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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 -
Mediastinum (Hong Kong, China) 2024Locally invasive thymic neoplasms are challenging clinical scenarios and typically require a multidisciplinary approach. The involvement of major mediastinal veins such... (Review)
Review
Locally invasive thymic neoplasms are challenging clinical scenarios and typically require a multidisciplinary approach. The involvement of major mediastinal veins such as the superior vena cava (SVC) used to be a contraindication to surgery, but with improved surgical technique and outcomes, this paradigm has shifted. In some situations, complex resections and reconstructions may be indicated and required to improve the long-term outcome of these patients. We report two of our cases along with a current review of literature. We also describe the preoperative workup, operative techniques, postoperative management, complications, and outcomes of patients with invasive thymic neoplasms that involve the mediastinal veins. Our first case describes a patient who was diagnosed with a thymoma extending from the diaphragm to the base of the neck that was also encasing major vascular structures including the SVC and left innominate vein. Our second case describes a patient who was also diagnosed with a large anterior mediastinal mass encasing the great veins and invading the chest wall. We describe the management of these patients and then delve deeper into operative techniques including SVC resection and reconstruction. We describe the types of conduits that can be used and complications to be mindful of when clamping the great veins, such as the SVC. Improvements in conduit materials and neoadjuvant and adjuvant therapies over the years have made it more feasible for patients with invasive thymic neoplasms to undergo surgery.
PubMed: 38322190
DOI: 10.21037/med-20-69 -
Cancer Radiotherapie : Journal de La... Apr 2024This study aimed to assess the shifting patterns of the mediastinum, including the target volume and the isocenter point during the postoperative radiotherapy (PORT)...
PURPOSE
This study aimed to assess the shifting patterns of the mediastinum, including the target volume and the isocenter point during the postoperative radiotherapy (PORT) process of non-small cell lung cancer (NSCLC), and to observe the occurrence of radiation injury. Additionally, we investigated the significance of mid-term assessment during the implementation of the PORT process.
MATERIAL AND METHODS
We established coordinate axes based on bone anatomy and measured the mediastinum's three-dimensional direction and the shift of the isocenter point's shift in the PORT process. Statistical analysis was performed using Wilcoxon, Kruskal-Wallis, and the Chi-square test. P<0.05 was considered statistically significant.
RESULTS
In this study, the analysis of patients revealed that the shift of anterior and posterior mediastinum (X), left and right mediastinum (Y), upper and lower mediastinum (Z), anterior and posterior isocenter point (X), and the left and right isocenter points (Y) in the PORT process were 0.04-0.53, 0.00-0.84, 0.00-1.27, 0.01-0.86, and 0.00-0.66cm, respectively. The shift distance of the mediastinum was Z>Y>X, and the shift distance of the isocenter point was X>Y. According to the ROC curve, the cut-off values were 0.263, 0.352, 0.405, 0.238, and 0.258, respectively, which were more significant than the cut-off values in 25 cases (25%), 30 cases (30%), 30 cases (30%), 17 cases (17%), and 15 cases (15%). In addition, there was a significant difference in the shift of the mediastinum and the isocenter point (all P=0.00). Kruskal-Wallis test showed no statistically significant difference between mediastinal shift and resection site in X, Y, and Z directions (P=0.355, P=0.239, P=0.256), surgical method (P=0.241, P=0.110, P=0.064). There was no significant difference in the incidence of RE and RP in PORT patients (P>0.05). No III-IV RP occurred. However, the incidence of ≥ grade III RE in the modified plan cases after M-S was significantly lower than in the original PORT patients, 0% and 7%, respectively (P=0.000).
CONCLUSION
In conclusion, this study provides evidence that mediastinal shift is a potential complication during the PORT process for patients with N2 stage or R1-2 resection following radical resection of NSCLC. This shift affects about 20-30% of patients, manifesting as actual radiation damage to normal tissue and reducing the local control rate. Therefore, mid-term repositioning of the PORT and revision of the target volume and radiation therapy plan can aid in maintaining QA and QC during the treatment of NSCLC patients and may result in improved patient outcomes.
Topics: Humans; Carcinoma, Non-Small-Cell Lung; Lung Neoplasms; Quality Control; Neoplasm Staging; Retrospective Studies
PubMed: 38320903
DOI: 10.1016/j.canrad.2023.06.032 -
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 -
A Canadian children's hospital's experience with cystic echinococcosis over 30 years: A case series.Journal of the Association of Medical... Jan 2024Cystic echinococcosis (CE) or hydatid disease caused by the cestode is an uncommon infection in Canada especially among children. There are limited reports describing...
BACKGROUND
Cystic echinococcosis (CE) or hydatid disease caused by the cestode is an uncommon infection in Canada especially among children. There are limited reports describing the clinical presentation and management in Canadian children.
METHODS
The medical records of all children diagnosed with CE at a quaternary paediatric centre in Ontario between January 1988 and August 2021 were retrospectively reviewed. The clinical course, management, and outcomes of each case were summarized.
