, weight, birth order, mode of delivery and birth status of the babies. During the study period, 7989 babies were born. The CFAs manifested in 1.8% of the total births and were more common in female (1.4%) than in male (1.0%) live births. 12.8% of stillbirths had CFAs, with lesions manifesting more in males (16.7%) than in females (6.9%). The commonest CFA was preauricular sinus (4.3/1000) followed by hydrocephalus (1.9/1000) then preauricular tags and cleft lip and palate (1.5/ 1000 and 1.3/1000 total births, respectively).

Congenital craniofacial anomalies (CFAs) are rare.1 Cleft lip and or palate are the most common, occurring in 0.06–2.13/ 1000 live births.2 The highest incidence of cleft lip and palate has been reported in the Indian tribe of Montana (1:276), followed by oriental groups (1:500); the least affected are the negroid population (1:2000).3 Single minor anomalies occur § This was a dissertation submitted in December 2007 to the University of Nairobi, School of Dental Sciences, Department of Maxillofacial and Reconstructive Surgery, P.O. Box 69375, Nairobi.

0901-5027/050596 + 08 $36.00/0

in 14% of newborns4–6 whilst major congenital malformations are found in 2% of live births and 22% of stillbirths.6 The maternal age most involved in anomalies is 20–35 years and the pregnancies affected are mostly breech presentations and often the first born. A higher frequency of major anomalies occurs in multiple births than in single births and males have more malformations than females.7,8 Major congenital anomalies are amongst the leading causes of neonatal mortality, they contribute substantially to chronic disease morbidity, profoundly affect families and their management is expensive and

Key words: craniofacial anomalies; fresh stillbirths; macerated stillbirths; spontaneous vertex delivery; caesarean section. Accepted for publication 13 January 2012 Available online 2 March 2012

long term. Minor anomalies may be unwanted, cosmetically disfiguring and may be a sign of internal anomalies; hence the need to know their pattern of occurrence in any population. Classification of CFAs is usually based on new theories but it always remains controversial.1 Anatomical classification includes oral, nasal, aural, orbital, cranial and other organ/structural anomalies. A review of the published literature (Table 1) reveals a paucity of information on the pattern of occurrence of CFAs in most African populations including those in Kenya. The purpose of the present study was to document the pattern of occurrence

# 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Craniofacial birth anomalies

597

Table 1. An overview of studies that show incidence of CFAs. Anatomical classification of the anomalies

Author and year

Specific anomalies

Al-Omari et al. (2004),9 Cawson (1991),10 Dilley et al. (1991),11 Day (1984),12 Fasana (1980),13 Jain and Krogan (1983),14 Gorlin et al. (2000),15 Hunter and Roberts (1988),16 Iregbulem (1982),17 Kalter and Warkany (1983),18 Khan and Ivanov (1977),2 Moore and Persand (2003),5 Murray et al. (1997),19 O’Doherty (1975),26 Persaud (1979),20 Russell et al. (2004),3 Scheinfeld et al. (2004),21 Screan and Connor (1996),22 Stricker et al. (1990),1 Tomizawa et al. (1999),23 Wilson and Clarke (1977),24 Yadav (2001)25

Oral anomalies

Clefts,1,2,3,5,9–14,17,19,25 micrognathia, aglosia, agnathia, leukoedema,1,25 mucocoeles, cysts,12,20,22 pits/fistulae,21 epulides, lymphangioma, notch,11,23–25 natal/neonatal teeth,2,6,11,16,23 aglosia, ankyloglosia, macroglosia5,10,11,15,18,24,25

Chung and Myrianthopoulos (1975),7 Moore and Persand (2003),5 Fasana (1980),13 Kalter and Warkany (1983),18 Stricker et al. (1990)1

Ocular anomalies

Cyclopia,13 ethmocephaly, synophthalmia, microphthalmia, anophthalmia, cryptophthalmia microblepharon, microorbitism, euryblepharon, coloboma, blue sclera1,5,7,18

Fasana (1980),13 Gorlin et al. (2001),15 Kohelet and Arbel (2002)26

Nasal anomalies

Nasal aplasia with proboscis, choanal atresia, nasoschisis, nasal duplication1,13,18

Durakbasa et al. (2004),27 Fasana (1980),13 Moore and Persand (2003),5 Scheinfeld et al. (2004),21 Stricker et al. (1990),1 Wang (2001)28

