Papillon-lefevre syndrome: review of literature and report of three cases in the same family.

AIMS AND OBJECTIVES: The objective of this paper is to give a review of the literature on the aetiopathogenesis, clinical presentation, differential diagnosis and treatment of Papillon-Lefevre Syndrome, and present three cases of PLS in siblings of the same parents demonstrating typical clinical and radiological features of the condition. CASES: Three cases of Papillon-Lefevre syndrome affecting three girls among six siblings of normal parents who are first-degree cousins. The three siblings aged 14, 6 and 4 ⅇ years all had severe gingivitis, periodontitis with grossly mobile teeth and tooth migration. Other findings included premature exfoliation of deciduous teeth (2/3), premature loss of permanent teeth in one sibling. Plantar hyperkeratosis was detected in all of the cases, leading to a difficult and painful walking in two cases due to lesions on the soles. Palmar hyperkeratosis was present in the oldest sibling as well. Radiologic study confirmed alveolar bone destruction in two cases. CONCLUSION: Early recognition of this entity as well as multidisciplinary management may help in the prognosis of these cases.

Prevalence of orofacial clefts in Nigeria.

Orofacial clefts are the most common malformations of the head and neck. In Africa, orofacial clefts are underascertained, with little or no surveillance system in most parts for clefts and other birth defects. A Nigerian craniofacial anomalies study, NigeriaCRAN, was established in 2006 to support cleft research specifically for epidemiological studies, treatment outcomes, and studies into etiology and prevention. We pooled data from seven of the largest Smile Train treatment centers in the six geopolitical zones in Nigeria. Data from September 2006 to June 2011 were analyzed and clefts compared between sides and genders using the Fisher exact test. A total of 2197 cases were identified during the study period, with an estimated prevalence rate of 0.5 per 1000. Of the total number of orofacial clefts, 54.4% occur in males and 45.6% in females. There was a significant difference (P = .0001) between unilateral left clefts and unilateral right clefts, and there was a significant difference (P = .0001) between bilateral clefts and clefts on either the left or right side. A significant gender difference (P = .03) was also observed for cleft palate, with more females than males. A total of 103 (4.7%) associated anomalies were identified. There were nine syndromic cleft cases, and 10.4% of the total number of individuals with clefts have an affected relative. The significant difference between unilateral clefts and the gender differences in the proportion of cleft palate only are consistent with the literature. The present study emphasizes the need for birth defects registries in developing countries in order to estimate the exact prevalence of birth defects including orofacial clefts.

Awareness, knowledge and attitude on cleft lip and palate among antenatal clinic attendees of tertiary hospitals in Nigeria.

BACKGROUND: Adequate knowledge and awareness of cleft lip and palate (CLP) deformity may help to counter the negative beliefs and attitudes toward the condition. The objective of this study was to assess the level of awareness, knowledge and attitude of women attending antenatal clinics about CLP. MATERIALS AND METHODS: A cross-sectional descriptive study with the aid of a structured interview administered questionnaire was conducted among 200 women attending antenatal clinics in three Federal Government Teaching Hospitals in the Northern and Southwestern regions of Nigeria. The main outcome measure was the level of awareness and the mean cumulative knowledge score. RESULTS: The mean age of the subjects was 28.9 ± 5.1 years (age range: 16-42 years). Half of the women (50.5%) reported that they had seen or heard about CLP. The mean cumulative knowledge score was 6.9, with only 19.8% having adequate knowledge. Many respondents had neither read an article on CLP nor participated in any public enlightenment program, and 31.5% indicated that they would like to know more about the condition. Level of educational attainment had a statistically significant effect on the level of awareness and knowledge on CLP, as more educated respondents tend to be more aware and knowledgeable ( P < 0.001). CONCLUSION: There is need for increased public enlightenment/health education to increase awareness and subsequently help develop more positive attitudes toward children with CLP. Such programs should include distribution of pamphlets on CLP at clinics, especially antenatal clinics, media campaigns on radio, TV and newspaper as well as establishment of cleft support groups by the relevant governmental and professional organizations.

