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Journal of Clinical Medicine Mar 2022Scimitar syndrome is a rare disease characterized by hypoplasia of the right lung and partial anomalous pulmonary venous drainage to the inferior vena cava. All cases...
Scimitar syndrome is a rare disease characterized by hypoplasia of the right lung and partial anomalous pulmonary venous drainage to the inferior vena cava. All cases with a prenatal diagnosis of scimitar syndrome with or without associated malformations in an 18-year period (2000-2018) in two large tertiary referral centers (University of Bonn and University of Cologne, Germany) were retrospectively reviewed for the intrauterine course and postnatal outcome. Six cases were diagnosed in the study period. All presented with hypoplasia of the right lung, right-sided mediastinal shift, and abnormal pulmonary venous drainage to the inferior vena cava. Systemic arterial blood supply to the right lung, albeit postnatally present in all cases, could not be detected prenatally. Major associated anomalies were present in all cases and included atrial septal defect ( = 5), coarctation ( = 3), diaphragmatic hernia ( = 2), and VACTERL association ( = 1). Half of the cohort died within 6 months after birth and all three survivors suffer from long-term pulmonary sequelae. The primary hint to the prenatal diagnosis of scimitar syndrome is the abnormal position of the heart in the chest. If searched for, abnormal venous drainage can be identified prenatally and confirms the diagnosis. The prognosis depends on the presence of associated major anomalies and the need for neonatal intervention.
PubMed: 35330021
DOI: 10.3390/jcm11061696 -
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 -
BMC Surgery Jul 2022Laparoscopic large hiatal hernia (LHH) repair remains a challenge despite three decades of ongoing attempts at improving surgical outcome. Its rarity and complexity,...
BACKGROUND
Laparoscopic large hiatal hernia (LHH) repair remains a challenge despite three decades of ongoing attempts at improving surgical outcome. Its rarity and complexity, coupled with suboptimal initial approach that is usually best suited for small symptomatic herniae have contributed to unacceptable higher failure rates.
RESULTS
We have therefore undertaken a systematic appraisal of LHH with a view to clear out our misunderstandings of this entity and to address dogmatic practices that may have contributed to poor outcomes.
CONCLUSIONS
First, we propose strict criteria to define nomenclature in LHH and discuss ways of subcategorising them. Next, we discuss preoperative workup strategies, paying particular attention to any relevant often atypical symptoms, indications for surgery, timing of surgery, role of surgery in the elderly and emphasizing the key role of a preoperative CT imaging in evaluating the mediastinum. Some key dissection methods are then discussed with respect to approach to the mediastinal sac, techniques to avoid/deal with pleural breach and rationale to avoid Collis gastroplasty. The issues pertaining to the repair phase are also discussed by evaluating the merits of the cruroplasty, fundoplication types and gastropexy. We end up debating the role of mesh reinforcement and assess the evidence with regards to recurrence, reoperation rate, complications, esophageal dilatation, delayed gastric emptying and mortality. Lastly, we propose a rationale for routine postoperative investigations.
Topics: Aged; Fundoplication; Gastroplasty; Hernia, Hiatal; Herniorrhaphy; Humans; Laparoscopy
PubMed: 35804332
DOI: 10.1186/s12893-022-01705-w -
Diagnostic and Interventional Radiology... Jun 2024Pediatric lung tumors are primarily discussed in the surgical literature. However, limited research has been reported on their imaging findings, and only a few tumor...
PURPOSE
Pediatric lung tumors are primarily discussed in the surgical literature. However, limited research has been reported on their imaging findings, and only a few tumor types have been documented. Therefore, the aim of this article is to describe the imaging features of primary lung tumors in children.
METHODS
The archives of the pediatric radiology unit were reviewed for primary lung tumors documented between 2007 and 2023. In total, 24 patients (9 girls and 15 boys; aged 5 months to 16 years) were included in the study. Their demographic characteristics, clinical presentation, and histopathologic results were obtained. All imaging studies were reviewed by two radiologists for various findings (e.g., lymphadenopathy, atelectasis, pleural effusion, calcification, multiplicity, pneumothorax, axial and lobar location, laterality, tumor margin, mediastinal shift, contrast enhancement pattern, signal intensity on T1- and T2-weighted images, and diffusion pattern), and a final decision was made by consensus. The mean tumor size was compared between the benign and malignant groups using a t-test.