Aural anomalies

Microtia, preauricular sinus,1,5,27 tags,5,28 fistulae,13,21 duplication of external auditory meatus, atresia, auricular hypoplasia5,21

Day (1984),12 Kalter and Warkany (1983),18 Mcintosh et al. (1954),4 Scheinfeld et al. (2004)21

Cranial anomalies

Acrania, microcephaly,4,12 macrocephaly,18 hydrocephalus,18,21 cranium bifidum18

Chung and Myrianthopoulos (1975),7 Fasana (1980),13 Moore and Persand (2003),5 Scheinfeld et al. (2004)21

Cutaneous anomalies

Congenital alopecia, random patches of white hair,13,21 absence of skin5,12

Most of these anomalies appear only as case reports in the literature.

of CFAs in two hospitals in Nairobi over a 4.5 month period. Material and methods

This survey was executed at the two largest government delivery centres in Nairobi, Kenya, after approval by the institutional review boards. The study population included all mothers who delivered and their babies. It was a descriptive cross-sectional study of incidence of clinically manifest CFAs at birth with the dependant variable being the presence of an anomaly. The demographic data documented included age of mother, birth status, mode of birth presentation, gender, birth weight, birth order and mode of delivery of each participating subject. The inclusion criteria entailed all births at 20 weeks or more of gestation and/or at least a 500 g birth weight. Only mothers with Kenyan citizenry were included in the study. Sample size was calculated using the Fisher et al. formula for population studies using the prevalence of single minor anomalies (14%) since these anomalies are the ones which have been widely reported on. All women admitted for delivery were requested to agree to an interview and

examination of their babies within 48 h of admission. An interview and examination form was used to document the demographic data and record findings from systematic examination of all births by midwives who had been trained by the principal investigator (PI) on how to complete the forms and how to perform a head to toe examination of the infants to elicit anomalies. The anomalies were classified by the structures involved, whether they were major or minor and whether single or multiple. Each centre was manned day and night. The PI visited the study sites daily and during each visit randomly picked the completed interview schedules at each centre and re-interviewed the mothers. The babies present were then re-examined. Informed consent was obtained from the mothers and confidentiality was ensured by use of in-patient numbers only. Whenever a case was delivered, the PI was alerted by mobile phone. Any infant with an anomaly transferred to the newborn unit before examination was examined whilst any stillbirth transferred to the mortuary before examination was followed by the investigator for examination and photography where indicated. Each malformation was counted once, such that if an infant had both cleft lip and ence-

phalocoele, it entered both classes for the tabulation of the number of infants with each anomaly born in the population. Parents with questions on malformations were counselled and educated by the investigators. Referrals for further management were made according to the rules of each hospital. Data analysis was done according to the statistical package for social sciences (SPSS) software version 12.0 and Epi Info packages. Results

During the study period there were 7989 new births: 4264 (53.4%) male, 3721 (46.6%) female and 4 (0.05%) with ambiguous external genitalia. 146 CFAs occurred in 1.8% of the total births. There were 7623 live births with CFAs in 1.3%. There were 366 (4.6%) stillbirths in the two hospitals, with 12.8% CFAs: 174 males, 188 females. Of these, 29 (16.7%) male and 13 (6.9%) female stillbirths had CFAs. Although there were more female stillbirths, the male babies had more anomalies than the females. The four babies with ambiguous external genitalia were all stillbirths and had major multiple CFAs (Table 2). The youngest mother was a 12-year-old primigravida

