Optimal gestational age at delivery in isolated left-sided congenital diaphragmatic hernia.

OBJECTIVE: To evaluate the morbidity and mortality of neonates with left-sided isolated congenital diaphragmatic hernia (CDH) according to gestational age at delivery. METHODS: This was a retrospective study of fetuses diagnosed prenatally with isolated left-sided CDH that were delivered in the University Hospitals of Antoine Béclère-Bicêtre and Leuven between 1 January 2010 and 31 December 2018. The Kaplan-Meier method was used to calculate cumulative survival at 28 days after birth according to gestational age at delivery. The association between gestational age at delivery, as a continuous variable, and survival at 28 days was modeled using a fractional polynomial. Adjustment for position of the liver, management center and mode of delivery was performed. The association was also evaluated according to the severity of CDH, as defined by the observed-to-expected lung-to-head ratio (o/e-LHR), which was classified as severe (o/e-LHR < 25%), moderate (o/e-LHR between 25% and 45%) or mild (o/e-LHR > 45%). RESULTS: We included 213 fetuses with isolated left-sided CDH, with a median gestational age at delivery of 38 + 2 weeks (interquartile range, 37 + 0 to 39 + 1 weeks). The survival rates at 28 days and at 6 months were 66.7% (142/213) and 64.3% (137/213), respectively. Kaplan-Meier analysis showed a higher survival rate at 28 days for babies delivered between 37 + 0 and 38 + 6 weeks than for those delivered at or after 39 + 0 weeks (log-rank test, P < 0.001). In the subgroup of moderate CDH, the 28-day survival rate was significantly higher in newborns delivered between 37 + 0 and 38 + 6 weeks than in those delivered at or after 39 + 0 weeks (81.5% vs 61.5%; P = 0.03), and this was also the case for survival rate at 6 months. In the subgroup with moderate CDH, 28-day survival significantly increased with advancing gestational age at birth up to about 38-39 weeks (P = 0.005), and significantly decreased from 39 weeks onwards. CONCLUSION: Delivery between 37 + 0 and 38 + 6 weeks' gestation is associated with a higher survival rate at 28 days in neonates with isolated left-sided CDH and moderate lung hypoplasia, independently of intrathoracic liver, management center and mode of delivery. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Invasive mucinous adenocarcinoma of the lung arising in a type 1 congenital pulmonary airway malformation in a 68-year-old patient: a case report.

INTRODUCTION: Congenital pulmonary airway malformation (CPAM), previously described as congenital cystic adenomatoid malformation (CCAM), is a congenital disorder of lung parenchyma. The association with the presence of a malignant transformation like rhabdomyosarcoma, pleuropulmonary blastoma, and most common invasive mucinous adenocarcinoma (IMA) is a rare development described in patients with CPAM. PATIENTS AND METHODS: Here, we report the case of a 68-year-old male patient who underwent a right lower lobectomy for a mass in the right pulmonary lobe. From his clinical history, we noted a recurrent pulmonary infection of a bullous malformation in the right lower lobe treated with antibiotics. RESULTS: The histopathological finding showed an invasive mucinous adenocarcinoma arising in a type 1 CPAM in the right lower lobe. A review of presentation, diagnosis, and treatment of this association is described in a case report. CONCLUSIONS: Surgical resection should be considered in adults with asymptomatic cysts to prevent malignant transformation. For further analysis, histopathological examination of specimen is essential for a proper diagnosis and eventually further postoperative treatment.

Single-Centre 20-Year Experience with Surgical Treatment of Thymic Tumours.

