Sports participation among children is declining. Sport and physical activity are important in childhood for optimising bone mass and reducing obesity and insulin resistance. Physical activity reduces cardiovascular risk factors in adults, and can improve survival in patients with cardiac failure. Musculoskeletal injury is the most common complication of sports participation in adults — not cardiac events. Some of the decline in function which occurs with ageing can be positively affected by regular physical activity.

Carolyn R Broderick MB BS, FACSP, GDSSc · Gregory J Winter FACSP, FRACGP, MSpMed · Roger M Allan FRACP, FCSANZ, FACC

Clinical records Patient 1 A 75-year-old woman, who was born in Greece and migrated to Australia 50 years ago, presented with acute shortness of breath secondary to cardiac failure due to valvular heart disease. Her only cardiovascular risk factor was hypertension. A transthoracic echocardiogram 1 month earlier had shown moderate aortic regurgitation, moderate left ventricular dysfunction, and a markedly dilated ascending aorta measuring 5.8 cm (normal, < 3.4 cm). Her symptoms were alleviated with diuresis and medication to treat cardiac failure. She underwent cardiac catheterisation, which revealed a dilated aortic root and ascending aorta, measuring 4.3 cm and 6 cm, respectively, with normal coronary arteries. Syphilis serology, requested because of the dilated aortic root, was positive with a rapid plasma reagin titre of 1 : 2 and a reactive Treponema pallidum particle agglutination test. Because of penicillin allergy, intravenous ceftriaxone 1 g daily was given for 21 days. No lumbar puncture was performed. She had an aortic valve replacement, and replacement of the ascending aorta with a synthetic graft. Macroscopically, the aortic wall was extremely thick. Histological sections showed features consistent with syphilitic aortitis (Figure 1). Her recovery was slow, but she is now living independently 15 months after surgery. 1 Aortic wall of Patient 1 A: Aortic wall (2 x objective) with thickened media (m) and intima (i). B: Aortic media with inflammatory infiltrate of plasma cells (arrowhead) and lymphocytes (arrow) (40 x objective). C: Aortic adventitia with narrowed vasa vasorum (endarteritis obliterans) (20 x objective). Patient 2 A 40-year-old Indonesian man who had lived in Australia for 14 years presented with acute onset severe central chest pain, on a background of similar but less severe pain over the previous 2 months. He smoked one packet of cigarettes per day, but had no other risk factors for coronary artery disease. He was in a monogamous heterosexual relationship of 18 months. His electrocardiogram revealed 3 mm ST elevation in the anterior leads (V1–3) and reciprocal ST depression in the inferior leads (II, III, aVF). His serum troponin level was 4.9 μg/L (reference range, 0–0.4 μg/L). A coronary angiogram revealed a completely occluded left main coronary artery at the ostium, and a 90% ostial lesion of his right coronary artery; the remainder of his right coronary system was clear of disease (Figure 2). He underwent emergency coronary artery bypass grafting. The operative notes do not comment on the appearance of the native coronary arteries, nor were these sent for histological examination. An aortic wall biopsy showed mild degenerative changes only — syphilis could not be excluded as causing these changes. In view of the bilateral coronary ostial lesions, syphilis serology was performed. His rapid plasma reagin titre was 1 : 64, together with a reactive Treponema pallidum particle agglutination test. Treatment with intravenous penicillin 1.8 g every 4 hours was given for 15 days, and prednisolone 25 mg twice daily for 2 days to prevent a Jarisch–Herxheimer reaction. The patient declined a lumbar puncture. 2 Coronary angiograms of Patient 2 \ A: Tapering of the aortic root (thin arrows) and left main coronary stump (arrowhead). B: 90% occlusion of right coronary ostium with no distal disease. Details of five patients The details of these two patients and another three patients seen between 1998 and 2004 are summarised in the Box. Although confirmatory histology of cardiovascular syphilis was not available for four of the five patients, this was considered the most likely diagnosis. All patients were born outside Australia. None of the three men reported having sex with men. No patients reported a history of syphilis. Syphilis has become a rare disease, although peaks of syphilis notifications occurred in Australia in the mid 1970s to mid 1980s and again more recently.1 Clinicians need to be aware of the manifestations of syphilis, and to consider the diagnosis outside the groups considered at risk of this infection in the modern era, such as men who have unprotected sex with other men. The five patients in our series ranged in age from 40 to 77 years. Notably, all were born overseas, and none belonged to a group considered at high risk of syphilis in the contemporary Australian context. Serology was consistent with acquisition of infection in the distant past in four of the five patients. Among patients with untreated syphilis, aortitis occurs in up to 70%–80%, and the clinically apparent manifestations of aortic regurgitation, coronary ostial stenosis and aortic aneurysms are seen in 10%–15%.2 Although aortic regurgitation is rarely caused by syphilis, it occurs in 20%–30% of patients with syphilitic aortitis.3 Coronary ostial lesions may be seen in 20%–25% of patients with syphilitic aortitis,3 but it is uncommon for such coronary ostial lesions to lead to acute myocardial infarction.4 In contrast, ostial lesions are only seen in 0.1% of patients with coronary artery disease, and bilateral lesions are even less common.5 The high plasma reagin titre seen in Patient 2 is also unusual in cardiovascular syphilis, but has been previously noted.6 Lessons from practice Cardiovascular syphilis may occur in patients outside of the usual risk groups for syphilis. Testing for syphilis serology