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whilst the oldest was 47 years. The mean age of the mothers was 25.2 years (mode 24 years); the median age was 25 years. Most anomalies occurred during the peak reproductive age of 20–24 years, producing 88 (34.4%) of the total anomalies. Extremes of ages (<15 and >35 years) were not significantly associated with anomalies. The peak birth weight was 3.0–3.9 kg. Peak anomalies were noted in the 2.0– 2.9 kg birth weight group. The smallest baby weighed 0.5 kg and the heaviest 5.4 kg. CFAs were common at birth weights above 2.5 kg (60.2%), whilst weight below 2.5 kg was significantly associated with whole body anomalies (Tables 3 and 4). Figure 1 shows the proportion of anomalies according to birth weight to rule out the idea that there were more anomalies in a certain weight group because more babies belonged to that group. 73.8% of the babies born with anomalies were between the first and the second birth order. There were no anomalies beyond sixth borns. To rule out the idea that the first borns seemed heavily laden with anomalies just because there were more first borns in this study, a proportionality test was done which showed that the sixth borns had the highest proportion of anomalies. Fisher exact test (Fisher exact test x2 = 0.27: 1df p > 0.05) was carried out to determine whether the first or sixth borns were the most predisposed to anomalies. It confirmed that first borns were significantly associated with anomalies (Fig. 2). The 146 CFAs were broadly classified into aural (65), orbital (15), cranial (35), oral (26) and isolated nasal anomalies (5). Aural anomalies (Table 5) formed 44.5% of the CFAs and occurred at a rate of 8.1/ 1000 of the total births, of which the preauricular sinus was the most common minor anomaly. Microtia (Fig. 3A and B) occurred in a spurt within 1 week, ranging

Table 2. Distribution of births by hospital, gender and birth status. Centre

Total births

PMH

5579

KNH

2410

Total

7989

Frequency of anomalies

Gender

Live births

*

*

Stillbirths

M – 3002 F – 2575 AEG – 3 M – 1262 F – 1145 AEG – 1

141( 85)

5395( 44)

184(*41)

115(*61)

2228(*55)

182(*6)

M – 4264 F – 3721 AEG – 4

256(*146)

7623(*99)

366(*47)

* CFAs.M – males, F – females, AEG – ambiguous external genitalia. Outside the bracket in anomalies column is whole body anomalies.

Table 3. Tests of significance for birth weight, ages of mothers and birth order in association with anomalies. n per group

Variable

Normal Birth weight <2.5 kg >2.5 kg Birth order First borns Others Mothers’ age <35 years >35 years

Odds ratio

95% confidence

p value

Anomalous

1399 5931

113 134

3.58

2.74–4.66

*

3195 4423

139 106

1.82

1.40–2.37

*

6661 418

208 14

1.07

0.59–1.90

0.05 (0.912)

0.000 (p < 0.01) 0.000 (p < 0.01)

*

Significantly associated with occurrence of anomalies. Not recorded (NR) values reduced the n per a class.

from the most severe (type III) to the least severe (type I). It was common in males and the right ear was the most involved. The syndromic low-set ear was noticed in 2 infants who had Down’s syndrome (0.3/ 1000 births): this occurred in conjunction with posteriorly oriented ears, webbed neck and mongoloid slanting palpebral fissures. Imperforate external auditory meatus, preauricular cyst and atretic ears had the least incidence. Preauricular sinus was common on the right, was single in all instances except in one case whereby two sinuses occurred with one superior to the other, both along the preauricular crease

above the tragus. It occurred 1.5 times more in females than in males. Preauricular tags showed a preponderance for the right side with no gender predilection and both the sinuses and tags were observed more in live births. Preauricular tags were mostly single and ranged from negligible skin elevations to large ones interfering with cosmesis. Some were the same colour as the surrounding skin whilst others were darker or lighter: they coexisted with normal and abnormal helices in equal proportions. In one instance, three preauricular tags of different colours and sizes were observed anterior to the auricle (Fig. 3D).

Table 4. Distribution of anomalies according to birth weight of babies. Baby

Birth weight of babies (kg)

Centre NR

0.1–0.9

1.0–1.9

2.0–2.9

3.0–3.9

4.0 and above

Mean weight (SD)

Normal

KNH

204

71

196

663

1086

100

2.7 (0.81)

Abnormal

PMH KNH-WB

217 8

23 0

210 31

1586 41

3200 30

178 4

3.04 (0.548) 2.53(0.82)

0 5

18 22

17 25

58 58

36 25

12 11

2.52 (0.98)

429

112

454

2348

4352

294

PMH-WB CFAs KNH + PMH Total *

Statistical tests 2

x = 207.09; 4df *p < 0.01 (0.000) x2 = 25.09; 4df *p < 0.01 (0.000)

7989

p significant differences at a = 0.01.WB – whole body anomalies include CFAs. KNH, Kenyatta National Hospital; PMH, Pumwani Maternity Hospital.