BACKGROUND: Large single-centre institutional series on thymic tumours are rare. Complete resection remains the mainstay of successful treatment. Characteristics and survival were reviewed in all patients treated between 19932013. METHODS: Hospital databases revealed 134 patients with pathologically-proven thymic tumour. Follow-up (median 63 months) was through patient notes and telephone contact with general practitioner. RESULTS: Patients were classified in Masaoka-Koga stages: I: 50 (37%); Ila: 14 (10%); lib: 14 (10%); III: 27 (20%); IVa: 19 (14%); IVb: 4 (3%); unknown: 6 (5%). According to WHO classification, pathological subtypes were A: 19 (14%); AB: 25 (19%); B1: 21 (16%); B2: 31 (23%); B3: 15 (11%); thymic carcinoma: 23 (17%). Parathymic syndromes were diagnosed in 45 patients: myasthenia gravis (84%); pure red-cell aplasia (4%); hypogammaglobulinemia (2%); and others. 124 patients (93%) underwent surgery with complete resection in 104 (84%). Surgical approach was: sternotomy: 79; thoracotomy: 35; cervicotomy: 2; other/unknown: 8. In 73 patients (59%) no biopsy was taken prior to surgical resection, 25 were treated with induction chemotherapy, 36 received adjuvant radiotherapy. Hospital mortality was 0.81%. 35 patients died during follow-up (13 of tumour or treatment-related causes). Overall and recurrence-free survival at 5, 10, and 15 years were 86%; 64%; 47% and 67%; 49%; and 31%, respectively and were significantly (p < 0.01) different according to Masaoka-Koga stage. There was a significant association between WHO classification and Masaoka-Koga stages I-IIa-IIb versus III-IVa-IVb (p < 0.01). CONCLUSIONS: Operability and complete resectability of thymic tumours in our experience is high resulting in prolonged overall and recurrence-free survival. Masaoka-Koga stage is an important predictor for survival and shows a significant association with WHO classification.

Single-centre 20-year experience with surgical treatment of thymic tumours.

BACKGROUND: Large single-centre institutional series on thymic tumours are rare. Complete resection remains the mainstay of successful treatment. Characteristics and survival were reviewed in all patients treated between 1993-2013. METHODS: Hospital databases revealed 134 patients with pathologically-proven thymic tumour. Follow-up (median 63 months) was through patient notes and telephone contact with general practitioner. RESULTS: Patients were classified in Masaoka-Koga stages: I: 50 (37%); IIa: 14 (10%); IIb: 14 (10%); III: 27 (20%); IVa: 19 (14%); IVb: 4 (3%); unknown: 6 (5%). According to WHO classification, pathological subtypes were A: 19 (14%); AB: 25 (19%); B1: 21 (16%); B2: 31 (23%); B3: 15 (11%); thymic carcinoma: 23 (17%). Parathymic syndromes were diagnosed in 45 patients : myasthenia gravis (84%); pure red-cell aplasia (4%); hypogammaglobulinemia (2%); and others. 124 patients (93%) underwent surgery with complete resection in 104 (84%). Surgical approach was: sternotomy: 79; thoracotomy: 35; cervicotomy: 2; other/unknown: 8. In 73 patients (59%) no biopsy was taken prior to surgical resection, 25 were treated with induction chemotherapy, 36 received adjuvant radiotherapy. Hospital mortality was 0.81%. 35 patients died during follow-up (13 of tumour or treatment-related causes). Overall and recurrence-free survival at 5, 10, and 15 years were 86%; 64%; 47% and 67%; 49%; and 31%, respectively and were significantly (p < 0.01) different according to Masaoka-Koga stage. There was a significant association between WHO classification and Masaoka-Koga stages I-IIa-IIb versus III-IVa-IVb (p < 0.01). CONCLUSIONS: Operability and complete resectability of thymic tumours in our experience is high resulting in prolonged overall and recurrence-free survival. Masaoka-Koga stage is an important predictor for survival and shows a significant association with WHO classification.

Learning curve in tracheal allotransplantation.

The first vascularized tracheal allotransplantation was performed in 2008. Immunosuppression was stopped after forearm implantation and grafting of the recipient mucosa to the internal site of the transplant. Nine months after forearm implantation, the allograft was transplanted to the tracheal defect on the radial blood vessels. Since then, four additional patients have undergone tracheal allotransplantation, three (patients 2-4) for long-segment stenosis and one (patient 5) for a low-grade chondrosarcoma. Our goal was to reduce the time between forearm implantation and orthotopic transplantation and to determine a protocol for safe withdrawal of immunosuppressive therapy. Following forearm implantation, all transplants became fully revascularized over 2 months. Withdrawal of immunosuppression began 4 months after graft implantation and was completed within 6 weeks in cases 2-4. Repopulation of the mucosal lining by recipient cells, to compensate for the necrosis of the donor mucosa, was not complete. This resulted in partial loss of the allotransplant in patients 2-4. In patient 5, additional measures promoting recipient cell repopulation were made. The trachea may be used as a composite tissue allotransplant after heterotopic revascularization in the forearm. Measures to maximize recipient cell repopulation may be important in maintaining the viability of the transplant after cessation of immunosuppression.

