-
Life (Basel, Switzerland) Dec 2023Unilateral proximal interruption of the pulmonary artery (UPIPA) is a rare congenital disease, and its optimal management remains undefined in the existing literature.... (Review)
Review
BACKGROUND
Unilateral proximal interruption of the pulmonary artery (UPIPA) is a rare congenital disease, and its optimal management remains undefined in the existing literature. The occasional necessity for pneumonectomy is still supported by limited evidence.
METHODS
A systematic review of the literature was conducted using the PubMed search engine, focusing on UPIPA cases that received pneumonectomy. Thirty-one pertinent articles were selected and included in the analysis. A case reported from our institution was included in the analysis.
RESULTS
We found 25 adults and seven children affected by UPIPA who received an indication for pneumonectomy, plus an additional case that was reported by our institution. Among adult patients, the predominant indication was hemoptysis (57%), followed by suspected or confirmed lung cancer (23%). Approximately 46% of surgical procedures were classified as urgent or emergent. Postoperative complications were observed in 36% of cases, with no recorded mortality. In pediatric cases, pneumonectomy was primarily a life-saving intervention, performed urgently or emergently in 75% of instances. A possible late complication in pediatric patients involves a mediastinal shift leading to respiratory distress, which may be mitigated using an inflatable prosthesis.
CONCLUSIONS
Pneumonectomy achieves complete resolution of UPIPA symptoms. In the adult population, its primary indication is hemoptysis, with procedures conducted in both elective and urgent/emergent settings. Despite a mortality rate of zero, a notable proportion of patients may experience postoperative complications. In pediatric cases, the clinical presentation varies more extensively, and pneumonectomy is typically reserved for life-threatening situations, emphasizing the need for careful patient selection.
PubMed: 38137929
DOI: 10.3390/life13122328 -
Medicine Dec 2023Paraesophageal hernias, accounting for a mere 5% to 10% of all hiatal hernias, occasionally present an exceedingly uncommon yet gravely consequential complication...
RATIONALE
Paraesophageal hernias, accounting for a mere 5% to 10% of all hiatal hernias, occasionally present an exceedingly uncommon yet gravely consequential complication characterized by the inversion of the stomach. Delving into the clinical manifestations and optimal therapeutic approaches for patients afflicted by this condition merits substantial exploration.
PATIENT CONCERNS
A 60-year-old man was referred to our hospital with acute onset of severe epigastric pain, abdominal distension, and vomiting. A chest radiograph unveiled an elevated left diaphragmatic dome accompanied by a pronounced rightward shift of the mediastinum. Subsequent abdominal computed tomography imaging delineated the migration of the stomach, spleen, and colon into the left hemithorax, facilitated by a significant diaphragmatic defect.
DIAGNOSES
The diagnosis of a giant paraesophageal hernia with complete gastric inversion was established through a comprehensive evaluation of the patient's clinical manifestations and imaging findings.
INTERVENTIONS
Surgical intervention was performed on the patient. During the procedure, a left diaphragmatic defect measuring approximately 10 × 8 cm was identified and meticulously repositioned, followed by the repair of the diaphragmatic hernia. The herniated contents comprised the pancreas, stomach, spleen, a segment of the colon, and a portion of the greater omentum.
OUTCOMES
The patient experienced a smooth postoperative recuperation and was discharged 12 days following the surgical procedure. Subsequently, during a 7-month follow-up period, the patient continued to exhibit favorable progress and recovery.
LESSONS
Paraesophageal hernias are rare, and the presence of an inverted stomach in a giant paraesophageal hernia is exceptionally uncommon. Clinical presentation lacks distinct features and can lead to misdiagnosis. This case emphasizes the importance of timely surgical intervention guided by imaging, offering valuable clinical insights.
Topics: Male; Humans; Middle Aged; Hernia, Hiatal; Stomach; Hernias, Diaphragmatic, Congenital; Diaphragm; Abdominal Pain
PubMed: 38134072
DOI: 10.1097/MD.0000000000036734 -
Pneumologie (Stuttgart, Germany) Jun 2024A 24-year-old male patient, without further symptoms or comorbidities presented to the emergency room with acute dyspnea after heavy lifting two days before. On...