Craniofacial birth anomalies n = 7989 16.07%

10.57%

4.26% 1.52% 0.37%

0.1-0.9kg

1.0-1.9kg

2.0-2.9kg Birth weight

3.0-3.9kg

4kg & above

Fig. 1. Proportions of anomalies according to birth weight (n = 7989). n = 7989 4.8% 4.2%

4.1%

2.2%

2.2%

0.0% 1st born

2rd born

3rd born

4th born

5th born

0.0% 0.0% 0.0% 6th born

7th born

8th born

9th born

0.0% 0.0% 10th born

11th born

Fig. 2. Proportions of anomalous babies by birth orders (n = 7989).

Orbital anomalies formed 10.25% of the CFAs. Microphthalmia (Fig. 4B) was the most common at 0.5/1000 births, followed by hypertelorism at 0.3/1000 births (Fig. 4B). Hypotelorism (Fig. 4A), ankyloblepharon (Fig. 4C), mongoloid slanting palpebral fissures (Fig. 4B), cyclopia (Fig. 4D) and congenital glaucoma were noticed in 0.1/1000 births. Cranial anomalies (Table 6) occurred in 4.4/1000 births and constituted 23.3% of CFAs. Hydrocephalus (Fig. 5A) had the highest prevalence followed by anencephaly (Fig. 5B), then pseudencephaly (Fig. 5B). Hydrocephalus occurred at an equal rate in the live and stillbirths with no

gender predilection. Anencephaly also had no gender predilection but was incompatible with life in all cases. Pseudencephaly was associated with anencephaly and was posterior in 0.03%; anterior and vertex pseudencephaly comprised 0.01% of the cases. Scaphocephaly was common in females; triphyllocephaly (Fig. 5D), failed fusion of sutures, macrocephaly, plagiocephaly (Fig. 5C), turicephaly, clinocephaly and bulging fontanelles had a male predilection with a male to female ratio of 1.5:1. Congenital alopecia (Fig. 5E) occurred at a prevalence rate of 0.1/1000 births. Oral anomalies occurred in 3.0/1000 births and formed 16.44% of CFAs. Clefts

Table 5. Distribution of aural anomalies by type. Aural anomalies

Frequency

Preauricular sinus Preauricular tag Microtia Low-set ears Imperforate Ex. auditory meatus Atretic ears Posteriorly oriented ears Preauricular cyst

34 12 6 6 3 2 1 1

Total

65

% of anomaly 54.8 19.4 9.7 9.7 1.6 1.6 1.6 1.6 100

% in total births

Incidence/1000

0.43 0.15 0.08 0.08 0.04 0.03 0.01 0.01

4.3 1.5 0.8 0.8 0.4 0.3 0.1 0.1

0.81

8.1

599

of the lip and palate (Fig. 6A) in combination formed the highest oral anomaly with a prevalence rate of 1.3/1000 births. Clefts of the hard palate occurred at 0.5/1000 births followed by high arched palate at a prevalence rate of 0.4/1000 births. In about three-quarters of the cases, the clefting was bilateral, involving females and males equally. Gingival cysts (Fig. 6B), natal teeth and micrognathia had no gender predilection and occurred at a rate of 0.3/1000 births. Gingival cysts had a predilection for the mandibular posterior ridge whilst natal teeth were in the mandibular incisor region. One baby had only one tooth which was firmly attached, whilst the other had two natal teeth, loosely attached by a soft tissue pedicle and could be moved in any direction by the tongue. Macrostomia, glossoschisis (Fig. 4B), alveolar notch (Fig. 6C), macroglosia, inferior ankyloglosia, congenital epulides and congenital ranula (Fig. 6D) had prevalence rates of 0.1/1000 births each. Isolated nasal (not associated with clefts) anomalies had a prevalence rate of 0.4/1000 births. All occurred in association with other anomalies and were seen in stillbirths. Depressed nasal bridge, depressed alae nasi and nasal aplasia with proboscis, each occurred in 0.1/1000 births. The proboscis occurred together with cyclopia (Fig. 4D) whilst the depressed nasal bridge and alae nasi occurred with severe scaphocephaly, atretic low-set ears, short neck and bilateral talipes. In this study major CFAs occurred at a rate of 6.1/1000 births. Hydrocephalus was the most common (1.9/1000 births) followed by anencephaly and pseudencephaly at 0.08% and 0.06% respectively (Table 2). 65.3% of the infants with major CFAs were stillbirths, and 85.7% had multiple anomalies. The rate of occurrence of major CFAs had no gender predilection, but only 8.2% males with major CFAs were live births compared to 26.5% females. Minor CFAs occurred at a rate of 11.5/1000 births with aural anomalies being the most common in 8.0/1000 of the total births and 66.0% of minor anomalies. Microphthalmia was the commonest minor orbital anomaly in 0.5/1000 total births and formed 4.4% of the minor CFAs. This was followed by high-arched palate at a prevalence rate of 0.4/1000, and constituted 3.3% of the minor CFAs. About 66.0% of the minor CFAs were isolated cases but 34.8% occurred with other anomalies. Of the ones with multiple anomalies 32.3% were associated with major anomalies and 67.1% with other minor anomalies.

