A 14-year-old girl who presented in 2017 with headache, unilateral right eye ptosis and secondary amenorrhoea had an initial workup consistent with non-functioning pituitary macroadenoma. She underwent debulking of pituitary tumour in October 2017. Postoperatively, she developed recurrent cavernous sinus thrombosis. In view of recurrent thrombosis, she was reinvestigated and was found to have adrenocorticotropic hormone-dependent Cushing. Follow-up MRI 1 year after initial presentation showed that there was structural recurrence of pituitary macroadenoma. She subsequently underwent a petrosal craniotomy for debulking of tumour. Postsurgery she remained biochemically Cushingnoid. MRI 5 months after second surgery showed an enlarging pituitary mass which was deemed inoperable. A multidisciplinary meeting discussion consensus for treatment included radiotherapy and somatostatin analogue, pasireotide. She completed 30 cycles of radiotherapy and MRI post radiotherapy showed reduction in the size of the macroadenoma. Currently, she is waiting for pasereotide initiation.
Disorders of esophagogastric junction (EGJ) outflow, including achalasia and EGJ outflow obstruction, are motility disorders characterized by inadequate relaxation of lower esophageal sphincter with or without impaired esophageal peristalsis. Current guidelines are technical and less practical in the Asia-Pacific region, and there are still massive challenges in timely diagnosis and managing these disorders effectively. Therefore, a Malaysian joint societies' task force has developed a consensus on disorders of EGJ outflow based on the latest evidence, while taking into consideration the practical relevance of local and regional context and resources. Twenty-one statements were established after a series of meetings and extensive review of literatures. The Delphi method was used in the consensus voting process. This consensus focuses on the definition, diagnostic investigations, the aims of treatment outcome, non-surgical or surgical treatment options, management of treatment failure or relapse, and the management of complications. This consensus advocates the use of high-resolution esophageal manometry for diagnosis of disorders of EGJ outflow. Myotomy, via either endoscopy or laparoscopy, is the preferred treatment option, while pneumatic dilatation can serve as a secondary option. Evaluation and management of complications including post-procedural reflux and cancer surveillance are recommended.
Diagnosing atrial myxoma in pregnancy is challenging because patients may present with non-specific symptoms that might be overlooked. The timing of non-obstetric operation usually depends on the nature of the disease, after careful consideration of feto-maternal safety, including the use of cardiopulmonary bypass and placental transfer of anaesthetic drug. A woman in her 30s at 18 weeks of pregnancy presented with recurring dizziness. She underwent successful myxoma excision at 20 weeks under general anaesthesia and cardiopulmonary bypass. The 6×5 cm myxoma was histologically confirmed as myxoma. Early detection of atrial myxoma in pregnancy is crucial, and a clinician has to consider the diagnosis of left atrial myxoma with mitral valve obstruction as a cause of severe dizziness. Optimal outcomes require multidisciplinary management. In this case, surgery during the second trimester of pregnancy enabled a full-term pregnancy with the patient's and foetal well-being and normal postprocedural echocardiography.
A middle-aged woman with recurrent malignant melanoma presented initially with massive left pleural effusion. There was a complete obliteration of the left main bronchus on flexible bronchoscopy caused by a mass. Serial cryo-debulking of the tumour was done under rigid bronchoscopy; however, the outcome was not favourable due to the aggressive tumour growth. Vemurafenib was planned after thoracic radiation. She was not keen for the biologics treatment due to financial constraints. We report a case of central airway obstruction due to recurrent aggressive melanoma. More evaluations are needed on the role of interventional pulmonologist for bronchoscopic debulking of this rapidly growing tumour as well as the role of biological agents in treating such cases.