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Translational Pediatrics Apr 2024In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development... (Review)
Review
In congenital diaphragmatic hernia (CDH), abdominal organs are displaced into the chest, compress the lungs, and cause mediastinal shift. This contributes to development of pulmonary hypoplasia and hypertension, which is the primary determinant of morbidity and mortality for affected newborns. The severity is determined using prenatal imaging as early as the first trimester and is related to the laterality of the defect, extent of lung compression, and degree of liver herniation. Comprehensive evaluation of fetal CDH includes imaging-based severity assessment, severity assessment, and evaluation for structural or genetic abnormalities to differentiate isolated from complex cases. Prenatal management involves multispecialty counseling, consideration for fetal therapy with fetoscopic endoluminal tracheal occlusion (FETO) for severe cases, monitoring and intervention for associated polyhydramnios or signs of preterm labor if indicated, administration of antenatal corticosteroids in the appropriate setting, and planned delivery to optimize the fetal condition at birth. Integrated programs that provide a smooth transition from prenatal to postnatal care produce better outcomes. Neonatal care involves gentle ventilation to avoid hyperinflation and must account for transitional physiology to avoid exacerbating cardiac dysfunction and decompensation. Infants who have undergone and responded to FETO have greater pulmonary capacity than expected, but cardiac dysfunction seems unaffected. In about 25-30% of CDH neonates extracorporeal life support is utilized, and this provides a survival benefit for patients with the highest predicted mortality, including those who underwent FETO. Surgical repair after initial medical management for the first 24-48 hours of life is preferred since later repair is associated with delayed oral feeding, increased need for tube feeds, and increased post-repair ventilation requirement and supplemental oxygen at discharge. With overall survival rates >70%, contemporary care involves management of chronic morbidities in the context of a multidisciplinary clinic setting.
PubMed: 38715680
DOI: 10.21037/tp-23-602 -
Saudi Journal of Anaesthesia 2024Post-pneumonectomy syndrome (PPS) is a rare complication after pneumonectomy defined by mediastinum shift toward the vacated pleural space with compression of the distal...
Post-pneumonectomy syndrome (PPS) is a rare complication after pneumonectomy defined by mediastinum shift toward the vacated pleural space with compression of the distal trachea or mainstem bronchi, resulting in dyspnea. This report describes a 32-year-old woman who presents with limiting symptoms of progressive dyspnea and chest pain 2 years after a right pneumonectomy. In computed tomography scan, there was no evidence of airway compression but suggested torsion of the inferior vena cava with preload compromise, confirmed during the surgical mediastinum repositioning using a transesophageal echocardiography-guided approach. This case report presents this unprecedented variant of PPS syndrome, highlighting the diagnostic and peri-operative management challenges.
PubMed: 38654858
DOI: 10.4103/sja.sja_855_23 -
Journal of Cardiothoracic Surgery Apr 2024Cardiac herniation occurs when there is a residual pericardial defect post thoracic surgery and is recognised as a rare but fatal complication. It confers a high...
BACKGROUND
Cardiac herniation occurs when there is a residual pericardial defect post thoracic surgery and is recognised as a rare but fatal complication. It confers a high mortality and requires immediate surgical correction upon recognition. We present a case of cardiac herniation occurring post thymectomy and left upper lobectomy.
CASE PRESENTATION
Initial presentation: A 48-year-old male, hypertensive smoker presented with progressive breathlessness and was found to have a left upper zone mass confirmed on CT biopsy as carcinoid of unclear origin. PET-CT revealed avidity in a left anterior mediastinal area, left upper lobe (LUL) lung mass, mediastinal lymph nodes, and a right thymic satellite nodule. Intraoperatively: Access via left thoracotomy and sternotomy. The LUL tumour involved the left thymic lobe (LTL), left superior pulmonary vein (LSPV), left phrenic nerve and intervening mediastinal fat and pericardium, which were resected en-masse. The satellite nodule in the right thymic lobe (RTL) was adjacent to the junction between the left innominate vein and superior vena cava (SVC). The pericardium was resected from the SVC to the left atrial appendage. Clinical deterioration: Initially the patient was doing well clinically on day 1, however there was sudden bradycardia, hypotension, clamminess, and oligoanuria, with raised central venous pressures and troponins. ECG: no capture in leads V1-2, but positive deflections seen on posterior leads. Echo: no acoustic windows, but good windows seen posteriorly. CXR: left mediastinal shift. Redo operation: After initial resuscitation and stabilisation on the intensive care unit, on day 2 a redo-sternotomy revealed cardiac herniation into the left thoracic cavity with the left ventricular apex pointing towards the spine, and inferior caval kinking. After reduction and repair of the pericardial defect with a fenestrated GoreTex patch, the patient recovered well with complete resolution of the ECG and CXR.