A 24-year-old male patient, without further symptoms or comorbidities presented to the emergency room with acute dyspnea after heavy lifting two days before. On auscultation an attenuated vesicular breath was noticed on the right lung. In the initial chest radiograph a right-sided primary spontaneous pneumothorax with minor mediastinal shift was diagnosed. After insertion of a 12-French chest tube the patient's clinical condition deteriorated. The following chest radiograph and computed tomography of the thorax showed a reexpansion pulmonary edema in the right lung. The patient was admitted to the ICU and supportive treatment was initiated. Pulmonary reexpansion edema after drainage of a pneumothorax is a very rare complication with mortality rates reaching up to 20%. The exact pathophysiology remains unknown. Typical Symptoms include dyspnea, hypotension, and tachycardia. To minimize the risk of a pulmonary reexpansion edema, not more than 1200-1800 ml of air should be drained at once and the drainage should be stopped when the patient starts coughing.
Topics: Humans; Male; Pneumothorax; Pulmonary Edema; Young Adult; Drainage; Chest Tubes; Treatment Outcome
PubMed: 38096911
DOI: 10.1055/a-2216-0404 -
The Journal of Surgical Research Mar 2024Congenital lung malformations (CLMs) have a variable natural history. Larger lesions with CLM volume ratio (CVR) ≥ 1.6 are associated with hydrops and fetal...
INTRODUCTION
Congenital lung malformations (CLMs) have a variable natural history. Larger lesions with CLM volume ratio (CVR) ≥ 1.6 are associated with hydrops and fetal mortality. The purpose of this study is to describe the management and outcomes of high-risk (CVR ≥ 1.6) CLM patients.
METHODS
A retrospective cohort study was performed for all fetuses evaluated between May 2015 and May 2022. Demographics, prenatal imaging factors, prenatal and postnatal treatment, and outcomes were collected. Descriptive statistics were used to compare the cohorts.
RESULTS
Of 149 fetal CLM patients referred to our fetal center, 21/149 (14%) had CVR ≥ 1.6. One CLM patient had intrauterine fetal demise, and 2 patients were lost to follow-up. Of the remaining 18 patients, 11/18 (67%) received maternal steroids. Seven out of 18 patients (39%) underwent resection at the time of delivery with 1/7 (14%) undergoing exutero intrapartum treatment (EXIT)-to-resection, 5/7 (71%) undergoing EXIT-to-exteriorization-to-resection, and 1/7 (14%) undergoing a coordinated delivery to resection; among those undergoing resection, there were 2 fatalities (28.5%). Seven out of 18 (39%) patients required urgent neonatal open lobectomies, and the remaining 4/18 (22%) patients underwent elective thoracoscopic lobectomies with no mortality.
CONCLUSIONS
The natural history and outcomes of severe CLM patients remain highly variable. The EXIT-to-exteriorization-to-resection procedure may be a safe and effective approach for a subset of CLM patients with persistent symptoms of mass effect and severe mediastinal shift due to the observed decreased operative time requiring placental support observed in our study.
Topics: Infant, Newborn; Humans; Female; Pregnancy; Retrospective Studies; Placenta; Respiratory System Abnormalities; Lung Diseases; Lung; Ultrasonography, Prenatal
PubMed: 38086256
DOI: 10.1016/j.jss.2023.11.048 -
Cureus Nov 2023We present a case of a previously fit and well 28-year-old male who presented to the emergency department with respiratory distress and hypoxia four days after an...
We present a case of a previously fit and well 28-year-old male who presented to the emergency department with respiratory distress and hypoxia four days after an alleged assault and blunt-force trauma to the chest wall. Initial clinical assessment and imaging suggested a likely diagnosis of delayed massive hemothorax associated with mediastinal shift. However, upon chest drain insertion, a large amount of pus was unexpectedly drained, leading to an immediate improvement in symptoms and restoration of mediastinal anatomy on repeat imaging. Our case illustrates that, although rare, empyemas can reach a significant volume before detection; they are capable of producing radiological and clinical intrathoracic tension configuration and can mimic hemothorax in the setting of trauma.
PubMed: 38084179
DOI: 10.7759/cureus.48617 -
Journal of Pediatric Surgery May 2024Open fetal resection for large lung lesions has virtually been replaced by maternal steroid administration. Despite this paradigm shift, little is known about the...
