Hrev_master [page 146] [Emergency Care Journal 2020; 16:8911] Emergency Care Journal 2020; volume 16:8911 Abstract We present a case of a 66-year-old man with history of myas- thenia gravis, severe obesity and osteopenia self-presented to our Emergency Department (ED) with severe pain in his left hemitho- rax, occurred after an episode of cough three days before. No his- tory of trauma was reported. The patient underwent a chest-XR showing uncomplicated spontaneous fractures of the 5th and 6th left ribs. He was therefore discharged with appropriate analgesic treat- ment. Five days later, the patient came back to our ED for a wide left abdominal hematoma, though hemodynamically stable and eupneic. A CT-scan with contrast showed a rare and unexpected spontaneous left intercostal lung herniation complicated with a dif- fuse subcutaneous emphysema, pneumothorax, loculated bilateral pleural effusion and abdominal hematoma. The patient was admit- ted, treated conservatively and safely discharged after two weeks. We also provide a pathophysiological discussion of the case and a literature review. Case presentation A 66-year-old man self-presented to our Emergency Department (ED) with severe and increasing pain in his left hemithorax, occurred after an episode of cough three days before. No history of trauma was reported. The patient had medical history of myasthenia gravis, severe obesity, ulcerative colitis and type-2- diabetes in chronic treatment with prednisone 12,5 mg/day, repaglinide and metformin. In 2006, he was diagnosed with osteopenia and a spontaneous vertebral compression fracture. The patient was also an active smoker. He underwent a chest-RX (CXR) which demonstrated com- pound fractures of the 5th and 6th left ribs (Figure 1), classified as uncomplicated and spontaneous. The patient was therefore dis- charged with appropriate analgesic treatment. Five days later, the patient came back to our ED for a wide left abdominal hematoma. The patient was hemodynamically stable and eupneic. The only symptom he reported was some tenderness in the site of the rib fractures occurred on the same day. He denied once again any traumas. The physical examination was inconclu- sive: a wide hematoma was found in the left side of the abdomen; the left rib cage was tender at superficial palpation and the respira- tory sounds were reduced in the inferior left lung field. Ordinary laboratory tests were taken, showing normal hemo- globin levels, leukocytosis (WBC: 18,2 x 103 μL), increased C- reactive protein (CRP: 281,3 mg/L) and procalcitonin (PCT: 0,34 ng/mL, normal value <0,05). The Arterial Blood Gas (A.B.G.) demonstrated a moderate respiratory insufficiency with chronic compensated respiratory acidosis (pH 7,4; pCO2 48,4 mmHg; pO2 89 mmHg; HCO3- 29; PaO2/FiO2=287). Coagulation and other blood tests were all in the normal range normal. A plain CXR was performed, showing a displacement of the rib fragments, possible bibasal consolidations and a left pleural effusion (Figure 2). The lung ultrasound pattern was unclear, showing a diffuse ultrasonographic barrier effect. In the suspicion of a hemothorax, a complete chest and abdom- inal CT-scans with contrast were performed, describing an unex- pected picture of moderate intercostal lung herniation (2,3 cm) in the 5th space, a diffuse subcutaneous emphysema from the neck to the abdomen, a minimal left-sided pneumothorax and mild-to- moderate bilateral pleural effusion, partially loculated in the left side. The CT-scan also confirmed a non-refurnished abdominal wall hematoma and the known 5th and 6th ribs detached fractures (Figures 3-5). Our patient underwent an urgent surgical consult, suggesting a conservative approach in a medical ward. The patient did not require non-invasive ventilatory supports, but we administered oxygen via face mask and nasal canulae. A prophylactic antibiotic regimen was started first with amoxicillin/clavulanic acid then switched to piperacillin/tazobactam on the 4th day. During the hos- pitalization, Hb values decreased to a minimum of 12,0 g/dL. No blood transfusions were prescribed. The patient was discharged after 15 days, with complete normalization of the clinical picture. Further CXRs in the following months described a complete spon- taneous reduction of the hernia. Correspondence: Nicola Artusi, Medicina d’urgenza, Strada di Fiume 447, 34149 Trieste TS, Italy E-mail: nicola.artusi.1@gmail.com Key words: Lung herniation; spontaneous; pulmonary hernia; obesity; myasthenia gravis. Conflict of interest: No one. This work was not supported