CONCLUSION
Cardiac herniation can even occur following sub-pneumonectomy lung resections and should be considered as a differential when faced with a sudden clinical deterioration, warranting early surgical correction.
Topics: Male; Humans; Middle Aged; Thymectomy; Vena Cava, Superior; Positron Emission Tomography Computed Tomography; Clinical Deterioration; Heart Diseases; Hernia; Pneumonectomy
PubMed: 38627781
DOI: 10.1186/s13019-024-02713-y -
Cureus Mar 2024Giant bullous emphysema (GBE) is a progressive disease that commonly presents with severe progressive dyspnea attributed to the progressive destruction of alveolar walls...
Giant bullous emphysema (GBE) is a progressive disease that commonly presents with severe progressive dyspnea attributed to the progressive destruction of alveolar walls and the formation of large air pockets, resulting in impaired gas exchange. This presentation is most commonly seen in young, thin male smokers. GBE poses an interesting and unique clinical challenge due to its radiologic findings, which can be easily mistaken for tension pneumothorax. Despite the decreased acuity of GBE as compared to tension pneumothorax, inadequate treatment in a severe case can lead to spontaneous pneumothorax, infection, and/or respiratory failure. In this report, we highlight a case of severe GBE that presents similarly to tension pneumothorax in both symptomatology and radiologic findings. The case at hand is of a 50-year-old male patient with a history of chronic obstructive pulmonary disease (COPD) with complaints of dyspnea and subsequent findings of tachycardia, tachypnea, and hypoxemia with significantly decreased breath sounds in the right lung. Radiologic findings showed increased lucency of the right hemithorax and a mass effect with a mediastinal shift to the left. History and further imaging with CT led to an ultimate diagnosis of severe GBE and COPD exacerbations. The patient was treated with non-invasive medical management. With the challenges of overlapping presentations, landing on the correct diagnosis is imperative to accurately and adequately treat the patient since GBE and tension pneumothorax significantly differ in acuity and overall management, hence the need for a high level of suspicion based on the clinical picture and the use of high-resolution CT.
PubMed: 38606232
DOI: 10.7759/cureus.55988 -
SAGE Open Medical Case Reports 2024We hereby present a 70-year-old male Ethiopian farmer who presented with clinical manifestations suggestive of acute large bowel obstruction, which had persisted for 3...
We hereby present a 70-year-old male Ethiopian farmer who presented with clinical manifestations suggestive of acute large bowel obstruction, which had persisted for 3 days. Over the past 3 years, he has experienced three similar recurring episodes, which were alleviated by decompression using a rectal tube. Upon initial examination, the patient displayed signs of cardiopulmonary distress, although no indications of peritonitis were observed. Further evaluation through plain abdominal and chest X-rays revealed findings consistent with sigmoid volvulus, as well as mediastinal shift, elevated diaphragm, and a right-positioned heart. Consequently, a sigmoid resection and an end colostomy were done. The patient showed improvement and was discharged from the hospital on the 14th day following the surgery. It is atypical for an individual to present with acute sigmoid volvulus accompanied by a mediastinal shift resulting from mass action on the hemidiaphragm. Therefore, it may be imperative to involve a pulmonologist in managing this rare situation. By ensuring that the surgical team is well-informed in such circumstances, patients can receive enhanced care and treatment.