RESULTS
There were 15 (62.5%) benign tumors, as follows: inflammatory myofibroblastic tumor (IMT; n = 10, 41%), hemangioma (n = 2, 8%), pneumocytoma (n = 2, 8%), and mature cystic teratoma (n = 1, 4%). Moreover, there were 9 (37.5%) malignant tumors, as follows: pleuropulmonary blastoma (PPB; n = 6, 25%), adenocarcinoma (n = 2, 8%), and lymphoepithelioma-like carcinoma (LELC) (n = 1, 4%). The most frequently reported symptoms were cough, fever, dyspnea, chest pain, and recurrent infection; six patients reported no clinical symptoms. Fifteen tumors (62%) were located in the right lung. The mean tumor diameter at the time of diagnosis was 6.4 ± 3 cm (benign group: 6.7 ± 3.4 cm; malignant group: 6 ± 2.3 cm, > 0.050). Calcification was present in 80% of the patients with IMT. At the time of diagnosis, two (8.3%) patients were found to have metastasis: one was diagnosed with adenocarcinoma and the other with LELC. Tumors were located peripherally in 18 (75%) patients.
CONCLUSION
The symptoms associated with lung masses are non-specific. There is no correlation between tumor size and malignancy. The most common tumors observed in this study were IMT and PPB, respectively. IMT is highly associated with calcification.
CLINICAL SIGNIFICANCE
Primary lung tumors are rarely seen in children, and they have different histopathological types. Calcification might be an important radiological clue for the diagnosis of IMT, which is the most common lung tumor in children.
PubMed: 38856321
DOI: 10.4274/dir.2024.242714 -
BMC Cancer Jan 2020To the best of our knowledge, no study has reported mediastinal shift accompanied with obstructive atelectasis due to bulky primary esophageal tumor components treated...
BACKGROUND
To the best of our knowledge, no study has reported mediastinal shift accompanied with obstructive atelectasis due to bulky primary esophageal tumor components treated with adaptive radiotherapy and concurrent chemotherapy.
CASE PRESENTATION
Here we report the case of a 65-year-old male patient diagnosed with locally advanced thoracic esophageal squamous cell cancer, clinical T4bN1M0, stage IVA. Bronchoscopy and computed tomography (CT) revealed an almost complete obstruction of the lumen of the left bronchus due to compression by bulky primary esophageal tumor components. On admission, the patient presented with dyspnea and decreased arterial oxygen saturation. Chest radiography and CT on admission revealed mediastinal shift with left atelectasis, as opposed to findings from the chest radiography performed 26 days before admission. Because of the patient's overall good condition, we recommended definitive chemoradiotherapy instead of palliative bronchial stent placement. After obtaining the patient's consent, chemoradiotherapy was initiated on the following day and it comprised three-dimensional conformal radiotherapy with 60 Gy in 30 fractions with concurrent administration of cisplatin and 5-fluorouracil. During chemoradiotherapy, tumor location was monitored with cone-beam CT and chest radiography. Chemoradiotherapy on day 8 revealed no evidence of the mediastinal shift. CT simulation was reperformed to adjust the radiotherapy fields to account for geometrical changes induced by the absence of the mediastinal shift. Subsequently, the mediastinal shift and bronchial obstruction did not recur during the course of chemoradiotherapy. The patient completed the planned radiotherapy with concurrent and adjuvant chemotherapy, and no non-hematological grade ≥ 3 adverse events were observed. Complete response was confirmed 7 months after initiating chemoradiotherapy. Currently, no disease recurrence, dysphagia, or respiratory symptoms have been reported at 13 months after initiating chemoradiotherapy.
CONCLUSIONS
In this study, a bulky primary esophageal tumor caused mediastinal shift due to ipsilateral bronchial obstruction. The close follow-up for monitoring resolution of the mediastinal shift during the course of chemoradiotherapy enabled adequate dose delivery to targets, thus reflecting the geometrical changes induced by the absence of the mediastinal shift. Adaptive radiotherapy technique was crucial for favorable patient outcomes in this challenging clinical situation.
Topics: Aged; Antineoplastic Agents; Carcinoma, Squamous Cell; Chemoradiotherapy; Chemotherapy, Adjuvant; Cisplatin; Deglutition Disorders; Esophageal Neoplasms; Fluorouracil; Humans; Male; Mediastinum; Pulmonary Atelectasis; Remission Induction
PubMed: 31906910
DOI: 10.1186/s12885-019-6505-4 -
Medicine May 2020Foreign body (FB) aspiration is a common and serious problem in childhood as it requires early recognition and treatment to avoid potentially lethal consequences. This...