by any grant. Ethics approval and consent to participate: The manuscript does not contain any elements that would allow the recognition of the patient. Received for publication: 21 February 2020. Accepted for publication: 18 May 2020. This work is licensed under a Creative Commons Attribution 4.0 License (by-nc 4.0). ©Copyright: the Author(s), 2020 Licensee PAGEPress, Italy Emergency Care Journal 2020; 16:8911 doi:10.4081/ecj.2020.8911 A serious fit of cough: A 66-year-old patient with myasthenia gravis presenting with spontaneous intercostal lung herniation after coughing. Case report and pathophysiological discussion Riccardo Gerloni,1 Nicola Artusi,1 Ugo Giulio Sisto,1 Saverio Tollot,2 Roberto Copetti1,3 ¹Emergency Department, University Hospital of Cattinara; ²Radiology Department, University Hospital of Cattinara, Trieste; ³Emergency Department, Hospital of Latisana, Latisana, Italy Non -co mmerc ial us e o nly [Emergency Care Journal 2020; 16:8911] [page 147] Discussion Spontaneous rib fractures are a common complication in osteopenic patients affected by respiratory infections, particularly in those patients treated chronically with corticosteroids. The ini- tial evaluation and management of spontaneous stress fractures of the ribs involve the ruling out of main complications such as pneu- mothorax and thoracic bleeding. Further, a correct pain manage- ment is required: an association of paracetamol, NSAIDs/COX-2 inhibitors and as needed opioids (i.e. codeine, tramadol, etc.) is suggested, with an increasing role of ultrasound-guided locore- gional anesthesia.1 Since the surgical fixation is rarely needed, the usual approach considers early discharge and conservative therapy as first choice. The late development of a thoracic and abdominal wall hematoma suggested a delayed intercostal hemorrhage.2 The patient never reported the typical signs or symptoms of intercostal lung herniation such as a palpable soft mass or worsening dyspnea. Though a thoracic wall hematoma is frequently associated with lung hernias, it’s unspecific for this condition.3 The initial physical examination was possibly misled by the severe obesity of the patient. Despite the attested sensitivity and specificity of chest ultrasound in the ED for pleural effusions, rib fractures and pneu- Case Report Figure 1. laying AP CXR (panel A) and left hemithorax (panel B) showing a compound antero-lateral fracture of the 5th and 6th left ribs (in the circle) and an elevation of the left hemidiaphragm. Figure 2. Standing AP CXR showing possible detachment of the fracture lines (a), subcutaneous emphysema (b), left pleural effusion (c), possible bibasal consolidations and a prominent elevation of the left hemidiaphragm (d). Non -co mmerc ial us e o nly [page 148] [Emergency Care Journal 2020; 16:8911] mothorax and, therefore, it’s possible role in the detection of a lung herniation,4 the concomitant presence of obesity and subcutaneous emphysema reduced the diagnostic power of lung ultrasound.5 The first standing CXR was inconclusive. A CT-scan with contrast was therefore indicated in this condition.6 The CT-scan with contrast showed a self-limiting intercostal hemorrhage with no active bleeding, not needing for interventional hemostasis or blood trans- fusions. The CT-scan interestingly reported a lung herniation and subcutaneous emphysema, too. Lung herniations are rare findings in non-traumatic fractures, mostly associated with corticosteroid chronic therapies, primitive pulmonary affections, or previous invasive procedures.10,11 In our patient, the predisposing factor is likely to be the prednisone therapy. Corticosteroid-induced osteopenia is a common side-effect in chronic corticosteroid treat- ments; (in these patients, the bone samples show a lower density in osteoblasts and an increased rate of osteocyte apoptosis, with a general reduction in bone strength. On the other hand, glucocorti- coid effects include an increase in osteoclasts lifespan).12-15 Anyway, the rib fractures alone can’t explain the lung hernia- tion, thus a global thoracic wall weakness needed to be present. Case Report Figure 3. CT-scan coronal sections in lung window (panel A) and soft tissue window (panel B) showing the pulmonary herniation (a), the minimal pneumothorax (b) and the diffuse subcutaneous emphysema (c). Figure 4. CT-scan transverse sections (cranial, panel A, and caudal including the spleen in panel B) showing the pulmonary herniation (a), the left sided loculated pleural effusion (b), the right sided pleural effusion (c) and the diffuse subcutaneous