PubMed: 38606032
DOI: 10.1177/2050313X241246658 -
Turk Gogus Kalp Damar Cerrahisi Dergisi Jan 2024Pneumothorax is a condition that describes the presence of air between the visceral and parietal pleura sheets and the consequent collapse of the lungs. The collapse of... (Review)
Review
Pneumothorax is a condition that describes the presence of air between the visceral and parietal pleura sheets and the consequent collapse of the lungs. The collapse of the lungs can be partial or total and can present in different clinical stages, such as a high-pressure pneumothorax that can cause a mediastinal shift. Pneumomediastinum is the presence of free air between the mediastinal tissues due to various causes. It can manifest spontaneously and be minimally symptomatic but can also develop due to severe complications. Its etiology includes numerous iatrogenic and traumatic factors. Although spontaneous pneumothorax and pneumomediastinum that develop in childhood are similar to adult patients, it is important to determine the appropriate treatment strategy in addition to the age group, the effectiveness of the treatment, the role of the applied treatment in reducing recurrence, and the etiologyoriented treatments if there is an underlying pathology.
PubMed: 38584791
DOI: 10.5606/tgkdc.dergisi.2024.25755 -
Bone Reports Jun 2024Tumor-induced osteomalacia is caused by excessive fibroblast growth factor 23 production mainly from phosphaturic mesenchymal tumors. Surgical excision or tumor ablation...
Healing of tumor-induced osteomalacia as assessed by high-resolution peripheral quantitative computed tomography is not similar across the skeleton in the first years following complete tumor excision.
Tumor-induced osteomalacia is caused by excessive fibroblast growth factor 23 production mainly from phosphaturic mesenchymal tumors. Surgical excision or tumor ablation are the preferred treatment. Information on bone microarchitecture parameters assessed by high-resolution peripheral quantitative computed tomography is limited. We report a woman with hypophosphatemic osteomalacia with generalized pain, weakness and recurrent fractures, and a large thoracic vertebral mass extending to the posterior mediastinum. Detailed radiologic and histopathologic evaluation revealed a phosphaturic mesenchymal tumor. Two surgeries were necessary for complete removal of the mass. Clinical symptoms improved after attaining normophosphatemia. Four-year post-surgical HR-pQCT parameters, compared to baseline, showed in the left distal radius, stable trabecular and cortical volumetric bone mineral density although below reference range. There was stability of trabecular number and thickness. Both stiffness and failure load decreased. A shift in cortical parameters was noted in year 2. In the left distal tibia, trabecular volumetric bone mineral density decreased whereas cortical volumetric bone mineral density markedly increased, as did cortical area. There was stability in the trabecular number and thickness. Both stiffness and failure load improved. Findings from HR-pQCT measurements in this patient disclosed that the healing of osteomalacia is not similar across the peripheral skeletal sites in the first years following tumor removal. Results contrasted low but stable volumetric bone mineral density in the distal radius with increase in the distal tibia at the expense of cortical bone. Our report helps further delineate the pattern of bone healing after treatment of this rare bone disorder.
PubMed: 38584681
DOI: 10.1016/j.bonr.2024.101758 -
Mucosal Immunology Apr 2024Sublingual allergen immunotherapy (SLIT) is an emerging treatment option for allergic asthma and a potential disease-modifying strategy for asthma prevention. The key...
Sublingual allergen immunotherapy (SLIT) is an emerging treatment option for allergic asthma and a potential disease-modifying strategy for asthma prevention. The key cellular events leading to such long-term tolerance remain to be fully elucidated. We administered prophylactic SLIT in a mouse model of house dust mite (HDM)-driven allergic asthma. HDM extract was sublingually administered over 3 weeks followed by intratracheal sensitization and intranasal challenges with HDM. Prophylactic SLIT prevented allergic airway inflammation and hyperreactivity with a low lab-to-lab variation. The HDM-specific T helper (Th)2 (cluster of differentiation 4 Th) response was shifted by SLIT toward a regulatory and Th17 response in the lung and mediastinal lymph node. By using Derp1-specific cluster of differentiation 4 T cells (1-DER), we found that SLIT blocked 1-DER T cell recruitment to the mediastinal lymph node and dampened IL-4 secretion following intratracheal HDM sensitization. Sublingually administered Derp1 protein activated 1-DER T cells in the cervical lymph node via chemokine receptor7 migratory dendritic cells (DC). DCs migrating from the oral submucosa to the cervical lymph node after SLIT-induced Foxp3 regulatory T cells. When mice were sensitized with HDM, prior prophylactic SLIT increased Derp1 specific regulatory T cells (Tregs) and lowered Th2 recruitment in the lung. By using Foxp3-diphtheria toxin receptor mice, Tregs were found to contribute to the immunoregulatory prophylactic effect of SLIT on type 2 immunity. These findings in a mouse model suggest that DC-mediated functional Treg induction in oral mucosa draining lymph nodes is one of the driving mechanisms behind the disease-modifying effect of prophylactic SLIT.