Foreign body (FB) aspiration is a common and serious problem in childhood as it requires early recognition and treatment to avoid potentially lethal consequences. This study aimed to characterize the clinical and epidemiological features of airway FBs in a pediatric Chinese population.A retrospective review of medical records of children aged 0 to 14 years who attended with a diagnosis of FB aspiration the Shanghai Children's Hospital between January 2013 and December 2017 was carried out. Descriptive analysis was used to assess patient's demographics, clinical, radiographic, bronchoscopic findings, time to presentation, and characteristics of the FBs.Among the 200 patients included in the study, 92% were under 3 years of age, with a peak incidence of FB aspiration occurring between 1 and 2 years old. The male to female ratio was about 2.6:1. Twenty-three percent of the patients were admitted within 24 hours of the event, 40% within 1 week, 30% within 1 month, and 7% more than 1 month after aspiration. The most common presenting symptoms of laryngotracheal FBs were cough, dyspnea, and wheezing; those of bronchial FBs were cough, decreased air entry, and wheezing. Chest X-ray was normal in four-fifths of the children with laryngotracheal FBs, whereas most common abnormal X-ray findings in children with bronchial FBs were mediastinal shift, obstructive emphysema, and pneumonia. There was a trend that in children younger than 2 years FBs were more frequently found in the left bronchus, whereas in older children FBs were more frequently found in the right bronchus. Ninety-three percent of the removed FBs were organic materials such as food items and the most frequently aspirated FBs were peanuts. Flexible bronchoscopy was performed in 82.5% of the patients, while rigid bronchoscopy or direct laryngoscopy in 17.5% of the patients. Four patients were subjected to thoracic surgery and 1 died during rigid bronchoscopy due to acute respiratory failure.FBs is a frequent pathology among Chinese children. Tracheobronchial FBs should be strongly suspected in young children who have sudden onset of cough and wheezing episode, even when physical and radiographic evidence is absent.
Topics: Adolescent; Bronchi; Bronchoscopy; Child; Child, Preschool; China; Cross-Sectional Studies; Female; Foreign Bodies; Humans; Incidence; Infant; Infant, Newborn; Laryngoscopy; Male; Respiratory Aspiration; Retrospective Studies; Trachea
PubMed: 32481458
DOI: 10.1097/MD.0000000000020480 -
Kyobu Geka. the Japanese Journal of... Sep 2021Lymph node (LN) recurrence is frequently encountered in esophageal cancer. The prognosis of patients with LN recurrence is usually very poor due to systemic disease, but...
Lymph node (LN) recurrence is frequently encountered in esophageal cancer. The prognosis of patients with LN recurrence is usually very poor due to systemic disease, but there are some patients with relatively favorable prognosis by multidisciplinary treatment including surgery. It would be a good indication for surgical treatment that number of LN recurrence is the just one, and the region of LN recurrence is the just one as cervical, upper mediastinal or abdomen. However, recurrence of LN is likely to be part of a systemic disease and should be operated on as one of the multidisciplinary treatments. It is very important to carefully perform the procedures in the surgical treatment for LN recurrence, because the anatomical shift due to the previous surgery and the tissue fibrosis after surgery and irradiation make it difficult to grasp the positional relationship and boundaries with recurrence tumor as well as normal tissues such as nerves and vessels. Even if recurrence is found after radical resection, it is important to always consider what the surgeon can do to cure it.
Topics: Esophageal Neoplasms; Esophagectomy; Humans; Lymph Node Excision; Lymph Nodes; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Survival Rate
PubMed: 34548463
DOI: No ID Found -
Pediatric Radiology May 2024The mediastinal shift angle is a new fetal magnetic resonance imaging (MRI) index that is reportedly correlated with postnatal survival in fetuses with congenital...
BACKGROUND
The mediastinal shift angle is a new fetal magnetic resonance imaging (MRI) index that is reportedly correlated with postnatal survival in fetuses with congenital diaphragmatic hernia. However, its correlation in patients with congenital pulmonary airway malformation (CPAM) has not been assessed.
OBJECTIVE
This study aimed to establish a normal range for the right/left mediastinal shift angles, to evaluate the mediastinal shift angle in fetuses with CPAM, to compare the mediastinal shift angle with the CPAM volume ratio, and to evaluate the predictive value of the mediastinal shift angle measurements.