Effectiveness of buffered and non-buffered local anaesthetic in inferior alveolar nerve block: a randomised study.

The objective of this study was to compare the effectiveness of buffered and non-buffered local anaesthetic (LA) following inferior alveolar nerve block (IANB). This study was conducted at Usmanu Danfodiyo University Teaching Hospital Sokoto from June 2020 to January 2021. Subjects were randomised into Groups A and B. Group A received 2 ml of freshly prepared 2% lignocaine with 1:100,000 adrenaline buffered with 0.18 ml of 8.4% sodium bicarbonate solution while those in Group B received non-buffered 2% lignocaine with 1:100,000 adrenaline LA solutions. The onset of action of the LA was assessed by subjective and objective methods, while pain at the injection site was assessed using a numerical rating scale. Data obtained were analysed using statistical package for social sciences (IBM SPSS) version 21. The mean (SD) ages for Groups A and B were 37.4 (14.9) and 40.1 (14.4) years, respectively. The mean (SD) onset times of the LA by subjective testing were 126 (31.7) and 201 (66.8) seconds for Groups A and B, respectively. Similarly, the mean (SD) onset times of the local anaesthesia by objective testing for Groups A and B were 186 (41.0) and 287 (85.0) seconds, respectively, and both were significant (p<0.001). Pain at the injection site assessed objectively and subjectively were also statistically different (p<0.001). The findings of this study suggest that buffered LA is more effective than non-buffered LA of the same composition when used for IANB particularly concerning a significantly faster rate of onset and less pain at the injection site.

Genetic studies in the Nigerian population implicate an MSX1 mutation in complex oral facial clefting disorders.

BACKGROUND: Orofacial clefts are the most common malformations of the head and neck, with a worldwide prevalence of 1 in 700 births. They are commonly divided into CL(P) and CP based on anatomic, genetic, and embryologic findings. A Nigerian craniofacial anomalies study (NigeriaCRAN) was set up in 2006 to investigate the role of gene-environment interaction in the origin of orofacial clefts in Nigeria. SUBJECTS AND METHODS: DNA isolated from saliva from Nigerian probands was used for genotype association studies and direct sequencing of cleft candidate genes: MSX1 , IRF6 , FOXE1, FGFR1 , FGFR2 , BMP4 , MAFB, ABCA4 , PAX7, and VAX1 , and the chromosome 8q region. RESULTS: A missense mutation A34G in MSX1 was observed in nine cases and four HapMap controls. No other apparent causative variations were identified. Deviation from Hardy Weinberg equilibrium (HWE) was observed in these cases (p = .00002). A significant difference was noted between the affected side for unilateral CL (p = .03) and bilateral clefts and between clefts on either side (p = .02). A significant gender difference was also observed for CP (p = .008). CONCLUSIONS: Replication of a mutation previously implicated in other populations suggests a role for the MSX1 A34G variant in the development of CL(P).

A survey of facial nerve dissection techniques in benign parotid surgery among maxillofacial and ear, nose, and throat surgeons in Nigeria.

OBJECTIVE: The aim of the present study is to identify the facial nerve dissection technique routinely used during parotidectomy for benign parotid tumors by Nigerian Oral and Maxillofacial (OMF) and Ear, Nose, and Throat (ENT) Surgeons. MATERIALS AND METHODS: A questionnaire-based study was conducted among Oral and Maxillofacial and Ear, Nose, and Throat Surgeons in Nigeria, on their experience with antegrade and retrograde facial nerve dissection techniques in parotid surgery. The respondents were asked to indicate their choice of dissection techniques in revision parotidectomy, limited superficial parotidectomy, and in obese patients with large tumors. They were also asked to indicate if they routinely used perioperative facial nerve monitoring devices in parotid surgery for benign tumors. RESULT: About half (47.5%) of them routinely used the antegrade technique, while only a few (12.5%) used the retrograde technique. A large number of them (40%), however, used a combination of antegrade and retrograde routinely. Technical ease was the main reason for the choice of technique. The antegrade technique was the technique of choice by most respondents for revision parotidectomy (60%) and limited superficial parotidectomy (62%). However, the retrograde approach was the technique of choice by most of them (47%) in case of parotidectomy in obese patients with large tumors. The routine use of perioperative facial nerve monitoring devices is an uncommon practice among OMF and ENT surgeons in Nigeria. CONCLUSIONS: The antegrade approach for facial nerve dissection is the most common technique used in parotid surgery by Nigerian OMF and ENT surgeons. Nigerian surgeons need to consider the retrograde approach in selected cases of parotid surgery especially for localized tumors that are amenable to limited superficial parotidectomy. Inclusion of perioperative facial nerve monitoring devices is also advocated.