should be considered in patients with aortic regurgitation, and particularly bilateral coronary ostial lesions or aortic aneurysms. Cerebrospinal fluid should be examined in patients with cardiovascular syphilis to exclude neurosyphilis. Patients with bilateral coronary ostial lesions but no distal coronary artery disease and those with ascending aortic aneurysms should be screened for syphilis. Screening should be considered for people with aortic regurgitation, especially those with risk factors, including birth in a country where syphilis has been or continues to be common. Although rates of neurosyphilis are low in patients with cardiovascular syphilis,3,7 a cerebrospinal fluid examination is recommended to exclude neurosyphilis. In addition, screening for other sexually transmitted infections, including HIV, should be considered, and appropriate contact tracing instituted. Sexual transmission of syphilis occurs only when mucocutaneous lesions are present, but long-term sexual partners of patients with late latent or tertiary syphilis should be screened serologically. The duration and route of penicillin therapy for cardiovascular syphilis are controversial. Each of our five patients received a different therapy. Penicillin has never been formally evaluated as treatment for cardiovascular syphilis, but a study from the 1950s showed that few patients had progressive disease after penicillin therapy, and up to 60% reported symptomatic relief.8,9 The Australian Therapeutic guidelines: antibiotic suggest intravenous benzylpenicillin for 15 days,10 the Australian National management guidelines for sexually transmissible infections suggest intramuscular procaine penicillin for 20 days,11 and the World Health Organization, European and United States guidelines suggest intramuscular benzathine penicillin 2.4 million units once weekly for three doses.12-14 These variations reflect the paucity of good clinical data comparing the different regimens. Although definitive evidence of the efficacy of benzathine penicillin is lacking, there is also no evidence to the contrary. There is little evidence that Jarisch–Herxheimer reactions complicate treatment of cardiovascular syphilis.8,9,15 Cardiovascular syphilis: one proven and four presumptive cases Age, sex and country of origin Indications for syphilis testing Syphilis serology and cardiovascular risk factors Management and follow-up 75 years, female, Greece Aortic regurgitation; dilated aortic root RPR: 1 : 2 TPPA: reactive Hypertension Aortic valve and aortic root replacement. Histological confirmation of syphilitic aortitis. Treated for cardiovascular syphilis with intravenous ceftriaxone 1 g daily for 3 weeks. No evidence of a Jarisch–Herxheimer reaction. Lumbar puncture not performed. Follow-up 15 months after therapy: mild exertional shortness of breath. Follow-up syphilis serology has not been requested. 40 years, male, Indonesia Acute myocardial infarction; bilateral ostial lesions and no visible disease in the right coronary artery RPR: 1 : 64 TPPA: reactive Smoker CABG. Histology from aortic wall biopsy was inconclusive for cardiovascular syphilis; no biopsy of the coronary arteries. Presumptively treated for cardiovascular syphilis with intravenous penicillin 1.8 g every 4 hours for 15 days. Oral prednisolone 25 mg twice daily for 2 days was given to prevent a Jarisch–Herxheimer reaction. No evidence of a Jarisch–Herxheimer reaction. CSF normal (lumbar puncture performed after treatment completed). Follow-up 2 years after therapy: symptom-free with RPR 1 : 8. 56 years, female, Vietnam Angina; bilateral ostial lesions; minor irregularities of the left circumflex artery, but no visible disease in the left anterior descending and right coronary arteries RPR: 1 : 1 TPPA: reactive Type 2 diabetes mellitus Hypercholesterolaemia Ostial lesions were not sufficiently severe to require revascularisation procedures, so no biopsies were taken of the aortic wall or coronary arteries. Presumptively treated for cardiovascular syphilis with intramuscular procaine penicillin 1 g daily for 15 days. No evidence of a Jarisch–Herxheimer reaction. CSF normal (lumbar puncture performed after treatment completed). Follow-up 1 year after therapy: symptom-free with RPR 1 : 1. Cardiovascular MRI revealed no further evidence of ostial coronary disease. 77 years, male, Hong Kong Aortic regurgitation; dilated aortic root RPR: 1 : 2 TPPA: reactive Hypertension Type 2 diabetes mellitus Aortic regurgitation was not sufficiently severe to warrant valve replacement, so no aortic wall biopsy was obtained. Presumptively treated for cardiovascular syphilis with intramuscular benzathine penicillin 2.4 g weekly for 3 doses. No evidence of a Jarisch–Herxheimer reaction. CSF normal. Follow-up: returned to Hong Kong and lost to follow-up. Follow-up syphilis serology has not been requested. 72 years, male, Timor Wife had been found to have positive syphilis serology. Following positive syphilis serology, aortic regurgitation was noted on clinical examination and then echocardiographically RPR: Non-reactive TPPA: reactive No cardiovascular risk factors present Aortic regurgitation was not sufficiently severe to warrant valve replacement, so no aortic wall biopsy was obtained. Presumptively treated for cardiovascular syphilis with intravenous penicillin 1.8 g 4 hourly for 14 days. Intravenous hydrocortisone 50 mg four times a day given for 48 hours because of concern about a Jarisch–Herxheimer reaction when atrial fibrillation occurred on antibiotic therapy. No other manifestations of a possible Jarisch–Herxheimer reaction. Atrial fibrillation reverted to sinus rhythm with amiodarone and sotalol therapy. CSF normal. Follow-up: ongoing medical management for cardiac failure. Repeat syphilis serology 3 years after therapy, showed RPR non-reactive. CABG = coronary artery bypass graft. CSF = cerebrospinal fluid. MRI = magnetic resonance imaging. RPR = rapid plasma reagin titre. TPPA = Treponema pallidum particle agglutination test.

