Clinical spectrum of children receiving palliative care in Malaysian Hospitals.

INTRODUCTION: Awareness for paediatric palliative care has resulted in the impetus for paediatrician-led palliative care services across Malaysia. However, there is paucity of local data on patients receiving hospital-based paediatric palliative care. We aim to review the clinical spectrum of patients referred to these services. METHODS: An observational study of children aged between 0-18 years receiving palliative care at 13 hospitals between 1st January and 31st December 2014 was carried out. RESULTS: There were 315 patients analysed, 90 (28.6%) and 46 (14.6%) were neonates and adolescents respectively. The main ICD-10 diagnostic categories for all patients were identified to be 'Congenital malformations, deformations and chromosomal abnormalities' 117 (37.1%), 'Diseases of nervous system' 76 (24.1%) and 'Neoplasms' 60 (19.0%). At referral 156 (50%) patients had holistic needs assessments. Patients with 'Diseases of nervous system' were assessed to have significantly more physical needs than the other two diagnostic categories. Majority of patients who knew of their diagnosis and prognosis were those with malignancy. Over a fifth of referrals were at their terminal admission. Of 144 who died, 111 (77.1%) had advanced care plans. There was bereavement follow-up in 98 (68.1%) patients. CONCLUSION: Patients referred for palliative care have varied diagnoses and needs. To ensure all paediatricians are competent to deliver quality care to all children, further education and training initiatives is imperative.

Management of neonatal jaundice in primary care.

The Clinical Practice Guidelines on Management of Neonatal Jaundice 2003 was updated by a multidisciplinary development group and approved by the Ministry of Health Malaysia in 2014. A systematic review of 13 clinical questions was conducted using evidence retrieved mainly from Medline and Cochrane databases. Critical appraisal was done using the Critical Appraisal Skills Programme checklist. Recommendations were formulated based on the accepted 103 evidences and tailored to local setting as stated below. Neonatal jaundice (NNJ) is a common condition seen in primary care. Multiple risk factors contribute to severe NNJ, which if untreated can lead to adverse neurological outcomes. Visual assessment, transcutaneous bilirubinometer (TcB) and total serum bilirubin (TSB) are the methods used for the detection of NNJ. Phototherapy remains the mainstay of the treatment. Babies with severe NNJ should be followed-up to detect and manage sequelae. Strategies to prevent severe NNJ include health education, identification of risk factors, proper assessment and early referral.

Early presentation of right adrenal mass, hepatoblastoma and hepatic cavernous haemangioma in Beckwith-Wiedemann Syndrome.

Beckwith-Wiedemann Syndrome (BWS) is associated with early development of embryonal tumours usually in the first four years of life. We describe a patient who presented with a right adrenal cyst in the first month of life and hepatoblastoma in the third month of life. A cavernous haemangioma was subsequently found in the resected tumour.

Aortic fenestration for acute or chronic aortic dissection: an uncommon but effective procedure.

BACKGROUND: Aortic fenestration is rarely required for patients with acute or chronic aortic dissection. To better define its role and the indications for its use and to evaluate its success at relieving organ or limb malperfusion, we reviewed our experience with direct fenestration of the aorta. METHODS: A retrospective analysis of all consecutive aortic fenestrations performed between January 1, 1979, and December 31, 1999, was performed. Fourteen patients, 12 men and two women (mean age, 59.6 years; range, 43-81), underwent fenestration of the aorta. All patients were hypertensive and had a history of tobacco use. By Stanford classification, there were three type A and 11 type B patients. In the acute dissection group (n = 7), indications for surgery were malperfusion in six patients (leg ischemia, 4; renal ischemia, 5; bowel ischemia, 3) and intra-abdominal bleeding from rupture in two. In the chronic dissection group (n = 7), indications for surgery were abdominal aortic aneurysm in 4 patients (infrarenal, 3; pararenal, 1), thoracoabdominal aneurysm in 1, hypertension from coarctation of the thoracic aorta in 1, and aortic occlusion with disabling claudication in 1. RESULTS: Emergency aortic fenestration was performed in seven patients (surgically for 6 and percutaneously for 1). Fenestration level was infrarenal in four and pararenal in three. Concomitant abdominal aortic graft replacement was performed in four patients, combined with ascending aortic replacement (n = 1) and bilateral aortorenal bypasses (n = 1). In two patients, acute fenestration was performed for organ malperfusion after prior proximal aortic replacement (ascending aorta, 1; descending thoracic aorta, 1). Seven elective aortic fenestrations were performed for chronic dissection (descending thoracic aorta, 2; paravisceral aorta, 2; infrarenal aorta, 2 and pararenal aorta, 1). Concomitant aortic replacement was performed in six patients (abdominal aorta, 5; thoracoabdominal aorta, 1). Fenestration was successful at restoring flow in all 10 patients with malperfusion. Operative mortality for emergency fenestration was 43% (3/7). The three deaths that occurred were of patients with anuria or bowel ischemia, or both. There were no postoperative deaths for elective fenestration. At a mean follow-up of 5.1 years, there were no recurrences of malperfusion and no false aneurysm formations at the fenestration site. CONCLUSION: Fenestration of the aorta can effectively relieve organ or limb ischemia. Bowel ischemia and anuria are indicators of dismal prognosis and emergency fenestration in these patients carries a high mortality. Elective fenestration combined with aortic replacement can be performed safely in chronic dissection. Aortic fenestration is indicated for carefully selected patients with malperfusion and offers durable benefits.

Should an appendix that looks 'normal' be removed at diagnostic laparoscopy for acute right iliac fossa pain?

OBJECTIVE: To find out whether the removal of the appendix from patients in whom laparoscopy for acute right iliac fossa pain shows no abnormality is justified to avoid the risk of missing acute appendicitis. PATIENTS: The records of patients who, between 1990 and 1997 had emergency laparoscopy for acute right iliac fossa pain were reviewed. Only those in whom laparoscopy had shown no abnormality and had not had the appendix removed were included in the study. METHODS: Outcome was assessed by telephone questionnaire to the patient, the general practitioner, or both. RESULTS: Emergency laparoscopy had been done for 254 patients. No abnormality was detected in 41. Full follow up was available on 34 patients (83%). 21 patients have remained entirely free of symptoms. Of the 13 patients who had recurrent symptoms, 2 subsequently had a histologically normal appendix removed, yet still had symptoms; 2 had a second laparoscopy that showed no abnormality; 5 had ultrasound; and 4 had colonoscopy or a barium enema examination. CONCLUSION: Removal of an appendix that looks 'normal' at emergency laparoscopy for right iliac fossa pain is unjustified.

Colonic pseudo-obstruction following acute pancreatitis.

The purpose of this case presentation is to illustrate the rate association between acute pancreatitis and colonic pseudo-obstruction and to highlight the difficulties of assessing intestinal motility in a defunctioned segment of bowel prior to closure of a defunctioning stoma.