BACKGROUND
Open fetal resection for large lung lesions has virtually been replaced by maternal steroid administration. Despite this paradigm shift, little is known about the effects steroids have on lung lesion growth in utero.
METHODS
A 10-year retrospective review of all prenatally diagnosed lung lesions cared for at our fetal care center was performed. We evaluated the effects of prenatal steroids on congenital pulmonary airway malformation (CPAM)-volume-ratio (CVR), distinguishing change in CVR among CPAMs, bronchopulmonary sequestrations (BPS), and bronchial atresias. We also correlated fetal ultrasound and MRI findings with pathology to determine the accuracy of prenatal diagnosis.
RESULTS
We evaluated 199 fetuses with a prenatal lung lesion. Fifty-four (27 %) were treated with prenatal steroids with a subsequent 21 % mean reduction in the CVR (2.1 ± 1.4 to 1.1 ± 0.4, p = 0.003). Fetuses with hydrops and mediastinal shift who were treated with steroids rarely had resolution of these radiographic findings. Postnatal pathology was available for 91/199 patients (45.7 %). The most common diagnosis was CPAM (42/91, 46 %), followed by BPS (30/91, 33 %), and bronchial atresia (14/91, 15 %). Fetuses who received steroids and had pathology consistent with CPAM were more likely to have a reduction in their CVR (p = 0.02). Fetal ultrasound correctly diagnosed the type of lung lesion in 75 % of cases and fetal MRI in 81 % of cases.
CONCLUSIONS
Prenatally diagnosed CPAMs are more likely to respond to maternal steroids than BPS or bronchial atresias. Knowing the diagnosis in utero could aid to steward steroid usage, however, fetal imagining modalities are not perfect in distinguishing subtype.
LEVEL OF EVIDENCE
III.
Topics: Pregnancy; Female; Humans; Cystic Adenomatoid Malformation of Lung, Congenital; Respiratory System Abnormalities; Bronchopulmonary Sequestration; Ultrasonography, Prenatal; Retrospective Studies; Steroids; Lung
PubMed: 38042733
DOI: 10.1016/j.jpedsurg.2023.11.004 -
EJHaem Nov 2023Lymphoid cancers are among the most frequent cancers diagnosed in adolescents and young adults (AYA), ranging from approximately 30%-35% of cancer diagnoses in... (Review)
Review
Lymphoid cancers are among the most frequent cancers diagnosed in adolescents and young adults (AYA), ranging from approximately 30%-35% of cancer diagnoses in adolescent patients (age 10-19) to approximately 10% in patients aged 30-39 years. Moreover, the specific distribution of lymphoid cancer types varies by age with substantial shifts in the subtype distributions between pediatric, AYA, adult, and older adult patients. Currently, biology studies specific to AYA lymphomas are rare and therefore insight into age-related pathogenesis is incomplete. This review focuses on the paradigmatic epidemiology and pathogenesis of select lymphomas, occurring in the AYA patient population. With the example of posttransplant lymphoproliferative disorders, nodular lymphocyte-predominant Hodgkin lymphoma, follicular lymphoma (incl. pediatric-type follicular lymphoma), and mediastinal lymphomas (incl. classic Hodgkin lymphoma, primary mediastinal large B cell lymphoma and mediastinal gray zone lymphoma), we here illustrate the current state-of-the-art in lymphoma classification, recent molecular insights including genomics, and translational opportunities. To improve outcome and quality of life, international collaboration in consortia dedicated to AYA lymphoma is needed to overcome challenges related to siloed biospecimens and data collections as well as to develop studies designed specifically for this unique population.