Challenges of cleft care in Africa.

Professionals involved in the care of patients with clefts in Africa face unique challenges when compared with what obtained in western countries. It is the purpose of this paper to review some of the challenges of cleft care in Africa. A computerized literature search using electronic databases; Ovid MEDLINE, EMBASE and AJOL databases was conducted for published articles from 1965 to 2006. Mesh phrases used in the search were: Cleft lip, Cleft palate, Cleft care, Management, Challenges and Africa. The search produced 46 articles. A critical evaluation of the 46 articles using the inclusion and exclusion criteria led to selection of 20 articles for the review. The findings were: (1) No reliable data on incidence of cleft; (2) Strong traditional beliefs system exist regarding etiology of cleft; (3) Health problems attributed to clefts common at presentation; (4) Patients with cleft commonly present late; (5) Lack of multidisciplinary approach to care. The chance that a child born with a cleft tomorrow in Africa will receive the best care possible can not yet be guaranteed. The need to formulate basic strategies to improve the standard of cleft care in Africa and the rest of the developing world is overdue. We hope that the findings in this review will provoke solutions that might ultimately improve the standard of cleft care in Africa and the rest of the developing world.

A study of clinical teachers' attitude to teaching and perceived learning needs in a medical college in Nigeria.

BACKGROUND AND PURPOSE: Debates on medical educational reform initiatives in Nigeria have gradually shifted from access to medical colleges to improving learning quality. Understandably, clinical teachers are being encouraged to acquire basic teaching pedagogical knowledge through learning activities. Considering the fact that the concept of faculty development for medical teachers' is still evolving in Sub-Saharan Africa, this study aims to explore clinical teachers' attitude to teaching and perceptions of continuing education in teaching knowledge and skills in a medical college in Nigeria. METHODS: Using a mixed-method research approach, quantitative data were collected from a sample of teachers through survey questionnaires, and qualitative data were obtained through face-to-face individual semi-structured interviews of teachers from the same institution. RESULTS: Sixty-one survey questionnaires (response rate of 88%) and 10 interviews were completed and analyzed. Findings revealed that teachers' derived satisfaction from teaching and maintain a strong commitment to teaching. Bedside teachings (64%) and lectures (21%) were reported to be the most frequent mode of teaching. Although four out of every five respondents (80%) reported not having previous training in teaching, a large proportion (97%) self-assessed their teaching abilities to be average or above average, with most indicating that the experience of teaching observed during undergraduate medical training may be sufficient preparation for their teaching roles. The majority of the teachers' were of the opinion that there is a need to improve their individual teaching skill. However, in the absence of formal faculty development programs in the college, most of the teachers indicated that their teaching skills are currently being improved through sporadic informal community of practice involving interested colleagues and modeling identified good teachers. CONCLUSION: A catalyst of learning by teachers' may come from their belief and self-rating of teaching ability. In this under-resourced context, explicit classifications of existing informal learning opportunities coupled with greater institutional support could improve teaching and teachers' development.

Cultural and religious components in Nigerian parents' perceptions of the aetiology of cleft lip and palate: implications for treatment and rehabilitation.