MATERIALS AND METHODS
To establish the normal range, we measured the mediastinal shift angle bilaterally in 124 fetuses without any lung abnormality (the control group). Subsequently, the mediastinal shift angle was measured in 32 fetuses pathologically diagnosed with CPAM. Moreover, the mediastinal shift angle and CPAM volume ratio were compared using fetal MRI.
RESULTS
The mean values for the right/left mediastinal shift angles were 18.6°/26.3° and 39.2°/35.9° for control fetuses and fetuses with CPAM, respectively. The mediastinal shift angle and the CPAM volume ratio showed a positive statistical correlation. The area under the curve demonstrated high discriminatory accuracy for the mediastinal shift angle (0.76).
CONCLUSION
The mediastinal shift angle has potential to replace the CPAM volume ratio for evaluating the severity of CPAM in fetal MRI.
Topics: Humans; Female; Magnetic Resonance Imaging; Prenatal Diagnosis; Pregnancy; Mediastinum; Lung; Cystic Adenomatoid Malformation of Lung, Congenital; Reference Values; Retrospective Studies
PubMed: 38285191
DOI: 10.1007/s00247-024-05852-5 -
Journal of Education & Teaching in... Jan 2021This case demonstrates the importance of quickly identifying a pediatric patient in acute respiratory distress secondary to pneumothorax and highlights the emergent...
UNLABELLED
This case demonstrates the importance of quickly identifying a pediatric patient in acute respiratory distress secondary to pneumothorax and highlights the emergent management of these patients. The 16-year-old male patient presented to the emergency department (ED) with a chief complaint of right-sided rib pain and shortness of breath that began acutely after an all-terrain vehicle (ATV) accident. The patient was in respiratory distress upon presentation and had diminished right-sided breath sounds. A portable chest X-ray demonstrated pneumothorax with significant mediastinal leftward shift. Needle decompression of the right chest wall was performed and right sided thoracostomy was placed. It is important to maintain a high index of suspicion for tension pneumothorax in pediatric trauma patients with respiratory distress, even when hemodynamically stable. This case report discusses proper management of traumatic tension pneumothorax and discusses current recommendations for needle decompression and thoracostomy.
TOPICS
Tension pneumothorax, pediatrics, respiratory distress, portable chest x-ray, ultrasound.
PubMed: 37465541
DOI: 10.21980/J8ZD1S -
Radiographics : a Review Publication of... May 2024Thymic imaging is challenging because the imaging appearance of a variety of benign and malignant thymic conditions are similar. CT is the most commonly used modality...
Thymic imaging is challenging because the imaging appearance of a variety of benign and malignant thymic conditions are similar. CT is the most commonly used modality for mediastinal imaging, while MRI and fluorine 18 fluorodeoxyglucose (FDG) PET/CT are helpful when they are tailored to the correct indication. Each of these imaging modalities has limitations and technical pitfalls that may lead to an incorrect diagnosis and mismanagement. CT may not be sufficient for the characterization of cystic thymic processes and differentiation between thymic hyperplasia and thymic tumors. MRI can be used to overcome these limitations but is subject to other potential pitfalls such as an equivocal decrease in signal intensity at chemical shift imaging, size limitations, unusual signal intensity for cysts, subtraction artifacts, pseudonodularity on T2-weighted MR images, early imaging misinterpretation, flow and spatial resolution issues hampering assessment of local invasion, and the overlap of apparent diffusion coefficients between malignant and benign thymic entities. FDG PET/CT is not routinely indicated due to some overlap in FDG uptake between thymomas and benign thymic processes. However, it is useful for staging and follow-up of aggressive tumors (eg, thymic carcinoma), particularly for detection of occult metastatic disease. Pitfalls in imaging after treatment of thymic malignancies relate to technical challenges such as postthymectomy sternotomy streak metal artifacts, differentiation of postsurgical thymic bed changes from tumor recurrence, or human error with typical "blind spots" for identification of metastatic disease. Understanding these pitfalls enables appropriate selection of imaging modalities, improves diagnostic accuracy, and guides patient treatment. RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.
Topics: Humans; Fluorodeoxyglucose F18; Positron Emission Tomography Computed Tomography; Neoplasm Recurrence, Local; Thymus Neoplasms; Thymoma; Positron-Emission Tomography; Magnetic Resonance Imaging; Radiopharmaceuticals
PubMed: 38602866
DOI: 10.1148/rg.230091