Steven Y C Tong MB BS · Alan C Street MB BS, FRACP · Haris Haqqani MB BS

Objective: To determine whether pancreatic islet transplantation can control diabetes and prevent severe life-threatening hypoglycaemia.Design, setting and participants: A single-arm observation study of six patients undergoing islet transplantation. All patients had had type 1 diabetes mellitus for over 5 years and documented episodes of repeated severe hypoglycaemia. Islets were isolated from donor pancreases digested by Liberase. Separated islets were infused into the recipient’s liver via the portal vein. Patients were immunosuppressed with daclizumab, sirolimus and tacrolimus. The transplants were performed at Westmead Hospital, NSW, between October 2002 and February 2005.Main outcome measures: Normal blood glucose control without administration of exogenous insulin; demonstration of islet function and abolition of hypoglycaemia.Results: Five of the patients received two islet infusions, and the sixth was withdrawn after one infusion following a portal vein thrombosis. Three patients became insulin-independent, with excellent glycaemic control. Two had islet function with circulating C-peptide, improved glycaemic control, reduced insulin requirement and abolition of severe hypoglycaemia. However, over a 2-year period, graft function deteriorated. Recipients who were initially insulin free remained C-peptide positive but required supplemental insulin. Complications included one postoperative bleed, two portal vein thromboses (which resolved completely), presumed recurrence of tuberculosis in one patient, and deterioration in renal function in one patient.Conclusions: Islet transplantation is effective at improving glycaemic control and hypoglycaemia unawareness in the short to medium term. However, problems with long-term safety of immunosuppression, islet-induced thrombosis and early detection of loss of islet function remain to be addressed.

Philip J O’Connell MB BS, FRACP, PhD · Wayne J Hawthorne MHSc, MD · Brian J Nankivell MD, PhD, FRACP · Anita T Patel BSc · Stacey N Walters DipAppSci · Henry C C Pleass MD, FRCS, FRACS · Richard D M Allen MB BS, FRACS · Jeremy R Chapman MD, FRACP, FRCP · D Jane Holmes-Walker MB BS, FRACP, PhD · Jenny E Gunton MB BS, FRACP, PhD

Objectives: To examine changes in asthma prevalence in the context of other population changes between 1990 and 2003, for specific age and sex groups.Design: Cross-sectional survey based on household interviews, repeated annually.Setting and participants: Representative samples of the South Australian population between 1990 and 2003 (around 3000 people per year).Main outcome measures: Current prevalence of doctor-diagnosed asthma and other health and demographic variables potentially associated with asthma, and asthma management.Results: Response rate was over 71%. Between 1990 and 2003, asthma prevalence increased significantly, doubling in females (from 7.3% in 1990 to 14.6% in 2003), with a smaller increase in males (from 7.8% to 9.4%). Asthma also increased in all age groups, but the largest relative increases occurred in people aged 55 years and older. Logistic regression analyses showed that obesity was a major predictive variable for every age group studied. The prevalence of asthma morbidity (waking at night and days lost from usual activities because of asthma) among those with asthma showed no significant changes between 1990 and 2003. Asthma action plans (introduced on a population basis in 1992) peaked in their distribution at 42% in 1994, and then declined to half that percentage in 2003. The increase in asthma prevalence occurred at the same time as increases in population prevalence of obesity (10.3% to 18.7%) and diabetes (3.1% to 6.9%), and decline in recent vigorous exercise (42.4% to 32.7%).Conclusions: The increase in asthma prevalence over a decade was large, but concentrated among specific sex and age groups. The increase accompanied population increases in obesity and diabetes and a decline in vigorous exercise.

David H Wilson PhD, MPH, BEd, AdvDipEd · Robert J Adams MD · Richard E Ruffin MD · Graeme Tucker BSc · Anne W Taylor MPH · Sarah Appleton BA