The present study was conducted to find out the perceptions of mothers from two Nigerian ethnic groups who had children with cleft lip and palate (CLP) about the aetiology of the defect. Mothers of 16 children with CLP from the Yoruba ethnic group who attended the maxillofacial clinic of the Obafemi Awolowo University teaching hospital in southern Nigeria and 20 children with CLP from the Hausa/Fulani ethnic group who attended the maxillofacial clinic of the University of Maiduguri teaching hospital in northeastern Nigeria were interviewed over an 8-month period. We used standardised interviews including questions with ethnographic components to allow us to collect information about traditional beliefs about clefts. Interviews were recorded on tape for later analysis. Thirteen of the 16 parents from the Yoruba group attributed the aetiology of CLP to supernatural forces (evil spirits and ancestral spirits), while 16 of the 20 Hausa/Fulani parents attributed it to the "will of God". Twelve of 16 Yoruba parents had consulted traditional healers for treatment before coming to the hospital. Various plants and animal products were used to treat clefts and 10 of the Yoruba parents were referred to the hospital for further treatment by the traditional healers. Cultural and religious factors seem to have an important role in the explanations, labels and treatment that followed the birth of a child with CLP in this environment. There is a need for greater collaboration and sharing of information between modern medical practitioners and traditional healers.

Pierre Robin syndrome: an update.

OBJECTIVES: 'Pierre Robin' is one of the most readily recognised eponyms in medicine, yet it is a poorly understood nonspecific grouping of malformations that has no prognostic significance. Previously known as 'Pierre Robin syndrome', the way this diagnostic entity is viewed is now undergoing change. It is the purpose of this paper to review previous thinking about Robin and provide an update on recent clinical observations. SOURCES OF DATA: A computerised literature search using MEDLINE, EMBASE, AJOL and OMIM was conducted for published articles up to March 2006. Mesh phrases used in the search were: Pierre Robin syndrome, Robin anomalad and Robin sequence (RS). RESULTS: This relatively uncommon association of micrognathia with cleft palate and upper airway obstruction which was initially thought to be a specific disease and entire treatment regimens established to deal with presumed problems is now understood to be a grouping of clinical findings that does not represent a distinct multiple anomaly syndrome. The condition is therefore now described as 'Pierre Robin sequence'. Evidence of distinct cytogenetic anomalies has also highlighted the aetiological heterogeneity associated with RS in recent times. CONCLUSION: Infants with Robin sequence can present with varied problems, some of them emergencies. Clinicians must be aware of the high prevalence of associated syndromes and the possible contribution of other syndromic features to the problems for proper patient care. Candidate loci and potential candidate genes are currently being proposed in the literature for RS.

A survey of temporomandibular joint dislocation: aetiology, demographics, risk factors and management in 96 Nigerian cases.

A retrospective study of 96 cases of temporomandibular joint dislocation was undertaken. Patients' ages ranged from 9 to 85 years (mean+/-SD, 35.3+/-17.4 years) and peak incidence was at 20-29 years. Mean duration was 7.9 weeks (range, 1h to 3 years). Acute, chronic and recurrent dislocations were seen in 46 (47.9%), 29 (30.2%) and 21 (21.9%) patients, respectively. Males dominated in all three categories but this was not statistically significant (P = 0.8). Excessive mouth opening while yawning (44 cases) was the commonest cause of dislocation, followed by road traffic accidents (13 cases). Ten patients (10.4%) had an underlying systemic disease, the commonest being epilepsy (four cases); those with acute dislocation recorded the highest incidence of underlying illness. Bilateral anterior (86 cases) dislocations were the most frequent. Of the 96 patients, 89 (92.7%) were available for treatment. Manual reduction with or without anaesthesia proved effective for 38/45 acute, 5/24 chronic and 14/20 recurrent cases. Chronic dislocations were treated mainly by surgical osteotomy (13/24). Vertical subsigmoid and oblique ramus osteotomies were the commonest surgical techniques recorded. Treatment was satisfactory for all patients surgically handled except for one case of anterior open bite postoperatively. This study has shown that excessive mouth opening while yawning is the commonest cause of temporomandibular joint dislocation in Nigerians, and conservative approaches to management remain quite effective irrespective of the duration and clinical subtype. The best choice of surgical technique should be determined by proper clinical evaluation and the need to avoid or minimize postoperative morbidity.

An unusual bilateral oblique facial cleft in a newborn.

