Paget's disease is a metabolic disorder of bone caused due to defect in the remodelling process and is very common in western countries but is very rare in Asians and Africans. It was first described by a British scientist Sir James Paget in 1877. It can be monostotic or polyostotic depending on the number of bones involved. It most commonly affects older people of more than 50 years. Disease involvement can be symptomatic or asymptomatic depending on the extent of the disease process. Diagnosis of Paget's disease can be made by raised serum alkaline phosphatase levels, radiological examination and by radioisotope bone scans.
Branchial cleft anomalies result from abnormal persistence of branchial apparatus, which is located at the lateral part of the neck. These occur due to failure of obliteration of the branchial apparatus during embryonic development. Differential diagnoses of lateral neck mass are salivary gland or neurogenic neoplasms, paragangliomas, adenopathies, cystic hygroma or cystic metastasis from squamous cell carcinoma or thyroid papillary carcinoma. Clinically, a branchial cyst is smooth, round, fluctuant and non-tender, and usually occurs over the upper part of the neck, anterior to the sternocleidomastoid muscle. Sometimes, it may present as infected cyst (or abscess), a sinus or fistula. Surgical excision is the definitive treatment for branchial anomalies. The objective of the work was to study the demographic data, clinical presentation, definite diagnostic workup and treatment of patients diagnosed with branchial anomalies. This is a retrospective study of 26 patients who were diagnosed with branchial anomalies (branchial cyst and fistula), of which only 12 patients had data available between July 1999 and June 2009 at the Otorhinolaryngology, Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Centre. Twelve cases of branchial anomalies were seen, in which 10 patients had second branchial cyst anomalies, 1 had third branchial fistula and 1 had bilateral branchial lesion. There were seven females and five males. The age of the patients varied over a wide range (4-44 years), but the majority of the patients were in their second and third decade of life. All branchial anomalies occurred at the classical site; eight patients had left-sided neck lesion. Correct clinical diagnosis was made only in five patients (41.6 %). All patients underwent surgical excision with no reported recurrence. Branchial anomalies are frequently forgotten in the differential diagnosis of lateral neck swelling. Diagnosis is usually delayed, leading to improper treatment. The diagnosis of patients who present with lateral neck cystic swelling with or without episodes of recurrent neck abscess should be considered with a high suspicion for branchial anomalies. FNA cytology is a good investigative tool in reaching toward a diagnosis of branchial lesion, with the concurrent assistance of radiological modalities. Surgical excision is the gold standard treatment of lesions of branchial anomalies.
Diprosopus or duplication of the lower lip and mandible is a very rare congenital anomaly. We report this unusual case occurring in a girl who presented to our hospital at the age of 4 months. Surgery and problems related to this anomaly are discussed.
The aim of this study was to compare the skeletal and soft tissue patterns between obstructive sleep apnoea (OSA) patients and control group of non-OSA patients. Fifty Malays (32 males and 18 females) aged 18-65 years divided into two equal groups 25 (17 males and 8 females) with OSA and a control group 25 subjects (15 males and 10 females). Both groups were diagnosed using polysomnography. Nineteen variables related to craniofacial skeletal and soft tissue morphology were measured on lateral cephalometric films. Analysis of covariance was used to compare the means between the two groups. The results showed that OSA subjects had a significant increase in body mass index (BMI) and neck circumference than the control group. The soft palate and tongue were longer and thicker in OSA patients. In addition, upper, middle, and lower posterior airway spaces were narrower, the hyoid bone was more inferior and posterior, and the cranial base flexure angle was significantly acute when compared with the control group. The findings indicate that craniofacial abnormalities play significant roles in the pathogenesis of OSA in Malay patients.