Cervical videomediastinoscopy.

Cervical mediastinoscopy is a frequently used technique to assess the mediastinum, in particular the mediastinal lymph nodes in patients presenting with non-small cell lung cancer (NSCLC). The senior author of this article is credited for developing in 1989 the concept of what is now called videomediastinoscopy. The introduction of videomediastinoscopy has proven to be superior to conventional mediastinoscopy and has made teaching of this operation much easier. However, imaging modalities, in particular positron emission tomography, have substantially decreased the need for mediastinoscopy in early stage NSCLC, while in more advanced stages the indication for primary staging and/or restaging after induction therapy is now challenged by the increasing experience with endobronchial ultrasound, endoesophageal ultrasound, and fine-needle aspiration. This article discusses the current deployment of videomediastinoscopy in the diagnosis and management of NSCLC.

Application of a postnatal prediction model of survival in CDH in the era of fetal therapy.

Background: The disease severity in patients with a congenital diaphragmatic hernia (CDH) is highly variable. To compare patient outcomes, set up clinical trials and come to severity-based treatment guidelines, a performant prediction tool early in neonatal life is needed.Objective: The primary purpose of this study was to validate the CDH study group (SG) prediction model for survival in neonates with CDH, including patients who had fetal therapy. Secondary, we aimed to assess its predictive value for early morbidity.Methods: This is a retrospective single-center study at the University Hospitals Leuven on all infants with a diagnosis of CDH live-born between April 2002 and December 2016. The prediction model of the CDHSG was applied to evaluate its performance in determining mortality risk. Besides, we examined its predictive value for early morbidity parameters, including duration of ventilation, respiratory support on day 30, time to full enteral feeding and length of hospital stay.Results: The CDHSG prediction model predicted survival well, with an area under the curve of 0.796 (CI: 0.720-0.871). It had poor value in predicting infants who needed respiratory support on day 30 (area under the curve (AUC) 0.606; CI: 0.493-0.719), and correlated poorly with duration of ventilation, time to full enteral feeding and length of hospital stay.Conclusion: The CDHSG prediction model was in our hands also a useful tool in predicting mortality in neonates with CDH in the fetal treatment era. Correlation with early morbidity was poor.RationaleObjectives: (1) Validation of the CDHSG prediction model for survival in a cohort of neonates with CDH, in whom fetal endoscopic tracheal occlusion was applied according to the severity of lung hypoplasia. (2) Evaluation of performance of the model in the prediction of early morbidity.Main results: (1) Confirmation of the predictive value of the model for survival in neonates with CDH in the era of fetal therapy. (2) No correlation of the model with early morbidity parameters.

Morbidity and mortality after induction chemotherapy followed by surgery in IIIa-N2 non small cell lung cancer.

OBJECTIVE: To evaluate the frequency and risk of postoperative complications and mortality in patients with IIIa-N2 non small cell lung cancer after induction chemotherapy and surgery. METHODS: In a surgical database records from ninety two patients, operated between January 1, 2000 and December 31, 2006 were reviewed. Univariate analysis was used to identify predictors of postoperative complications and in-hospital mortality. RESULTS: All cases were histologically confirmed stage IIIa-N2. All patients received preoperative platinum based chemotherapy without radiotherapy. Pneumonectomy was performed in 20 cases (23.5%), from which 9 right sided. (Bi)lobectomy was performed in 53 cases (62.4%) and sleeve lobectomy in 11 cases (17.2%). One wedge resection was performed (1.2%). In 7 cases (7.6%) only an exploration was done. Complications developed in 35 patients (38%). Major complications in 15 patients (16%). No bronchopleural fistulae were observed. Analysis identified increased age and high physiological and operative severity score for the enumeration of mortality and morbidity (POSSUM) as a risk factor to develop complications, and a high simplified comorbidity score as a risk factor to develop a major complication. Higher age, Charlson comorbidity index, simplified comorbidity score and POSSUM were a risk factor for developing pneumonia. CONCLUSION: Although surgery after induction therapy for IIIa-N2 NSCLC can be done with a morbidity and mortality comparable to surgery alone, it remains a high risk operation. It should therefore be performed in a center with experience. Bronchial stump protection should be used whenever there is an increased risk for developing a bronchopleural fistula. In deciding whether to do surgery or radiotherapy one should keep in mind the feasibility of performing a complete resection together with a preoperative assessment to predict complications and mortality. For the preoperative assessment several scoring systems can be used from which we find the simplified comorbidity score most useful.