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Fig. 3. Aural anomalies. (A) Right microtia II. (B) Right microtia I. (C) Left preauricular sinus and bilateral ulnar polydactyly. (D) Right preauricular tags.

Discussion

This study, like those of Scheinfeld et al.21 and Kohelet and Arbel,26 found preauricular sinus to have been the most common minor CFA in 4.3/1000 births. Preauricular sinus was common on the right in females (F:M = 1.5:1) and was unilateral or bilateral. When unilateral they were commonly single and just anterior to the root of the helix. Preauricular tags, as reported by Durakbasa et al.27 showed preponderance to the right; they were more prevalent in males than in females (2:1) and bilateral in 50% of cases. In one of the cases in this series there were bilateral multiple preauricular tags of different sizes and colours which could imply different soft tissue contents, hence a varying origin. As has been reported by various investigators,18,21 microtia was common in males and on the right. In bilateral cases the ears were either equal in size or different, with the right auricle being smaller or more distorted than the left one, and other aural anomalies such as preauricular sinus and imperforate external auditory meatus coexisted with microtia in about half of the cases. Non-syndromic microtia

in this study occurred at a prevalence rate of 0.2/1000 live births which concurred with the prevalence rate reported by Scheinfeld et al.21 In Lusaka, Zambia, Khan and Ivanov2 carried out a 6-month study of single observations for congenital malformations on 8505 children born at the university hospital and found an overall incidence of anomalies of 17.6/1000 births. Amongst the anomalous children 16.5/1000 live births had single malformations whilst multiple malformations occurred in 1.1/1000 neonates, with the highest incidence occurring in first or second borns and in children born to mothers aged 19–30 years. The birth order and maternal age most involved compared well with the present study but no classification of the anomalies into minor and major was done, hence the difficulty in comparing those results with the present ones. Clefts of the lip and palate occurred in 1.3/1000 of the total births. Of these, 90% were bilateral cleft lip and palate and 10% were left sided incomplete cleft lip. Only 30% of these babies were live births. This agrees with other reports in the literature.2,14 Half of the stillbirths with cleft lip and palate were also anencephalic

in this series. Cleft lip and palate had a male to female proportion of 3:1 whilst the high arched palate was observed solely in females. The prevalence of gingival cysts in this series was lower than the 11% reported by Dilley et al. but the prevalence of natal teeth (0.3/1000) was similar to that reported by Dilley et al.11 Intraoral anomalies showed a low prevalence rate (0.01%) compared to other reports in the literature11,16 probably because they were missed due to the one-off examination within hours of delivery or they could have been less prevalent in this community. As in a previous study,19 which reported that congenital anomalies were common in the underweight (2.5 kg) this study found that malformations occurred significantly in underweight babies. The peak delivery age of 20–24 years compared well with other studies7,8,30 but younger mothers gave birth to malformed babies. This series reports an anomalous male:female ratio of 1.5:1, which compares well with the previous reports.7,8,31 74.1% of babies born with defects were in the first and second birth order as in earlier studies.2,7,8 Hay et al.8 and Chung et al.7 found that congenital

Craniofacial birth anomalies

601

Fig. 4. Orbital anomalies. (A) Hypotelorism, depressed nasal bridge, adherent ears with imperforate external auditory meati, short upper lip. (B) Microphthalmia, upward slanting palpebral fissures, wide depressed nasal bridge, glossoptosis, hypertelorism in a macerated stillbirth. (C) Ankyloblepharon. (D) Proboscis with cyclopia and microstomia.

malformations tended to increase with advanced maternal age but this series and that of Fletcher31 did not find any significant difference in the effect of increased maternal age in the overall incidence of congenital malformations. Illesanmi et al.,32 in their Nigerian study of elderly primigravidae, did not find any

association of anomalies with advanced maternal age. This may mean that African mothers who deliver babies with anomalies are relatively younger than non-Africans but it could also be due to the fact that African women give birth earlier. A multicentre series would be required to reach a definite conclusion.