RESULTS
We report two paediatric cases of cystic echinococcosis (CE) in detail and review four additional cases seen at our institution over 33.5 years. The first case was a previously healthy 12-year-old boy with pulmonary CE resulting in unilateral lung collapse and mediastinal shift, who was presumedly infected while living in the Middle East. The second case was a previously healthy 3-year-old girl with pulmonary CE acquired locally in southern Ontario. Four other cases of CE with hepatic involvement (median age 12.5 years) were identified during the study period. Five out of six patients received both surgical and medical therapy.
CONCLUSION
CE is a rare but serious disease seen in southern Canada that has historically been associated with travel or migration. Due to changes in urban wildlife landscapes and increased global migration, CE may become more prevalent in Canadian children. We describe the first locally acquired case in rural southern Ontario diagnosed at our centre. Prompt recognition of this infection in children by health care providers is important to prevent morbidity and mortality.
PubMed: 38250618
DOI: 10.3138/jammi-2022-0041 -
Journal of Clinical Medicine Jan 2024Fetal magnetic resonance imaging (MRI) is broadly used as a method for assessing prognosis in congenital diaphragmatic hernia (CDH). In addition to the extent of lung...
OBJECTIVE
Fetal magnetic resonance imaging (MRI) is broadly used as a method for assessing prognosis in congenital diaphragmatic hernia (CDH). In addition to the extent of lung hypoplasia, determined by measuring the lung volume, cardiac impairment due to pulmonary hypertension and left cardiac hypoplasia is decisive for the prognosis. The percentage area of left ventricle (pALV) describes the percentage of the inner area of the left ventricle in relation to the total area, whereas the mediastinal shift angle (MSA) quantifies the extent of cardiac displacement. The prognostic value of pALV and MSA should be evaluated in terms of survival, the need for extracorporeal membrane oxygenation (ECMO) therapy, and the development of chronic lung disease (CLD).
METHODS
In a total of 122 fetal MRIs, the MSA and pALV were measured retrospectively and complete outcome parameters were determined regarding survival for all 122 subjects, regarding ECMO therapy in 109 cases and about the development of CLD in 78 cases. The prognostic value regarding the endpoints was evaluated using logistic regression and ROC analysis.
RESULTS
The MSA was significantly higher in children who received ECMO therapy ( = 0.0054), as well as in children who developed CLD ( = 0.0018). ROC analysis showed an AUC of 0.68 for ECMO requirement and 0.77 with respect to CLD development. The pALV showed a tendency towards higher levels in children who received ECMO therapy ( = 0.0824). The MSA and the pALV had no significant effect on survival (MSA: = 0.4293, AUC = 0.56; pALV: = 0.1134, AUC = 0.57).
CONCLUSIONS
The MSA determined in fetal MRI is a suitable prognostic parameter for ECMO requirement and CLD development in CDH patients and can possibly be used as a supplement to the established parameters.
PubMed: 38202274
DOI: 10.3390/jcm13010268 -
International Journal of Gynaecology... May 2024The main objective of the study was to determine if ultrasonographic measurement of mediastinal shift angle (MSA) can predict postnatal survival in fetuses with isolated...
OBJECTIVE
The main objective of the study was to determine if ultrasonographic measurement of mediastinal shift angle (MSA) can predict postnatal survival in fetuses with isolated left congenital diaphragmatic hernia (CDH). This relatively new technique may be used to enhance antenatal prediction of survival in fetuses with CDH.
METHODS
A retrospective cross-sectional study was conducted at the Philippine Children's Medical Center involving 16 cases with prenatally diagnosed isolated left CDH and 60 controls with normal fetuses. The cases with prenatally diagnosed isolated left CDH were allocated into two groups: Group A (survivors) and group B (non-survivors). For all fetuses (study and control groups), MSA was determined independently by two operators. The diagnostic capacity of MSA was assessed using the receiver operating characteristic curve.
RESULTS
The mean MSA for the control group was 17.18°. Among CDH cases, the mean MSA was 33.04° and 37.57° for survivors and non-survivors, respectively. Results showed that MSA significantly predicted the probability of neonatal survival status (OR = 0.46, P = 0.021). The best cutoff score of MSA based on the receiver operating characteristic curve was 33.80° with 87.50% sensitivity and specificity.
CONCLUSION
MSA has the potential to enhance antenatal prediction of survival in fetuses with CDH and may serve as a guide in prenatal counseling and evaluation of the need for in-utero treatment or postnatal procedures.
Topics: Infant, Newborn; Child; Pregnancy; Female; Humans; Hernias, Diaphragmatic, Congenital; Retrospective Studies; Cross-Sectional Studies; Ultrasonography, Prenatal; Fetus; Lung; Gestational Age
PubMed: 38189145
DOI: 10.1002/ijgo.15348 -
Cureus Dec 2023Morgagni hernia is a rare congenital defect of the diaphragm, especially seen in children but rarely observed in adults. It occurs due to a congenital defect during the...