Objective: To estimate the prevalence of heart failure (HF) and left ventricular (LV) systolic dysfunction in a population-based sample of older Australians.Design, setting and participants: A cross-sectional survey of 2000 randomly selected residents of Canberra, aged 60–86 years, conducted between February 2002 and June 2003. Participants were assessed by history, physical examination by a cardiologist, and echocardiography.Main outcome measures: Age- and sex-specific prevalence rates of clinical HF and LV systolic dysfunction (defined as LV ejection fraction ≤ 50%).Results: Of 1846 people eligible for our study, 1388 (75%) agreed to participate and 1275 completed all investigations (mean age, 69.4 years; 50% men). In the study sample, 72 subjects (5.6%; 95% CI, 4.4%–7.1%) had clinical HF that had been previously diagnosed and was confirmed by our assessment. A further 0.6% (95% CI, 0.3%–1.2%) had undiagnosed clinical HF (ie, evidence of structural heart disease and symptoms/signs of cardiac insufficiency without a previous diagnosis of clinical HF). Thus, the overall prevalence of clinical HF in the sample was 6.3% (95% CI, 5.0%–7.7%). Clinical HF increased in prevalence with advancing age (a 4.4-fold increase from the 60–64-years age group to the 80–86-years age group; P < 0.0001). Of the 75 subjects (5.9%; 95% CI, 4.7%–7.3%) with LV systolic dysfunction, 44 (59%) were in the preclinical stage of disease.Conclusion: Diagnosed HF cases represent the “tip of the iceberg” for the national burden of HF and LV systolic dysfunction. Clinically identifiable HF cases can remain undiagnosed, and the majority of people with LV systolic dysfunction are in a preclinical stage of the disease.

Walter P Abhayaratna MB BS, FRACP · Niels G Becker BSc, MSc, PhD · Wayne T Smith BMed, MPH, PhD · Thomas H Marwick MB BS, PhD, FRACP · Ian M Jeffery MB BS, FRACP · Darryl A McGill MB BS, PhD, FRACP

Clinical record Computed tomography of the thorax showed bilateral pleural effusions (PE) and diffuse thickening of the oesophagus (O). An 84-year-old woman presented to the emergency department following the sudden onset of retrosternal chest pain with associated nausea and sweating. The pain was exacerbated by swallowing, but this symptom was not felt to be significant at the time. She had no past history of ischaemic heart disease or diabetes and did not smoke, but did have a weak family history of heart disease, and a personal history of mild hypercholesterolaemia and refractory hypertension despite aggressive medical management (baseline systolic blood pressure consistently 150–220 mmHg, according to previous medical records). An electrocardiogram (ECG) showed a left bundle branch block, with no previous ECG available for comparison. Acute myocardial infarction was diagnosed. However, coronary angiography showed no abnormalities, and serial measurements of serum troponin and creatine kinase levels failed to confirm an infarction. The next morning, the patient developed profound dysphagia and had an episode of haematemesis. Full blood examination showed normocytic anaemia (haemoglobin concentration, 70 g/L; reference range [RR], 115–165 g/L), with mean cell volume of 91 fL (RR, 78–99 fL), neutrophilia (12.7 × 109 cells/L [RR, 2.0–8.0 × 109 cells/L]), and platelet count in the RR (172 × 109 cells/L [RR, 150–450 × 109 cells/L]). She developed a cough, and a chest x-ray showed left lower lobe consolidation. Intravenous ceftriaxone therapy was begun to treat this. Computed tomography was arranged to investigate the oesophageal symptoms. This showed a diffusely thickened oesophagus from the thoracic inlet to the gastro-oesophageal junction, bilateral pleural effusions, and left lower lobe collapse and consolidation (Figure). Endoscopy showed blackened oesophageal mucosa with extensive mucosal bleeding. The procedure was abandoned because of these findings, and the patient was intubated and transferred to our tertiary referral centre for ongoing care. On arrival, chest x-ray showed progression of the pulmonary effusion, obscuring the entire left lung field. At bronchoscopy, a substantial volume of old clot was evacuated from the left bronchial tree. Repeat endoscopy again showed an extensive mucosal lesion. A percutaneous endoscopic gastrostomy (PEG) tube was placed, and a provisional diagnosis of malignancy was made (biopsies subsequently showing benign tissue only). The patient remained haemodynamically stable throughout, and was extubated 2 days later. She remained asymptomatic and was able to eat a full diet 6 days later. A final diagnosis was made of dissecting haematoma of the oesophagus. The patient remained in excellent health at follow-up a month later, at which time the PEG tube was removed without incident. Dissecting haematoma of the oesophagus is a rare, relatively benign condition that mimics much more common and serious illnesses.1 The onset of sudden severe retrosternal chest pain in an elderly person, who often has other cardiovascular risk factors, may lead to an erroneous diagnosis of cardiac pain. In some cases, subsequent thrombolytic treatment has led to fatalities. In others, the appearance of intramural thrombus on radiological or endoscopic views has been mistaken for advanced oesophageal malignancy or rupture.2,3 Lessons from practice Dissecting haematoma of the oesophagus should be included in the differential diagnosis in elderly patients presenting with cardiac-type chest pain, and oesophageal symptoms should be specifically sought in the history. The presence of oesophageal symptoms (dysphagia or odynophagia) in the context of cardiac-type chest pain should prompt investigation with appropriate imaging studies (barium swallow or computed tomography of the thorax before administration of fibrinolytic agents. Concern about dissecting haematoma of the oesophagus can be clarified rapidly with a water "sip test". The course of dissecting haematoma of the oesophagus is essentially benign if unnecessary intervention is avoided. Because of the rarity of dissecting haematoma of the oesophagus, diagnosis depends on an accurate history and a high index of suspicion. Those affected are usually women (relative risk, 1.8 — the inverse to the male bias in cardiovascular and malignant oesophageal disease),3 elderly (median age, 63 years),3 and not uncommonly taking anticoagulant or antiplatelet agents. Patients present with a variable combination of chest pain (usually sudden in onset and of short duration), haematemesis (mainly small volume and occurring after the pain), and dysphagia and/or odynophagia. In one meta-analysis, 99% of patients had at least one of these symptoms, and 32% had all three.3 In particular, the presence of dysphagia or odynophagia in a patient otherwise thought to have angina or myocardial infarction should prompt oesophageal imaging before administration of fibrinolytic agents. A simple test is to ask the patient to take a sip of water as part of the examination. If this exacerbates symptoms or unmasks new ones, a more specific focus on the possibility of oesophageal abnormality is warranted. Dissecting haematoma of the oesophagus has a typical appearance on imaging.4,5 Barium swallow shows a long, smooth tubular filling defect in the lumen of the oesophagus, sometimes with the dissection space filled with a stripe of contrast (the “double-barrelled oesophagus”). As the dissection most commonly occurs along the posterior wall, the lateral view is most useful. Computed tomography demonstrates an obliterated lumen with thickening of the wall. This extends over a long length of the oesophagus, and can mimic oesophageal rupture or extensive malignancy. The haematoma is large, fluctuant, and blue or purplish when viewed at endoscopy. Delayed endoscopy shows a long ulcer, where the overlying mucosa has sloughed, followed by rapid regeneration and an ultimately normal appearance.3 For a condition with such dramatic presentation, the natural history is refreshingly benign. The best intervention is non-intervention: the haematoma almost always resolves, and full oesophageal function is restored. Intravenous hydration, supplemented by parenteral or enteral nutrition in appropriate cases, is usually all that is needed. Anti-ulcer medications have no proven benefit.3 Surgery is indicated if there is uncontrolled arterial bleeding, or if the partial rupture has been converted to a full thickness tear through endoscopy.3 A further indication for surgery is a focus of infection within the false lumen, preventing healing. In two such cases, endoscopic division of the overlying mucosal flap resulted in complete resolution of fever, odynophagia and neutrophilia.1,6 Unfortunately, aggressive intervention has led to fatalities.3