The symptoms of varicose veins: difficult to determine and difficult to study.

OBJECTIVES: To investigate the activities which may exacerbate symptoms in patients with varicose veins. METHODS: Questionnaires sent to patients before clinics and at least six months later. RESULTS: Both questionnaires were returned by 149 of 203 patients (74%) but only 124 contained adequate data for comparison--55 from patients who had surgical treatment and 69 who had no surgery. At initial presentation, worsening of discomfort attributed to varicose veins was common during (58%) or after (48%) standing and in hot weather (44%), but less when sitting with the feet down (31%), and after (31%) or when walking (19%). Surgery significantly reduced the total number of symptoms reported by patients at follow-up (p<0.02). However, none of the symptoms reported during specific activities was significantly lessened by surgery compared with no treatment--possibly because the attrition of patients during the study resulted in small numbers for analysis. CONCLUSIONS: Symptoms are a common indication for treating varicose veins and it is therefore important to be sure that they are due to the veins, rather than other causes. This report highlights traditional and logical questions which may help to identify symptoms caused by varicose veins but illustrates the difficulty of validating them.

Pulmonary metastasis of a meningioma presenting as a solitary pulmonary nodule: 2 case reports.

Distant metastases of meningioma are rare, especially in grade 1 meningiomas. In a recent literature review, only 115 cases were found. In almost all published cases, the meningioma was treated several years before the metastasis was diagnosed. The lungs are the most frequent site of metastasis. We describe two patients treated for meningioma (one case grade 1, the other grade 3) who were referred to the Respiratory Oncology Unit because of the incidental finding of a pulmonary nodule on routine chest radiography. Both had undergone several neurosurgical procedures but the last operation was more than 7 years before in both cases. Positron emission tomography scan was suggestive of a malignant lung tumour. The lesions were surgically removed. Pathology confirmed meningioma in both cases with the same WHO grade, immunohistochemical and genetic profiles as the original meningioma. Both patients recovered well from thoracic surgery. The patient with grade 3 meningioma died three years later from intracranial recurrence. When a patient previously treated for meningioma develops a nodular lung lesion, metastasis of the meningioma should be in the differential diagnosis list. Because of the occurrence of distant metastasis even in grade I meningiomas, we suggest that the grading system should take into account genetic changes in the meningioma. Chromosome 1p and 14q losses possibly explain the aggressive behaviour of the grade 1 meningioma.

Lobar lung transplantation from deceased donors: a valid option for small-sized patients with cystic fibrosis.

BACKGROUND: Small-sized patients with cystic fibrosis usually face long waiting times for a suitable lung donor. Reduced-size lung transplantation (LTx) was promoted to shorten waiting times. We compared donor and recipient characteristics and outcome in lobar ([L]) versus full-size ([FS]) lung recipients. METHODS: Between July 1, 1991, and February 28, 2011, 535 isolated LTx were performed, including 74 in cystic fibrosis patients (8 L, 66 FS). Patients were followed up until September 2012. RESULTS: [L] recipients were younger, smaller, and lighter. Sex, waiting times, and donor data (age, sex, height, weight, PaO2/FiO2, and ventilation time) were comparable. Cardiopulmonary bypass was used more often in [L]; cold ischemia was comparable for first lung but longer in [L] for second lung; implantation times were comparable. In-hospital mortality rate was 0% in [L] versus 3% in [FS]. Both intensive care unit and hospital stay were longer in [L]. Grade 3 primary graft dysfunction was more pronounced in [L] at T0 and at T48. FEV1 increased significantly in both groups from preoperative value. Bronchiolitis obliterans syndrome was absent in [L] and diagnosed in 18 patients in [FS], accounting for 6 of 15 late deaths. All [L] are still alive. No differences in survival were found between the groups. CONCLUSIONS: Although hindered by a higher incidence of primary graft dysfunction, L-LTx is a viable option with excellent survival and pulmonary function comparable to FS-LTx.

