Birla Roy Gnanamuthu

ECTOPIC CUSHING SYNDROME: A 10-YEAR EXPERIENCE FROM A TERTIARY CARE CENTER IN SOUTHERN INDIA

OBJECTIVE Ectopic adrenocorticotropic hormone (ACTH) secretion is a less common cause of Cushing syndrome and is seen in 5 to 10% of cases with endogenous hypercortisolemia. We hereby describe our experience of patients with ectopic ACTH syndrome, who have been managed over the past 10 years at a tertiary care center in Southern India. METHODS The inpatient and outpatient records of patients from 2006 to 2015 were retrospectively reviewed. The clinical features, clinical history, biochemical values, imaging features, including radiologic findings and positron emission tomography scans, management, details of follow-up, and outcomes, were documented. We compared the biochemical findings in these patients with 20 consecutive patients with Cushing disease (Cushing syndrome of pituitary origin). RESULTS A total of 21 patients were studied. The median age at presentation was 34 years (range, 19 to 55 years). Seven patients had thymic carcinoid, 7 had bronchial carcinoid, 3 had lung malignancies, 2 had medullary carcinoma thyroid, 1 patient had a pancreatic neuroendocrine tumor, and 1 patient had an occult source of ACTH. The most common clinical features at presentation were muscle weakness (95%), hyperpigmentation (90%), facial puffiness (76%), easy bruising (61%), edema (57%), and striae (52%). Extensive acne was seen in a large number of patients (43%). Only 3 patients (14%) had central obesity. The median 8 am cortisol was 55.5 μg/dL (range, 3.8 to 131 μg/dL), median 8 am ACTH was 207 pg/mL (range, 31.1 to 703 pg/mL), and the median 24-hour urinary free cortisol was 2,484 μg (range, 248 to 25,438 μg). Basal cortisol and ACTH, as well as midnight cortisol and ACTH level, were markedly higher in patients with ectopic Cushing syndrome as compared to patients with Cushing disease. Twelve of 21 patients had developed life-threatening infections by follow-up. Nine patients had undergone surgical intervention to address the primary tumor. However, only 1 patient exhibited a complete cure on follow-up. CONCLUSION In our series, ectopic Cushing syndrome was most commonly seen in association with intrathoracic tumors such as bronchial or thymic carcinoid. Hyperpigmentation and proximal myopathy were frequent, while central obesity was uncommon. Early and rapid control of hypercortisolemia was important in order to prevent life-threatening infections and metabolic complications. ABBREVIATIONS ACTH = adrenocorticotropic hormone CT = computed tomography DOTATATE = 68Ga-DOTA-Tyr3-octreotate ECS = ectopic Cushing syndrome FDG = fluorodeoxyglucose MTC = medullary thyroid cancer NET = neuroendocrine tumor PET = positron emission tomography.

Cushing's syndrome in a case of thymic carcinoma

A 29-year-old gentleman presented with episodic features suggestive of Cushings syndrome. He was evaluated and diagnosed with ectopic Adrenocorticotropic hormone (ACTH)–dependent Cushings syndrome due to a thymic tumor. The thymic lesion was excised and histopathology confirmed thymic carcinoma with neuroendocrine differentiation, with local, perineural, and vascular invasion. The postoperative problems and further treatment options have been discussed in this case report.

Embolization of the Device to the Left Pulmonary Artery after the Interventional Closure of Ruptured Sinus of Valsalva Aneurysm.

Formation of an aneurysm in the sinus of Valsalva of the aortic root is usually due to an area of congenital weakness in its wall. This aneurysm may progressively dilate and rupture into any of the cardiac chambers or into the pericardial cavity. Though this is conventionally treated by surgery, interventional therapy using various closure devices is becoming more common. Embolization of these closure devices may occur. We report a case of embolization of such a device into the left pulmonary artery which during surgical retrieval, unmasked the hidden ventricular septal defect (VSD). Therefore one has to be cautious while making a diagnosis of rupture of the sinus of Valsalva of right coronary sinus without VSD.

Endovascular treatment of isolated arterial pulmonary malinosculation

We report the endovascular management of a series of four cases of isolated systemic supply to normal lung or isolated arterial pulmonary malinosculation of the left lung. In these cases, the basal segments of the left lung lacked normal pulmonary arterial supply and instead received systemic arterial blood from the descending thoracic aorta. The relevant anatomy and literature are also reviewed.

Giant bullae of the lung presenting in the neck

A 41-year-old gentleman presented with a painful 10 × 8 cm hyperresonant swelling with an expansile cough impulse, on the right side of the lower neck for 2 weeks (Fig. 1a). The swelling increased with Valsalva maneuver but was not reducible. Chest X-ray showed a well-defined radiolucent lesion in the neck, extending into the upper zone of the right lung field (Fig. 2a). Computed tomography (CT) scan showed a welldefined air-containing lesion in the right supraclavicular region with no communication between the lesion and the major airways or pharynx, later confirmed by fiber-optic bronchoscopy (Fig. 2b and c). There was no intrathoracic component of the lesion. A diagnosis of a cervical bulla was made and he was taken up for surgical excision of the same. At surgery, there was a multiloculated thin-walled bulla in the right supraclavicular region with a narrow pedicle attached to the apex of the upper lobe through the thoracic inlet (Fig. 1b).