Deborah H Amott MB BS(Hons) · Gavin M Wright FRACS

Elizabeth May Pepper,* Dominique A Cadilhac,† Dora C Pearce,‡ James Burrow,§ Tarun S Weeramanthri¶ * Neurology Registrar, John Hunter Hospital, Newcastle, NSW. † Manager, Public Health Division; ‡ Biostatistician; National Stroke Research Institute, Melbourne, VIC. § Neurologist; ¶ Physician, Royal Darwin Hospital, NT. hornblowerATinternode.on.net To the Editor: Although the age-standardised stroke mortality rates among Australia’s Indigenous people is more than twice that of the non-Indigenous population,1 the medical literature contains only one audit of Indigenous stroke patients in Perth metropolitan hospitals.2 No review of hospital care has been reported. Royal Darwin Hospital (RDH) is the referral centre for Australia’s “Top End”, where 8.7% of Indigenous Australians reside; 40% of RDH inpatients are Indigenous. In 2002, while planning for the RDH stroke service, we audited stroke admissions from the previous year. Among 121 eligible patients admitted between 1 July 2001 and 31 June 2002 with International classification of diseases, 10th revision, Australian modification (ICD-10-AM) codes 160–164 (haemorrhages [subarachnoid, intracerebral, other non-traumatic intracranial] and cerebral infarction), records for 116 (96%) were available, but six patients were excluded because of incorrect coding. Box 1 outlines patient characteristics, while Box 2 examines risk factors and medication use for ischaemic stroke (because haemorrhages were few). Despite the observed differences between subgroups, there were no significant differences in mortality (4/36 for Indigenous v 7/42 for non-Indigenous; P = 0.204) or stroke severity at admission or discharge. Box 3 highlights differences in risk factors between Indigenous males and females. Retrospective data, particularly from a sample identified by medical record coding, should be interpreted with caution. In addition, the potential for random error due to small numbers, and the referral bias inherent in tertiary hospital admissions, mean our results may not truly represent the “Top End” Indigenous population. However, our data corroborate findings that Indigenous Australians suffer premature cerebrovascular disease, and have higher rates of vascular risk factors than other Australians,1 with some risk factor differences between males and females. Further, recent evidence suggests differences in standards of stroke care in regional (Queensland) hospitals.3 We found disparity in hospital care of Indigenous patients, and this requires further detailed investigation. A prospective, community-based study is urgently needed. 1 Baseline characteristics for 110 patients admitted to Royal Darwin Hospital with subarachnoid, intracerebral, and other non-traumatic intracranial haemorrhages and cerebral infarction in 2001–02 Baseline characteristics Indigenous Other P Number of patients 45 65 Female sex 22 (49%) 19 (29%) 0.018 Mean age (years) 54 61 0.005 Rural dwelling 41 (91%) 22 (34%) < 0.001 Ischaemic stroke 36 (80%) 42 (65%) 0.081 2 Risk factors and medication use for the 78 patients who had ischaemic stroke Risk factors and medications Indigenous Other P All patients 36 42 Smoking 23 (64%) 11 (26%) 0.001 Diabetes mellitus 16 (44%) 10 (24%) 0.030 Rheumatic heart disease 8 (22%) 1 (2%) < 0.001 Males 19 (53%) 30 (71%) 0.089 Smoking 14 (74%) 12 (40%) 0.017 Diabetes mellitus 10 (53%) 7 (23%) 0.030 Females 17 (47%) 12 (29%) 0.089 Smoking 9 (53%) 0 0.002 Rheumatic heart disease 6 (35%) 0 0.026 Antiplatelet therapy Before admission 11 (31%) 19 (45%) 0.078 Admission 20 (56%) 38 (91%) < 0.001 Discharge 18/32 (56%) 29/35 (83%) 0.013 Anticoagulant therapy Before admission 4 (11%) 1 (2%) < 0.001 Discharge 4/32 (13%) 6/35 (17%) 0.235 3 Risk factor differences between Indigenous males and females who had ischaemic stroke Males Females P Number of patients 19 17 Hypertension 16 (84%) 6 (35%) 0.003 Non cerebral vascular disease 6 (32%) 1 (6%) < 0.001 Excessive alcohol intake 7 (37%) 1 (6%) < 0.001