Morgagni hernia is a rare congenital defect of the diaphragm, especially seen in children but rarely observed in adults. It occurs due to a congenital defect during the development of the diaphragm. Bochdalek hernia is a common congenital form of diaphragmatic hernia. Morgagni hernia is usually rare with a prevalence of approximately 2-3%. Beaver tail liver, which is also called sliver of liver, is a rare variant of liver morphology. Sometimes elongated left lobe of the liver can extend laterally across the midline to contact and often surround the spleen. A 46-year-old female from Karnataka complained of fever with chills and lower back aches for seven days with no history of chest pain, vomiting, or diarrhea. There have been no similar complaints in the past. She had no other comorbidities. She was a non-smoker and non-alcoholic. Biomass gas exposure for 20 years was noted as she cooked food with firewood. She was a housewife by occupation with no history of trauma or surgeries in the past. The general physical exam was unremarkable. The respiratory system was normal. Auscultation showed decreased breath sounds in the mammary area of the right side of the chest with normal vesicular breath sounds in all other areas. Per abdominal exam showed a flat abdomen. Umbilicus was central in position. There was tenderness in the right hypochondriac and epigastric regions with no guarding or rigidity. On examining the cardiovascular system, apical impulses could not be palpated and normal heart sounds were heard with no cardiac murmurs. Other systems examination was normal. Routine blood investigations were done, revealing hemoglobin of 11.6%, total WBC of 6270 cells, and hematocrit of 33.1%. The renal function test was within normal limits (creatinine = 0.7 mg/dl). A chest X-ray revealed a right lower zone, para cardiac well-circumscribed structure suggestive of a cyst with an air-fluid level inside. Chest CT was suggestive of a hernia in the anterior aspect of the diaphragm measuring 3.5 x 3.3 cm at the level of D9 vertebral body with transverse colon and omentum as its contents, ascending upwards into anterior and superior mediastinum for a length of 13 cm causing shift of cardia posteriorly and to the left (anterior midline diaphragmatic hernia - Morgagni hernia). A hyperdense lesion (Hounsfield unit = 64) measuring 1.3 x 1.8 cm was noted in segment seven of the right lobe of the liver, suggestive of a complex cyst. Beaver tail was noted in the liver. Morgagni hernia usually presents in younger age groups with respiratory symptoms. Its incidental detection in adults is very rare. In this case, the patient was having lower backache and no other gastrointestinal symptoms. The respiratory and cardiothoracic systems get affected because the intestinal contents herniating through the diaphragm shift the position of the cardia and the lower lobes of the lungs, which may have implications such as repeated cough and infections. Symptomatic hernias are usually detected in an early age group. It can present with symptoms of gastrointestinal obstruction or acute chest symptoms or can even be asymptomatic. Treatment is primarily surgical repair of the hernia. This can be done either transthoracically or transabdominally. It is usually advised that surgical repair should be done even in asymptomatic cases as in this case, to avoid obstruction of the intestine or worsening of the hernia that is pulling the abdominal contents into the thorax.
PubMed: 38161555
DOI: 10.7759/cureus.49769 -
JTCVS Techniques Dec 2023Branch-first total aortic arch repair is a paradigm shift in the technical approach for uninterrupted neuroprotection during open aortic surgery. This technique is...
BACKGROUND
Branch-first total aortic arch repair is a paradigm shift in the technical approach for uninterrupted neuroprotection during open aortic surgery. This technique is further modified to instigate hazardous sternal reentry in patients with hostile mediastinal anatomy at risk of aortic injury.
METHODS
Intraoperative preparation and the illustrated operative technique of the cervical branch-first technique are described. The accompanying case series narrates the experiences and outcomes of 4 patients who underwent successful complex reoperative aortic surgery utilizing this technique.
RESULTS
The indications for resternotomy included a sixth reoperation for recurrent mycotic aortic pseudoaneurysm, a third reoperation for extensive infective endocarditis, a reoperation for complete Bentall graft dehiscence with contained aortic rupture, and a third reoperation for residual type A dissection. All patients survived their proposed surgery. Two patients were operated on in an emergency setting. Two patients separated from cardiopulmonary bypass with extracorporeal support. None experienced permanent neurological sequelae, gut ischemia, peripheral arterial complications, or in-hospital mortality. One mortality due to decompensated heart failure was reported at 6 months postoperatively.
CONCLUSIONS
The cervical branch-first technique offers unparalleled advantage in neuroprotection from an early stage of complex reoperative aortic surgery. It provides an independent circuit for complete antegrade cerebral perfusion, irrespective of suspension to circulatory flows to the rest of the body during complex reentry into hostile chests. Our experience to date has demonstrated promising outcomes and further refinements will guide patient selection best suited for this technique.
PubMed: 38152225
DOI: 10.1016/j.xjtc.2023.08.030