A rare aneurysm of the sinus of Valsalva—from the left sinus

Sinus of Valsalva aneurysm (SVA) is a rare anomaly which may be congenital or acquired. We present a rare case of an unruptured calcified SVA arising from the left coronary sinus and extending into the left ventricle. Transesophageal echocardiogram revealed moderate aortic regurgitation. The aneurysm was repaired using a synthetic fabric patch preserving the native valve. Only one case of unruptured left SVA prolapsing into the left ventricle has been reported so far in the English literature. Such lesions are rare since the high pressure of the left ventricle prevents the prolapsing of the aneurysm into it.

Late complication of a chest wall injury—images of an internal mammary artery pseudoaneurysm

A 59-year-old gentleman presented with complaints of right-sided chest pain since 8 months. The pain was in the same area of a penetrating injury sustained in the right parasternal region 20 years earlier. It had resulted in an osteomyelitis and had healed with a course of antibiotics. A chest X-ray (Fig. 1a) showed a well-rounded homogenous parasternal opacity behind the second and third costal cartilages. A computerized tomogram of the chest (Fig. 1b; Fig. 2a, b) and magnetic resonance angiogram (Fig. 3a, b) revealed a partially thrombosed pseudoaneurysm of the right Internal mammary artery (IMA) with a minimal haemothorax. The aneurysm was successfully embolized with a coil and the haemothorax managed conservatively.

Staphylcoccus aureus tricuspid valve endocarditis in a neonate

Neonatal endocarditis has a have a high mortality rate. Its signs and symptoms are rarely distinguishable from that of sepsis, since extra cardiac manifestations of endocarditis are seldom present. Staphylococcus is the most common causative organism in a neonate, mostly infecting neonates with congenital cardiac defects or indwelling central venous catheters. Failure of medical treatment warrants surgical intervention in some children. We present a report with echocardiographic images of a neonate with staphylococcal tricuspid valve endocarditis. A 12 day old term neonate, born by normal vaginal delivery presented with a history of fever and listlessness for 5 days and seizures the previous day. She had been treated elsewhere with antibiotics through a peripheral venous line for the past 4 days. There was no history of umbilical infection or central venous catheterization. On presentation, she was in septic shock with cardiac failure. There were no peripheral signs of infective endocarditis. A systolic murmur was heard over the precordium. Investigations revealed pancytopenia and two blood cultures grew methicillin resistant Staphylococcus aureus. A transthoracic echocardiogram showed a 1.4×0.6 mm mobile vegetation in the anterior tricuspid leaflet (Fig. 1), causing stenosis and regurgitation (Fig. 2a and b). No other congenital cardiac anomaly was detected.

Systemic arterial supply to left lower lobe of lung—images

Anomalous systemic arterial blood supply to the lung is a rare congenital anomaly. We present an interesting set of computerized tomography images and operative pictures of a patient with this anomaly. An 18-year-old girl presented with complaints of recurrent haemoptysis for the past 10 years. A chest x-ray showed increased vascularity in the left lower lobe. A computerized tomogram of the chest revealed an aberrant systemic artery from the supra diaphragmatic portion of the aorta supplying the basal segments of the lower lobe of left lung (Fig. 1). An aortogram confirmed the same (Fig. 2) and a pulmonary angiogram showed that the left pulmonary artery terminated after supplying only the apical segment of the lower lobe (Fig. 3) Pryce et al. in 1946 first described this anomaly and considered it to be a type of sequestration [1]. However, other authors cite reasons to argue against this being called a sequestration [2]. Patients with this anomaly may be asymptomatic or present with cough, symptoms of recurrent pulmonary infections, haemoptysis, or congestive cardiac failure [2, 3]. Advancements in radiological imaging techniques have improved the chances of detection of this rare anomaly. Upon diagnosis, definitive treatment should be considered to relieve symptoms and to prevent the possible complications [2]. The treatment options include interventional procedures to occlude the abnormal artery, anastomosis of the aberrant artery onto the pulmonary artery, or surgical division of the abnormal artery with or without a concomitant lobectomy [2–7]. A review of English literature indicates that all options other than the surgical division of the artery with concomitant lobectomy may have an occasional complication or poor outcome [6, 7]. Fig. 1 Computerized tomogram of the chest showing a large aberrant artery arising from the aorta and supplying the left lower lobe of lung

Unusual cystic lesions within pleural effusion.

A 50-year-old man presented with chronic chest pain and recent worsening. Ruptured pleural hydatidosis was suspected based on imaging findings (Figure 1). Surgery showed a lobulated soft tumor along the thoracic sympathetic chain. The histopathology was diagnostic for schwannoma (Figure 2). The majority of schwannomas are predominantly solid tumors. Occasionally they may be purely cystic lesions that are indistinguishable from other congenital and acquired cystic lesions, as in our patient. Funding