Elizabeth May Pepper · Dominique A Cadilhac · Dora C Pearce · James Burrow · Tarun S Weeramanthri

Clinical record A 37-year-old woman presented with a 2-month history of progressive restriction of movement of the left shoulder joint, associated with episodic pain over the region. She was unable to raise her left arm above the shoulder. The pain was mild, deep-seated, non-radiating and relieved by supporting the left elbow. She denied any weakness of the arm muscles. The patient also complained of diminished pain and temperature sensation over the affected limb, as evidenced by painless scalds of the fingers of her left hand — the first about 4 years previously, and the second, 7 days before presentation. She had had mild pain in the left shoulder region infrequently for the previous 17 years, and had noticed enlargement of her left arm since childhood. She had not noticed any lymphadenopathy. She had normal facial sensation and denied any history of diplopia, dysarthria, dysphagia, hoarseness, vertigo, sphincter abnormalities or weakness and ataxia of any extremity. There was no history of neck pain, trauma, radiation or prolonged fever. She had no hypertension or diabetes, and her family and obstetric history were unremarkable. Examination: The patient’s left upper limb was larger than the right (Box, A). The other limbs appeared normal. Girth of the left upper limb was 51.6 cm at the shoulder (right, 46.5 cm); 35.8 cm at the mid-arm (right, 31.4 cm); and 29.1 cm at the forearm (right, 25.2 cm). Wrist and fingers also had wider girth on the left compared with the right, but were equal in length. The swelling of the left upper limb was painless, non-pitting, with no colour change or abnormal sweating. Temperature of the limb was normal. Apart from evidence of a recent scald injury, no other superficial skin changes, vascular prominences or nerve thickening were observed. Loose bodies were present in the left shoulder joint, as evidenced by palpable crepitus. Left shoulder abduction was restricted beyond 90°. No hum, bruit or machinery murmur could be heard on auscultation in any part of the affected limb. The right breast was slightly larger than the left. Neurological examination revealed that pain, temperature and tactile sensation were diminished on the left side from C2 to T6 dermatomes anteriorly, and from C2 to T4 dermatomes posteriorly. Position and vibration senses were well preserved in these segments. All modalities of sensation in other parts of the body were normal. The patient had normal speech and higher functions. The hairline was at C2 level and the height–neck ratio was 11.67 (reference range, < 13). No meningism or cranial or spinal deformities could be identified. Examination of the cranial nerves gave results within normal limits. Apart from a mild weakness of the small muscles of the left upper limb, as evidenced by a positive card test and Grade 4/5 power in the left supraspinatus and deltoid muscles, the motor system appeared normal. Deep tendon reflexes in the left upper limb were present but grossly diminished. Plantar reflexes were bilaterally flexor, with preserved abdominal reflexes. The patient had normal coordination, stance and gait, with no evidence of nystagmus or Horner syndrome. Investigations: Complete blood count and routine biochemistry tests gave normal results. Fasting blood glucose level was 5.4 mmol/L (reference range [RR], < 6.1 mmol/L), and results of oral glucose tolerance were in the reference range. A serum VDRL (Venereal Disease Research Laboratory) test was negative. An x-ray of the left hand showed soft tissue swelling and a slight increase in the size of the metacarpals and proximal phalanges compared with the right (Box, B). X-ray of the left shoulder joint showed loss of normal architecture, multiple bony fragments and loose bodies (Box, C). Skeletal imaging showed no abnormalities of the skull, spine or other limbs. No abnormalities were seen in the affected limb on arteriovenous doppler examination, computed tomography, magnetic resonance imaging, angiography, lymphangiography and skin biopsy. Magnetic resonance imaging of the brain and spine showed a fairly large (12 mm) cerebellar tonsil dipping in the posterior portion of the foramen magnum, with a large syrinx involving the whole of the cervical and thoracic spine to level T8 (Box, D). Management: The patient was advised to undergo posterior fossa decompression, but was unwilling to have surgical intervention and is currently receiving regular follow-up. A syrinx is a cavity in the spinal cord (syringomyelia) or brain stem (syringobulbia).1 Syringomyelia is a chronic progressive degenerative or developmental disorder of the spinal cord, characterised clinically by brachial amyotrophy, cutaneous analgesia and thermoanaesthesia, with sparing of tactile, joint, position and vibration senses, and loss of deep tendon reflexes. Frequently, there are associated developmental abnormalities of the vertebral column, base of the skull, and particularly of the cerebellum and brain stem. About 90% of patients with syringomyelia have type I Chiari malformation (downward displacement of cerebellar tonsils). Patients with syringomyelia typically present with lower motor neurone signs at the level of the lesion, dissociated sensory loss in a cape or hemi-cape distribution on the arms or upper trunk, and spinal long tract dysfunction below the level of the lesion. Few patients show the complete picture, and the clinical features vary with the size, location and shape of the cavity, and the associated neurological condition.1 Common early signs are wasting and weakness of the small muscles of the hand, or loss of feeling in the hand and resulting injuries. Surprisingly, our patient — despite a relatively large syrinx — had no lower motor neurone signs in the upper limb, apart from diminished deep tendon reflexes and mild weakness, and no spinal long tract signs. Syringomyelia can also cause “neuropathic joint”, most commonly in the shoulder, elbow or wrist. The affected joint is often enlarged but painless, and movements evoke loud crepitus,2 as in our patient. While syringomyelia is more commonly associated with limb atrophy, hypertrophy can occur, involving bones, muscles and other tissues in one limb, one half of the body or even the tongue.2 Trophic changes in the skin can include cyanosis, hyperkeratosis and thickening of the subcutaneous tissue, leading to a swelling of the fingers described as “la main succulente”.2 Other findings associated with syringomyelia include increased ratio of arm to body length, size differences between the breasts, and curved fingers,2 (the latter two features documented in our patient). An association with hypertrophy of the limbs, hands or feet is also recognised in syringomyelia,3 and has been reported in four patients from Japan, including three of a series of 26,4,5 and in two patients from India. However, the latter two, in contrast to our patient, had prominent neurological features.6,7 Localised hypertrophy of the hand is also seen in syringomyelia: a radiological study of hand bones in four patients with syringomyelic chiromegaly found enlarged hand bones on the affected side.8 This feature was also evident in our patient. A range of mechanisms have been postulated to explain the segmental hypertrophy in syringomyelia, including stimulation of the sympathetic nervous system, causing defective circulation and oedema.9 An in-vitro study of muscle cells from patients with syringomyelia and muscle hypertrophy found the cells had accelerated growth in the presence of patient serum, suggesting involvement of serum factors and molecules released in response to neural lesions.10 To our knowledge, there are few other documented cases of syringomyelia presenting with both neuropathic joint and limb hypertrophy. Our case highlights this rare clinical presentation of a common spinal cord anomaly. It reminds clinicians that, although conventional teaching commonly associates syringomyelia with limb atrophy, it can be associated with limb hypertrophy. Hence, syringomyelia should be considered as a differential diagnosis in all cases of limb enlargement. Moreover, this case further emphasises the importance of considering a spinal cord anomaly as the aetiology of upper limb neuropathic joint. A patient with syringomyelia A: Hypertrophy of the left upper limb. B: X-ray of the hands showing bony and soft tisse enlargement on the left side. C: X-ray of the left shoulder showing gross disorganisation with multiple bony fragments. D: Magnetic resonance imaging of the cervical spine (T1-weighted) showing a large syrinx with type I Chiari malformation (arrow).