PubMed: 38570140
DOI: 10.1016/j.mucimm.2024.03.012 -
International Journal of Surgery Case... Apr 2024Primary pleural hydatid cysts (PPHCs) are a rare clinical condition caused by the larval stage of the parasite Echinococcus granulosus. They occur in <1 % of all...
INTRODUCTION
Primary pleural hydatid cysts (PPHCs) are a rare clinical condition caused by the larval stage of the parasite Echinococcus granulosus. They occur in <1 % of all hydatid cysts in the body and can cause serious complications such as pneumothorax, pleural effusion, and mediastinal shift.
PRESENTATION OF CASE
We report a rare case of a 28-year-old female who was initially misdiagnosed and ignored her pneumothorax for several months, resulting in progressive dyspnea and chest pain. After performing radiological images, a primary pleural hydatid cyst was suspected. She was surgically treated and the cyst was removed by our doctors and the patient improved without any significant complications.
DISCUSSION
PPHCs are a challenging diagnosis due to their nonspecific symptoms and low prevalence. They can mimic other pleural diseases such as tuberculosis, empyema, or malignancy. The diagnosis of PPHCs requires a high index of suspicion and a combination of imaging, serology, and histopathology. The treatment of choice is surgical removal of the cyst, along with perioperative anthelmintic therapy to prevent recurrence and anaphylaxis.
CONCLUSION
PPHCs are a rare but potentially life-threatening condition that requires early diagnosis and management. Clinicians should be aware of this entity and include it in the differential diagnosis of pleural diseases, especially in developing countries. Surgical treatment is effective and safe, and can improve the quality of life of patients with PPHCs.
PubMed: 38452642
DOI: 10.1016/j.ijscr.2024.109463 -
Andes Pediatrica : Revista Chilena de... Dec 2023Pleuropulmonary blastoma (PPB) is the most common pediatric malignant primary lung tumor. It's associated with the DICER1 gene pathogenic germline variants. Antenatal...
UNLABELLED
Pleuropulmonary blastoma (PPB) is the most common pediatric malignant primary lung tumor. It's associated with the DICER1 gene pathogenic germline variants. Antenatal presentation is infrequent and poses a challenge in the differential diagnosis of congenital pulmonary airway malformation (CPAM).
OBJECTIVE
to report a case of unusual presentation of PPB associated with DICER1 syndrome and to describe the difficulty in differentiating it from CPAM.
CLINICAL CASE
Male patient with prenatal diagnosis of hypervascular left lung lesion, with mediastinal shift and progressive growth, initially interpreted as CPAM. He was born at 38 weeks, requiring transitory treatment with positive pressure due to ventilatory impairment. A CT scan with contrast showed a large multilocular cystic mass containing air causing mass effect, requiring open left upper lobectomy. Histology results were compatible with type I PPB, with negative margins, and positive genetic study for DICER1 syndrome. Seven weeks post-resection, an aerial image was detected in the upper left side of the chest, with progressive growth, requiring a new tumor resection and upper segmentectomy, with biopsy corresponding to recurrence of type I PPB with negative margins. He received adjuvant treatment with chemotherapy, with follow-up for 2 years, remaining asymptomatic, without recurrence, and with negative screening for other neoplasms associated with DICER1 syndrome. Among the family history, the mother had papillary thyroid cancer and tested positive for the mutation.
CONCLUSION
PPB is a rare cancer, difficult to distinguish from CPAM, especially in its antenatal presentation. Nowing its association with DICER1 syndrome and performing a genetic study are key to the early detection of BPP and the search for other tumors associated with the syndrome.
Topics: Female; Humans; Male; Pregnancy; DEAD-box RNA Helicases; Diagnosis, Differential; Lung; Lung Neoplasms; Pulmonary Blastoma; Ribonuclease III; Infant, Newborn
PubMed: 38329309
DOI: 10.32641/andespediatr.v94i6.4663