emphysema (d). Non -co mmerc ial us e o nly [Emergency Care Journal 2020; 16:8911] [page 149] Actually, corticosteroid activity over muscle catabolism is well known: glucocorticoids inhibit protein synthesis while stimulating protein degradation, possibly leading to a skeletal muscle atrophy in several acute and chronic condition. Glucocorticoid-induced myopathy is a common complication in Cushing-like-syndromes which induce diffuse atrophy and substitution of muscle fibres, affecting both limbs and respiratory muscles.16,17 Severe obesity can be involved in the pathogenesis of lung her- niation, too, both due to its increasing effect on intrathoracic pres- sure, and to a diffuse respiratory muscle overstretching and related weakness.18 Myasthenia Gravis (MG) affects the neuromuscular junction, resulting in a localized or diffuse muscle weakness which can involve the respiratory system. Many clinical conditions can exac- erbate MG, as infections. Even if the idea of MG possibly involved in the pathogenesis of lung herniation is appealing, our patient did- n’t show any sign of myasthenic crisis as dyspnoea, fatigue or severe respiratory insufficiency; thus, a direct involvement of MG is unlikely.19,20 The patient was treated conservatively. Though some authors suggest an early interventional approach,21 in minor hernias, a watch-and-see conservative regimen, based on pain management and rest, could be sufficient to obtain a complete spontaneous resolution. Admission to hospital in case of acute complications is suggested. In case of large hernias or severe com- plications, as hernial incarceration or strangulation, with lung necrosis, a surgical reduction is required both with invasive or tho- racoschopic approaches.22 Surgery is also the treatment of choice in combined lung and intestinal hernias and lung hernias involving the diaphragm. Our patient presented a moderate intercostal herni- ation with a risk for acute medical complications and therefore an in-hospital medical treatment was needed, with no surgical indica- tions.23 A clinical diagnosis of pneumonia could be debated. WBC count, CRP and PCT were mildly increased, suggesting a possible bacterial infection24,25 The patient never developed fever, and the serial hemocultures were constantly negative. The finding of pleu- ral effusion, absent in the first hospital presentation while moder- ate at the second admission, could be consistent either with infec- tion or with an inflammatory response. PCT was later negative after one week of antibiotic therapy. Conclusions The lung herniation is a rare either congenital or acquired con- dition, mostly following thoracic surgical procedures or thoracic traumas. Few cases of spontaneous lung herniation are reported in literature, most of them associated with rib fractures and persistent coughing, mechanical ventilation with positive pressure, emphyse- ma and COPD. The surgical reduction of the hernia is the usual therapeutic option in these reported cases. Our case depicts a spontaneous lung herniation associated with pneumothorax and subcutaneous emphysema after atraumatic rib bone fractures in a patient in chronic corticosteroid therapy for his history of myasthenia gravis. We successfully treated this patient with a conservative approach. The pathophysiological mechanism of the lung herniation in this patient is due to the side effects of a long-term glucocorticoid therapy on the thoracic wall, muscles and bones. MG is unlikely to be a causal factor. Case Report Figure 5. CT-scan, 3D-volume rendered reconstruction of the lungs, showing a) the left herniation, b) the rib footprints on the lung parenchyma, c) the parenchymal compression secondary to the pleural effusion in right lateral-anterior (panel A), superior (panel B) left lateral-anterior (panel C) and antero-inferior (panel D) views. Non -co mmerc ial us e o nly [page 150] [Emergency Care Journal 2020; 16:8911] References 1. Karmakar M, Ho AM-H. Acute pain management of patients with multiple fractured ribs. J Trauma 2003;54:615–25. 2. Talbot BS, Gange CP Jr, Chaturvedi A, et al. Traumatic Rib Injury: Patterns, Imaging Pitfalls, Complications, and Treatment. Erratum In: Radiographics 2017;37:1004.. Radiographics 2017;37:628–51. 3. Maeda T, Sato R, Luthe SK, Russell MC. Spontaneous Intercostal Lung Hernia. Am J Med 2017;130:e399-e400. 4. O’ Mahony AM, Murphy KM, O’Connor TM, Curran DR. Spontaneous pulmonary hernia secondary to intercostal mus- cle tear. BMJ Case Rep 2019;12:e231706. 5. Lee FCY. 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