Partha P Chakraborty MB BS · Dipanjan Bandyopadhyay MD · Sanjay K Mandal MD · Subhasis Roy Chowdhury MB BS · Ramtanu Banerjee MD · Shounak Majumdar MB BS · Rana Bhattacharji MD

Objective: To assess the prevalence and impact of overweight and obesity in an Australian obstetric population.Design, setting and participants: The Mater Mother’s Hospital (MMH), South Brisbane, is an urban tertiary referral maternity hospital. We reviewed data for the 18 401 women who were booked for antenatal care at the MMH, delivered between January 1998 and December 2002, and had a singleton pregnancy. Of those women, 14 230 had an estimated pre-pregnancy body mass index (BMI) noted in their record; 2978 women with BMI ≤ 20 kg/m2 were excluded from further study; the remaining 11 252 women were divided into four categories: “normal” (BMI 20.01–25 kg/m2), “overweight” (BMI 25.01–30 kg/m2), “obese” (BMI 30.01–40 kg/m2) and “morbidly obese” (BMI > 40 kg/m2).Main outcome measures: Prevalence of overweight and obesity in an obstetric population; maternal, peripartum and neonatal outcomes associated with raised BMI.Results: Of the 14 230 women, 6443 (45%) were of normal weight, and 4809 (34%) were overweight, obese or morbidly obese. Overweight, obese and morbidly obese women were at increased risk of adverse outcomes (figures represent adjusted odds ratio [AOR] [95% CI]): hypertensive disorders of pregnancy (overweight 1.74 [1.45–2.15], obese 3.00 [2.40–3.74], morbidly obese 4.87 [3.27–7.24]); gestational diabetes (overweight 1.78 [1.25–2.52], obese 2.95 [2.05–4.25], morbidly obese 7.44 [4.42–12.54]); hospital admission longer than 5 days (overweight 1.36 [1.13–1.63], obese 1.49 [1.21–1.86], morbidly obese 3.18 [2.19–4.61]); and caesarean section (overweight 1.50 [1.36–1.66], obese 2.02 [1.79–2.29], morbidly obese 2.54 [1.94–3.32]). Neonates born to obese and morbidly obese women had an increased risk of birth defects (obese 1.58 [1.02–2.46], morbidly obese 3.41 [1.67–6.94]); and hypoglycaemia (obese 2.57 [1.39–4.78], morbidly obese 7.14 [3.04–16.74]). Neonates born to morbidly obese women were at increased risk of admission to intensive care (2.77 [1.81–4.25]); premature delivery (< 34 weeks’ gestation) (2.13 [1.13–4.01]); and jaundice (1.44 [1.09–1.89]).Conclusions: Overweight and obesity are common in pregnant women. Increasing BMI is associated with maternal and neonatal outcomes that may increase the costs of obstetric care. To assist in planning health service delivery, we believe that BMI should be routinely recorded on perinatal data collection sheets.