An extremely rare case of asymmetrical bilateral oblique facial cleft presumably secondary to amniotic bands is presented. The cleft on the right side of the patient commenced from the lower lip through the maxillary region of the face to the temporal area and was described as a lower oro-temporal cleft to differentiate it from the previously reported type commencing from the upper lip.

Penetrating arrow injuries of the maxillofacial region.

We operated on four civilian patients who had unusual penetrating maxillofacial injuries that resulted from assault with arrows. The patients were fully conscious at the time of presentation. Recovery was uneventful in three, but one died.

Incidence and aetiology of oral clefts: a review.

Craniofacial anomalies, most especially cleft lip and palate, are major human birth deformities with a world wide incidence of 1 in 700 and associated substantial clinical and psychosocial impact. Wide ranges of studies in developmental biology have shown that both genetics and environmental factors are involved in the etiology of oral clefts. However, genetics of cleft lip alone or accompanied by cleft palate, are different from those of isolated cleft palate. The prevention of oral clefts is not possible without knowing the precise etiology. Genetic counseling can now identify high risk families; the clefts themselves may be visible at 20 weeks gestation, but beyond early identification, we can only look into the future on the possibility of preventing oral clefts. This article reviews the available literature on the gene-environment contributions to nonsyndromic forms of clefting and their implication for possible preventive measures.

Assault cases in Maiduguri metropolis: A comparative study of police and accident and emergency units' records.

BACKGROUND: Information on the incidence of assault cases such as armed robbery, fights and house burglaries in most Nigerian cities are usually based on data collected from police records with little or no attempt to tap medical sources of information. OBJECTIVE: The aim of this study was therefore to compare the rate of recording of assault cases in the Maiduguri metropolitan area by the police at the Maiduguri central police station and the Accident and Emergency unit (A and E) of the University of Maiduguri Teaching Hospital. Possible reasons why assault victims report or do not report to the police before seeking for treatment was also investigated. METHOD: All victims of assault who attended the A and E unit of the hospital over a one year period (2002-2003) were interviewed and relevant data collected in a prepared form. Findings were compared with the record of assault cases within the same period at the Maiduguri central police station. RESULT: 185 patients presented at A and E with body injuries due to assault during period of study. 108 (58%) informed the police before attending hospital, out of which majority (38.9%) claimed an 'instinctive reaction to do so' as the reason for informing the police. A significant proportion (61%) of those that did not inform the police claimed that the feeling that 'nothing will come out of it' as the reason. Only 41% of cases reported to the police were recorded. There was a tendency by the police to record cases where gun was used or the victim was a female. CONCLUSION: The true picture of violence in most Nigerian cities can better be assessed by collecting data from both the relevant A and E department as well as from the police. The need for the populace to develop confidence and trust in the police cannot be overemphasised.

Plunging ranula: a report of two cases.

Plunging ranula, a rare differential diagnosis of cervicofacial masses is presented in two Nigerian patients. The authors highlight the pathophysiology and treatment methods of this condition. It is suggested that clinicians be aware of the various modes of presentation and natural history of the disease to facilitate prompt diagnosis and appropriate treatment.

Cervicofacial actinomycosis after partial mandibular resection: case report.

An unusual case of cervicofacial actinomycosis in a 60-year old male Nigerian is reported. The patient had had partial mandibular resection for plexiform ameloblastoma one year earlier with insertion of Kirschner wire to serve as a splint. The authors highlight the possible pathophysiology of this condition. It is suggested that clinicians be aware of the various modes of presentation and natural history of the disease to facilitate prompt diagnosis and appropriate treatment. This will assist in forestalling the numerous complications that may result from actinomycosis.

African oral histoplasmosis mimicking lip carcinoma: case report.

A case of localised African histoplasmosis with an unusual presentation in a 56 year old Nigerian farmer is reported. The lesion presented as an ulcer clinically mimicking squamous cell carcinoma of the lower lip. An incisional biopsy and culture studies confirmed African histoplasmosis and the ulcer healed spontaneously without treatment. This case is reported to highlight the unusual location and clinical course of African histoplasmosis.