Paediatric and adolescent ectopic Cushing's syndrome: systematic review.

OBJECTIVE: The data on clinical, biochemical, radiological characteristics, and outcomes in paediatric ectopic adrenocorticotropic hormone syndrome (EAS) are limited owing to rarity of the condition. We report three new cases and perform a systematic review of paediatric EAS. DESIGN AND METHOD: Case records of paediatric and adolescent EAS patient's ≤20 years presenting at our centre between 1997 and 2021 were retrospectively reviewed, and a systematic review of the literature published between January 1970 and December 2022 was performed. RESULTS: A total of 161 patients including 3 new patients from our centre were identified. Bronchial neuroendocrine tumours (NET) (28.5%), thymic NET (22.9%), primitive cell-derived tumours (18.6%), and gastro-entero-pancreatic-NET (13.7%) were the common causes. Primitive cell-derived tumours were the most common in the first decade (24/45, 53.4%) and were the largest (82 [60-100] mm), whereas bronchial NETs predominated during the second decade (42/116, 36.2%) and were the smallest (15 [10-25] mm). Computed tomography localized 92.9% (118/127) of paediatric EAS patients. Immediate postoperative remission was attained in 77.9% (88/113) patients, whereas 30.4% (24/79) relapsed over a median (IQR) period of 13 (8-36) months. Over a median (IQR) follow-up of 2 (0.6-4.6) years, 31.4% of patients died. The median survival was higher in bronchial NET than in other tumour groups. Distant metastasis and tumour size were independent negative predictors of survival. CONCLUSIONS: Aetiological profile of paediatric and adolescent EAS is distinct from that of adults. Bronchial NETs have the best long-term survival, whereas distant metastasis and tumour size predict poor survival.

Slipped capital femoral epiphysis as primary presentation in an adolescent with primary hyperparathyroidism due to ectopic mediastinal parathyroid adenoma.

Key Clinical Message: Ectopic mediastinal parathyroid adenoma causes primary hyperparathyroidism presenting as hypercalcemia. When children with hypercalcemia present with slipped capital femoral epiphysis, a detailed evaluation for hypercalcemia must be done before surgery. Abstract: The association between slipped capital femoral epiphysis (SCFE) and hyperparathyroidism has been reported and is rare. Each is known to affect different age groups. We report a case of a 13-year-old boy with SCFE and primary HPT leading to hypercalcemia and skeletal deformities.

Pulmonary artery pseudoaneurysm: an unsuspected cause of haemoptysis in post-COVID fungal infection of the chest.

Pulmonary Artery Pseudoaneurysm (PAP) is a rare but potentially fatal cause of haemoptysis, which often remains unsuspected by both clinicians and radiologists. Traditionally, infections like tuberculosis and bacterial endocarditis have been associated with the development of PAPs. However, additional causative factors like trauma, neoplasia, pulmonary hypertension and vasculitis are also to be considered. With the advent of the novel Coronavirus (COVID-19), attempts have been undertaken to study its multisystem implications. Also, a strong correlation has also been established between COVID-19 and fungal infestation of the paranasal sinuses and lung parenchyma. Hence, PAP should be suspected in post-COVID patients who develop new-onset haemoptysis or new focal consolidation on imaging. Imaging investigations like chest radiograph, CT chest, and CT Pulmonary Angiography help in the establishment of a diagnosis and assessment of the relevant anatomy, which aid in the classification of the PAP. Management strategies include endovascular treatment, surgical resection or conservative approach in form of prolonged antimicrobial therapy. Interventional radiological procedures like endovascular embolisation are especially useful in vitally unstable cases of massive haemoptysis who are poor surgical candidates. Our case highlights the unique presentation of pulmonary arterial pseudoaneurysm induced by a post-COVID-19 fungal infection.

Traumatic pseudocyst of the lung following blunt trauma to the chest.

Traumatic pseudocysts of the lung are a rare complication following blunt trauma to the chest. Differentiating them from other cystic lesions of the lung is important. Traumatic pseudocysts usually occur in children and young adults who present with haemoptysis and persistent pain. History of trauma and radiological evaluation with a chest radiograph and a CT scan help in making the diagnosis. Follow-up chest radiographs showing resolution of the pseudocyst can confirm thediagnostic confirmation. This is a report of one such patient, a young man who improved after conservative treatment, with spontaneous radiological resolution of the pseudocyst.

Pseudomyxoma retroperitonei masquerading as a psoas abscess.

A 52-year-old woman was referred to our department with multiple discharging sinuses and swelling in the right flank and iliac region for the past year. Ultrasound revealed a large collection in the right psoas muscle with the sinus tract reaching up to the skin in the right iliac region. Despite repeated attempts at drainage, the collection continued to increase in size. CT was requested and revealed a large heterogeneous irregular collection in the right psoas with fistulous communication with the cecum and skin with the erosion of the overlying ilium. Because of lack of vertebral involvement, enhancing internal septations, non-visualisation of the appendix and feculent material admixed with mucinous discharge from the sinus, pseudomyxoma retroperitonei secondary to ruptured mucinous neoplasm of the appendix was suspected. Mucoid material at the local site was sent for histopathology, which confirmed our suspicion. Our treatment plan after ileostomy was cytoreductive surgery along with adjuvant radiotherapy (40 Gy in 20 fractions) with chemotherapy (5-fluorouracil and folinic acid given for 30 weeks, once a week). However, after ileostomy, the patient refused further treatment, citing financial reasons.

Oesophageal lung: a rare cause of complete hemithorax opacification.

Congenital bronchopulmonary foregut anomalies are uncommon group of disorders that reflect upon the embryological development of the foregut. These conditions represent the intimate embryological proximity of the foregut and tracheobronchial tree. The radiological findings are typically of segmental or lobar consolidation with abnormal vascular supply or foregut communication. We report a case of a breathless neonate with oesophageal origin of the right main bronchus. This communication was well demonstrated with the help of an oesophagogram. The radiologist plays an important role by identifying this communication on a CT done for non-resolving lung collapse. Contrast-enhanced CT of the chest is also useful in evaluating the vascular supply of the lung that helps in diagnosis and also directs treatment.

Hysterosalpingography and ultrasonography findings of female genital tuberculosis.

Genital tuberculosis (TB) is an important cause of female infertility in the world, especially in developing countries. Majority of infertility cases are due to involvement of the fallopian tubes (92%-100%), endometrial cavity (50%), and ovaries (10%-30%); cervical and vulvovaginal TB are uncommon. Genital TB has characteristic radiological appearances based on the stage of the disease process (acute inflammatory or chronic fibrotic) and the organ of involvement. Hysterosalpingography (HSG) and ultrasonography (US) remain the main imaging modalities used in the diagnosis of genital TB. HSG is the primary modality for evaluating uterine, fallopian tube, and peritubal involvement and also helps in evaluating tubal patency. US, on the other hand, allows simultaneous evaluation of ovarian and extrapelvic involvement.