Leonie K Callaway MB BS(Hons), FRACP · Allan M Chang PhD, FRANZCOG · H David McIntyre MB BS(Hons), FRACP · Johannes B Prins PhD, FRACP

Further complications of the “obesity epidemic” The potential health burden to our community of escalating overweight and obesity is well documented and publicised. Attention has focused on the association with chronic diseases such as hypertension, diabetes, cardiovascular disease and all-cause mortality. The effects of obesity on reproductive function and outcomes have received less attention. The ability to conceive spontaneously is reduced by obesity. While many overweight women can conceive easily, they are over-represented among subfertile groups and those presenting with menstrual disorders.1 Overweight women, both with and without polycystic ovary syndrome, present with menstrual irregularity and anovulation more frequently than women with normal body mass index (BMI). The US Women’s Health Study concluded that even a slightly elevated BMI at age 18 was a risk factor for subsequent anovulatory infertility.2 Several studies have confirmed the association between obesity and reduced fertility.1,3 Elevated BMI is also associated with poorer outcomes from assisted reproduction.3 Obesity also affects pregnancy outcomes. The article in this issue by Callaway et al4 is an important and timely reminder of the high prevalence of obesity in women of reproductive age and the serious adverse effects of overweight during pregnancy. Risks are increased for both mother and baby. Maternal problems can result from pre-existing obesity-related illnesses such as hypertension and type 2 diabetes. The increased pregnancy-related risks of obesity include increased rates of miscarriage,1 gestational diabetes, pregnancy-induced hypertension, pre-eclampsia, thromboembolism, haemorrhage, caesarean section, sleep apnoea, wound infection and anaesthetic complications.5,6 These risks persist, even when adjusted for pre-existing illness. The offspring of overweight and obese women are more likely to require admission to neonatal intensive care and to have congenital abnormalities such as neural tube and cardiac defects. Birth-related injuries and fetal death in utero are also higher in this group, and babies are more likely to be macrosomic, placing them at risk of birth trauma and possible subsequent childhood (and, indeed, lifelong) obesity.5,7 What are the effects of weight loss on fertility and pregnancy outcomes? Weight loss alone often leads to improvement in conception rates. Clark et al,1 evaluating a 6-month diet and exercise program in overweight anovulatory women, found that participants in the program tended to lose weight and resume ovulation. Pregnancy rates, self-esteem and endocrine parameters improved, while rates of miscarriage fell. Seventy-eight per cent of the women conceived, with 67% achieving a live birth. Weight loss of dramatic proportions was not required — a relatively small weight loss (6–10 kg) could lead to resumption of spontaneous ovulation. The effects of weight loss on pregnancy outcomes have recently been reported by Dixon et al.8 Their prospective study sought to examine the effects of laparoscopic adjustable gastric banding (LAGB) in severe obesity. They concluded that “pregnancy outcomes after LAGB are consistent with general community outcomes rather than outcomes from severely obese women”. Callaway et al4 comment on the implications for health care delivery costs of obesity-related increases in maternal and neonatal morbidity. This problem will only worsen. The AusDiab study9 reported that the prevalence of obesity in 2003 was 2.5 times higher than in 1980. Moreover, obesity is occurring at a younger age, the problem increases with time, and women are becoming pregnant later in life.10 Solutions to such a complex problem will inevitably be multifaceted and costly. Callaway and colleagues recommend that maternal BMI should be recorded at the booking visit for all pregnancies. While this will assist in documenting the problem, any meaningful intervention must occur before presentation with an established pregnancy. Given the potential for adverse health outcomes for both mother and baby, and the potential lifelong effects of neonatal macrosomia, there is a pressing need for action well before conception. As part of general public health efforts to combat obesity, we strongly recommend pre-pregnancy counselling for all women, with every effort being made to intervene in the case of women who are overweight or obese. The majority of women are highly motivated to strive to have healthy babies, and the power of this commitment could well be used to achieve behavioural change that could have short-term (and potentially lifelong) benefits for both mothers and children.

Alison J Nankervis MB BS, MD, FRACP · Jennifer J Conn MB BS, MClinEd, FRACP · Rachael L Knight MB BS, MD, FRANZCOG