PubMed: 38024596
DOI: 10.1002/jha2.785 -
World Journal of Gastrointestinal... Oct 2023Minimally invasive surgery is increasingly indicated in the management of malignant disease. Although oesophagectomy is a difficult operation, with a long learning... (Review)
Review
Minimally invasive surgery is increasingly indicated in the management of malignant disease. Although oesophagectomy is a difficult operation, with a long learning curve, there is actually a shift towards the laparoscopic/thoracoscopic/ robotic approach, due to the advantages of visualization, surgeon comfort (robotic surgery) and the possibility of the whole team to see the operation as well as and the operating surgeon. Although currently there are still many controversial topics, about the surgical treatment of patients with gastro-oesophageal junction (GOJ) adenocarcinoma, such as the type of open or minimally invasive surgical approach, the type of oesophago-gastric resection, the type of lymph node dissection and others, the minimally invasive approach has proven to be a way to reduce postoperative complications of resection, especially by decreasing pulmonary complications. The implementation of new technologies allowed the widening of the range of indications for this type of surgical approach. The short-term and long-term results, as well as the benefits for the patient - reduced surgical trauma, quick and easy recovery - offer this type of surgical treatment the premises for future development. This article reviews the updates and perspectives on the minimally invasive approach for GOJ adenocarcinoma.
PubMed: 37969407
DOI: 10.4251/wjgo.v15.i10.1675 -
BMJ Case Reports Oct 2023Platypnoea-orthodeoxia syndrome is characterised by dyspnoea and oxygen desaturation in the upright position usually caused by an extracardiac shunt and less often due...
Platypnoea-orthodeoxia syndrome is characterised by dyspnoea and oxygen desaturation in the upright position usually caused by an extracardiac shunt and less often due to dynamic factors that accentuate an intracardiac right-to-left shunt. In our patient, the collapse of lower lobe of left lung secondary to bronchial stenosis due to endobronchial tuberculosis and resultant mediastinal shift was the factor that led to an otherwise unrecognised intracardiac right-to-left shunt leading to platypnoea-orthodeoxia. We postulate that there would have been an increased stretching and widening of a patent foramen ovale in the upright position due to gravity resulting in an increased shunt despite normal intracardiac pressures. Once the patency of the left main bronchus was restored by deploying a stent, the left lower lobe expanded, the mediastinum returned to its normal position and there was resultant resolution of the platypnoea-orthodeoxia. This interesting observation may be useful in managing similar scenarios in the future.
Topics: Humans; Platypnea Orthodeoxia Syndrome; Hypoxia; Foramen Ovale, Patent; Dyspnea
PubMed: 37907322
DOI: 10.1136/bcr-2023-255587 -
World Journal of Clinical Cases Sep 2023Diaphragmatic hernia (DH) is extremely rarely described during pregnancy. Due to the rarity, there is no diagnostic or treatment algorithm for DH in pregnancy.
BACKGROUND
Diaphragmatic hernia (DH) is extremely rarely described during pregnancy. Due to the rarity, there is no diagnostic or treatment algorithm for DH in pregnancy.
AIM
To summarize and define the most appropriate diagnostic methods and therapeutic options for DH in pregnancy based on scarce literature.
METHODS
Literature search of English-, German-, Spanish-, and Italian-language articles were performed using PubMed (1946-2021), PubMed Central (1900-2021), and Google Scholar. The PRISMA protocol was followed. The search terms included: Maternal diaphragmatic hernia, congenital hernia, pregnancy, cardiovascular collapse, mediastinal shift, abdominal pain in pregnancy, hyperemesis, diaphragmatic rupture during labor, puerperium, hernie diaphragmatique maternelle, hernia diafragmática congenital. Additional studies were identified by reviewing reference lists of retrieved studies. Demographic, imaging, surgical, and obstetric data were obtained.
RESULTS
One hundred and fifty-eight cases were collected. The average maternal age increased across observed periods. The proportion of congenital hernias increased, while the other types appeared stationary. Most DHs were left-sided (83.8%). The median number of herniated organs declined across observed periods. A working diagnosis was correct in 50%. DH type did not correlate to maternal or neonatal outcomes. Laparoscopic access increased while thoracotomy varied across periods. Presentation of less than 3 days carried a significant risk of strangulation in pregnancy.
CONCLUSION
The clinical presentation of DH is easily confused with common chest conditions, delaying the diagnosis, and increasing maternal and fetal mortality. Symptomatic DH should be included in the differential diagnosis of pregnant women with abdominal pain associated with dyspnea and chest pain, especially when followed by collapse. Early diagnosis and immediate intervention lead to excellent maternal and fetal outcomes. A proposed algorithm helps manage pregnant women with maternal DH. Strangulated DH requires an emergent operation, while delivery should be based on obstetric indications.
PubMed: 37900237
DOI: 10.12998/wjcc.v11.i27.6440