Table 6. Distribution of cranial anomalies. Cranial anomalies

Frequency

Hydrocephalus Anencephaly Pseudencephaly Trigonocephaly Wide open sutures Scaphocephaly Plagiocephaly Triphyllocephally Clinocephaly Macrocephaly Bulging fontanelle

15 6 5 1 1 2 1 1 1 1 1

Total

35

% anomaly in class 42.9 17.1 14.3 2.9 2.9 5.7 2.9 2.9 2.9 2.9 2.9 100

% of total births

Incidence/1000

0.19 0.08 0.06 0.01 0.01 0.03 0.01 0.01 0.01 0.01 0.01

1.9 0.8 0.6 0.1 0.1 0.3 0.1 0.1 0.1 0.1 0.1

0.44

4.4

Nasal aplasia with proboscis in this series was only 1 (1.0/10,000) out of total births, producing a higher incidence in this series than that reported by Moore and Persand5 of 1:100,000 newborns. It may be that no others would have been encountered in the next several births to concur with Moore and Persand’s observation. The prevalence of hydrocephalus was 1.9/1000, giving twice the prevalence rate of 0.4–0.8/1000 total births reported by other investigators.18,21 Anencephaly had no gender predilection and was incompatible with life. As reported by O’Doherty6 the incidence of anomalies in stillbirths in this study was high (12.8%) but did not reach the 22% that he reported. There was no follow-up in this study and some of the malformations could have been missed due to the one-off examination. In summary, the epidemiology of CFAs in the Kenyan population correlates well with that in the rest of the world. In Kenya these anomalies are being managed by local and visiting plastic surgeons, oral

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Fig. 5. Cranial anomalies. (A) Hydrocephalus. (B) Anencephaly, pseudencephaly, cup ears, head fused to thorax. (C) Plagiocephaly. (D) Triphyllocephaly, macrostomia (the right eye was traumatized in the mortuary). (E) Congenital alopecia, depressed nasal bridge and an imperforate external auditory meatus.

Fig. 6. Oral anomalies. (A) Bilateral cleft lip and palate. (B) Gingival cyst. (C) Alveolar notch. (D) Congenital ranula.

Craniofacial birth anomalies and maxillofacial surgeons, ear, nose and throat surgeons and general surgeons. There is no protocol for managing these cases and no follow-up, especially for patients operated on during the free surgical camps. The treatment outcomes of all these surgeons can never be the same because of differences in experience and treatment protocols. Long term follow-up of these patients until facial growth has ceased, together with an aesthetic evaluation may elucidate components that would help to improve the final treatment outcome of these patients and the quality of life of those children undergoing surgery at outreach camps. In economically endowed regions, management of CFAs is usually multidisciplinary involving genetic counsellors, paediatricians, plastic surgeons, oral and maxillofacial surgeons, specialist nurses, ear nose and throat surgeons, orthodontists, speech therapists and prosthodontists who may work in specific CFA centres. Funding

It was funded by the Kenya Navy. Competing interests

None. Ethical approval

This study was approved by the Kenyatta National Hospital – University of Nairobi and the Pumwani Maternity Hospital Ethics, Research and Standards Committees (KNH-UON ERC/01/3857 and PMH/ DMOH/84/34) respectively. Informed consent was obtained from the mothers and confidentiality was ensured by use of in-patient numbers only. Acknowledgements. The cooperation of the administrative organs of the Kenyatta National Hospital and the Pumwani Maternity Hospital is highly appreciated. Finally, sincere gratitude is due to Dr. Alice Lakati for her most diligent guidance during data analysis. References 1. Stricker M, Van Der Meulen JG, Raphael B, Tolhurst DE, Murray EJ, editors. Craniofacial malformations. Edinburgh: Churchill Livingstone; 1990. p. 149–309.

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