2023: Vol 10 No 11

Neurofibromatosis type 1 (NF1) is a genetic disorder that affects the skin and the neurological, ocular and skeletal systems. Many are unaware of the extent of pulmonary involvement, including lung cysts and emphysematous bullae, which enhances the risk of secondary spontaneous pneumothorax (SSP). We report the case of an 18-year-old male with NF1 who presented with acute dyspnoea and chest pain due to a right-sided pneumothorax caused by the rupture of lung apical bullae. The patient received supplemental oxygen and a chest tube of 18F was inserted, with a complete resolution of the pneumothorax. He was discharged on the third day of hospital stay.
This case highlights the importance of considering SSP as a possible clinical manifestation and complication of NF1. Early recognition and appropriate management of this condition can prevent serious complications and improve patient outcomes.

This case report presents a complex clinical scenario involving a 71-year-old female with aortic dissection accompanied by hypotension. The patient’s initial presentation of sudden loss of consciousness unveiled a large pericardial effusion and cardiac tamponade, leading to emergency surgery. Subsequent diagnostic findings revealed an intramural haematoma with an intimal tear in the ascending aorta. Postoperatively, the patient experienced an ischaemic stroke, necessitating prompt neurology consultation and treatment. This report underscores the significance of early recognition and collaborative management in achieving positive patient outcomes.

Anaesthetists and pulmonologists are well trained to follow the “can’t intubate, can’t oxygenate” (CICO) protocol but the procedure is rarely practised. This case report concerns an elective patient scheduled for endobronchial ultrasound bronchoscopy (EBUS) because of suspected sarcoidosis. Based on known medical history, anaesthesia for EBUS procedure was initiated with a laryngeal mask. The airway turned out to be difficult and the patient was not ventilable despite several efforts including curarization and orotracheal intubation. Rapid desaturation imposed to apply the CICO protocol with emergency cricothyroidotomy as extreme measure but also failed. 6-handed face mask ventilation was continued. Eventually, introduction of a microlaryngeal tube of the 3^rd generation laryngeal mask, placed on the fibrescope, allowed endotracheal intubation. The patient fell into pulseless electrical activity, and the CICO protocol was started. Immediate cardiopulmonary resuscitation totally recovered vital functions. In the post-operative follow-up, no temporary or permanent cardiological and neurological sequels were found, but new medical history such as inconstant use of C-PAP (Continuous Positive Airway Pressure) and a significant weight gain since the last notable difficult intubation were uncovered, which explained the patient’s compromised airways. Had this information been available prior to the scheduled operation, it would have indicated awake intubation with a local anaesthesia of the oropharynx and appropriate sedation of the patient.

We present a rare case of large bilateral pleural effusion, pericardial effusion and pneumomediastinum caused by a peripherally inserted central catheter (PICC) line rupturing the left brachiocephalic vein, causing severe respiratory failure. The PICC line had been inserted with the aim of providing total parenteral nutrition (TPN). The patient developed symptoms within a day with effusions diagnosed on computer tomography pulmonary angiogram (CTPA). Bilateral pleural drains were inserted with a white milk-like substance drained consistent with TPN, prompting a further review of the CTPA revealing the mediastinal-positioned PICC line. The patient was transferred to the thoracic unit and was successfully managed with conservative measures. We propose some ideas such as the use of intracavitary electrocardiogram (IC-ECG) guidance as an adjuvant to obtain a correct and safe position.

Spontaneous intracranial hypotension (SIH) is a rare cause of persistent headache, particularly among young and middle-aged people. A potentially life-threatening complication of SIH is a cerebral venous thrombosis (CVT). The authors present a case in which a patient presented with SIH complicated by CVT one week after receiving a booster dose of mRNA COVID-19 vaccine. According to our literature review, this is the first such case report.

Primary hepatic lymphoma (PHL) is extremely rare, accounting for less than 1% of all lymphomas, and is limited to the liver without extrahepatic involvement. A 30-year-old male was admitted in the Emergency Department complaining of weakness, fever, night sweats, significant weight loss, discrete ring alopecia, hepatomegaly, right axillary adenopathy and oedema of both legs. Laboratory evaluation showed normocytic normochromic anaemia, thrombocytosis, hyperbilirubinemia, cholestasis and increased international normalised ratio (INR). A computed tomography (CT) scan found an enlarged liver with a heterogeneous structure and moderate ascites. After admission in our ward further investigation revealed increased sedimentation velocity, ferritin and serum lactate dehydrogenase. A hepatic biopsy was performed which confirmed the diagnosis as a nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). The patient was transferred to a haematological ward and underwent chemotherapy with six cycles of R-CHOP. He is in complete remission after a year and half since the beginning of treatment. NLPHL, a very rare lymphoma, is more common in men between the third and fifth decades of life. Usually, the symptoms are very unspecific; a few patients have B symptoms at admission. This kind of presentation is also common in infectious, metabolic and autoimmune diseases, which were excluded in this case. Due to technical issues the final diagnosis was only possible due to the liver biopsy. Treatment with standard Hodgkin lymphoma protocols leads to complete remission in more that 95% of patients with NLPHL.

Introduction: Immune thrombocytopenic purpura (ITP) can be induced by several drugs but there are few case reports of ITP induced by clopidogrel. Second-line treatment with thrombopoietin receptor agonists (TPO-RA) presents solid evidence and should be considered in patients in need of elective surgery who are poor responders to steroids.
Case description: We report the case of a 79-year-old male who developed severe immune thrombocytopenic purpura after initiating treatment with clopidogrel. Because he needed elective orthopaedic surgery and he did not respond to corticotherapy and immunoglobulin, second-line treatment with romiplostim was initiated with a significant increase in platelet count.
Discussion and conclusion: Clopidogrel can induce ITP and this diagnosis should be considered in patients who present with isolated thrombocytopenia. First-line therapy of ITP is not always successful; second-line treatment with TPO-RA has a high response rate and should be considered in patients in need of elective surgery who have failed to respond to first-line therapy.

Lutembacher’s syndrome is a rare disease defined as the combination of atrial septal defect and mitral stenosis, which relieves the progression of symptoms resulting from mitral stenosis. We present a case of a previously asymptomatic 70-year-old man having Lutembacher’s syndrome with sinus venosus type of interatrial defect associated with partial anomalous pulmonary venous drainage. Our patient’s only complaint was of mild exertional dyspnea, which started six months prior to referral. Clinical suspicion of the combination of defects arose during transthoracic echocardiography. The patient underwent transesophageal echocardiography and multi-slice computed tomography for defect confirmation. The combination of Lutembacher’s syndrome with this complex deformity of pulmonary venous drainage has rarely been described. Such complex congenital defects with multifaceted physiological repercussions can go largely undiagnosed for the patients affected for most of their lifetimes.

This case report addresses segmental arterial mediolysis (SAM), a rare non-inflammatory vasculopathy. A 51-year-old man presented at the emergency department for epigastric and left upper quadrant pain. He had a history of arterial hypertension and had recently received methylprednisolone for knee pain. Blood tests revealed elevated C-reactive protein levels at 40 mg/l and lactate dehydrogenase levels at 496 IU/ml. Abdominal computerized tomography showed arterial thickening, arterial dilatations, and dissections of the splenic and renal arteries, leading to organ ischemia. This case emphasizes the importance of considering SAM in cases of unexplained abdominal pain or suspected arteriopathy.

Background: IgA vasculitis and hypersensitivity reactions following exposure to non-steroidal anti-inflammatory drugs (NSAIDs) are very rarely associated with purpura fulminans (PF). The latter is a coagulation event characterised by decreased levels of protein C and a rapidly progressive purpuric rash, often leading to ischaemia, amputations and death.
Case summary: A previously healthy 66-year-old man presented with a vasculitic rash and abdominal pain following exposure to naproxen (NSAID), which quickly deteriorated to purpura fulminans-like eruption and skin necrosis, mainly involving the face and hands. The presence of IgA sediments on skin biopsy and decreased levels of complement as well as protein C pointed to an immune-mediated inflammatory process. Dramatic clinical escalation with immediate risk to organs and life required an aggressive and broad-spectrum therapeutic approach in an intensive care setting. Clinical improvement and complete reconstitution of protein C were achieved following plasma exchange with fresh frozen plasma (FFP) and immunosuppression with glucocorticoids with no persistent organ damage.
Conclusions: This rare case illustrates the catastrophic cross links between NSAIDs, IgA-mediated hypersensitivity vasculitis and purpura fulminans-like syndrome. A high index of suspicion is required for the evaluation of environmental exposures such as drugs and infections in patients with vasculitis and/or purpura. A rapid and comprehensive therapeutic approach should be implemented to avoid multi-organ damage, amputations and death. Complete avoidance of the offending agent is key for future prevention of recurrence.

Salmonella osteomyelitis is an underdiagnosed pathology with unusual clinical presentations. The patient was a 24-year-old female with no previous medical history who presented to the emergency department with progressive pain in the left arm for several months.
She was initially treated unsuccessful with augmentin for 7 days for suspicious cellulites. Standard elbow X-rays described a lesion initially considered as metastatic. Investigations were completed with magnetic resonance imaging (MRI) compatible with osteomyelitis. Following surgical sampling, salmonella enterica infection was diagnosed. The patient recovered full use of her limb after 8 days of IV antibiotic therapy with third-generation cephalosporin, and she completed 11 weeks of antibiogram-targeted PO fluoroquinolone therapy. Salmonella osteomyelitis could mimic other diseases, making diagnosis difficult.

We report the first case of monkeypox virus (MPXV) associated acute respiratory distress syndrome (ARDS). A 34-year-old French woman with no medical history was admitted to the intensive care unit (ICU) for fever, altered mental status, hypotension and hypoxaemia. She presented with a diffuse skin rash with vesiculopustular lesions involving the four limbs and perineal ulcers with a skin swab positive for MPXV. On day 2, the patient presented moderate ARDS requiring invasive mechanical ventilation. She also had pleural empyema due to Streptococcus pyogenes. MPXV PCR was positive in the bronchoalveolar lavage, the pleural effusion and the blood. The patient was treated with tecovirimat. Despite the treatment, she had persistent viraemia for at least ten days. The patient condition rapidly improved; she was weaned from mechanical ventilation on day 18 despite the persistence of radiological lung opacities. She fully recovered and was discharged home on day 38 after admission.

Operative hysteroscopy intravascular absorption (OHIA) syndrome is a rare and potentially life-threatening complication related to irrigation fluid systemic absorption during hysteroscopy. It can lead to severe electrolyte disturbances, cerebral and pulmonary oedema, dysrhythmias and coagulopathy. We present the case of a 30-year-old woman who underwent a hysteroscopic myomectomy. After absorbing 2.5 l of normal saline, she experienced haemodynamic instability, respiratory distress and severe metabolic acidosis, initially mistaken for an anaphylactic or haemorrhagic shock. Insufficient monitoring of fluid deficit and irrigation fluid pressures contributed to the condition. This case underscores the importance of recognising OHIA and its risk factors to enable timely intervention and prevent adverse outcomes. Close fluid balance monitoring is vital in hysteroscopic surgeries to mitigate OHIA development.