Endoscopic thoracic sympathectomy for posttraumatic complex regional pain syndrome.

INTRODUCTION: Posttraumatic complex regional pain syndrome (CRPS) has a strongly negative impact on rehabilitation and activities of daily living. Treatment is most often unrewarding. AIM: To analyze the efficacy of endoscopic thoracic sympathectomy (ETS) in reducing pain and disability associated with CRPS prospectively. PATIENT AND METHODS: Over a 5-year period, 12 patients (7 females and 5 males; median age 46.5 [range 34-60 years]) with posttraumatic CRPS underwent unilateral ETS. The median duration of CRPS symptoms before ETS was 3.8 months (range 1.2-19.9). The sympathetic chain was resected from the 2nd to the 5th rib, and the nerve of Kuntz was severed. Median postoperative 16 months (range 12-40). Pain was assessed, at rest (passive) and during movement (active), using a visual analogue scale (VAS) from 0 to 10. RESULTS: One patient (8%) suffered a hydrothorax and 3 patients (25%) complained of contralateral compensatory hyperhydrosis. At 1 month (n = 12), 2 months (n = 7), 6 months (n = 12), and 1 year (n = 12) after ETS, there was a significant decrease in passive and active VAS (P < 0.05). Ten out of the 12 patients (83%) needed fewer analgesics after surgery, and eight (67%) did not need analgesics at all. The median sleep duration improved significantly from a preoperative value of 2 h (range 1-7) to a postoperative value of 6.25 h (range 3.5-8) (P < 0.001). Overall, patient satisfaction was 83%. CONCLUSION: ETS is effective at decreasing pain and improving quality of life, and should therefore be considered in the treatment of CRPS.

Most patients with varicose veins have fears or concerns about the future, in addition to their presenting symptoms.

INTRODUCTION: This study aimed to document fears and concerns of patients about their varicose veins. REPORT: Completed questionnaires about symptoms and concerns were brought to clinic by 62% (203/329) patients referred with uncomplicated primary varicose veins. 'Concerns, worries or fears' about their veins were reported by 79%. These were seldom mentioned in referral letters, and included fears about thrombosis (31%), trauma (16%), ulcers (15%) and general concerns about the future (57%). DISCUSSION: Many patients have unrecognised fears and concerns about their varicose veins. Specific enquiry and reassurance about these is part of good management and may avoid unnecessary treatment.

Mannan-binding lectin in children with Escherichia coli O157:H7 haemmorrhagic colitis and haemolytic uraemic syndrome.

Mannan-binding lectin (MBL) triggers complement activation upon binding to microbial surfaces. MBL deficiency has been associated with increased susceptibility to severe bacterial infections. We hypothesized that MBL deficiency may predispose children to Shiga toxin-producing Escherichia coli (STEC) O157:H7 infections and the associated haemolytic uraemic syndrome (HUS). We compared circulating levels of MBL among children with uncomplicated O157:H7 haemorrhagic colitis (HC), patients with O157:H7 HUS, normal and diseases control groups. Circulating MBL concentrations on admission were as follows: 3.22 +/- 2.43 micro g/ml among normal controls (n = 23); 2.90 +/- 2.44 micro g/ml in patients with rotavirus enteritis (n = 10); 2.78 +/- 1.65 micro g/ml in children with HC due to non-STEC bacterial pathogen (n = 15); 2.67 +/- 2.44 micro g/ml in patients with uncomplicated O157:H7 HC (n = 27); 2.80 +/- 2.97 micro g/ml in children with O157:H7 HUS (n = 15); 6.70 +/- 4.49 micro g/ml in patients with chronic renal failure unrelated to O157:H7 infection (n = 6). Higher MBL levels were found in patients with chronic renal failure compared to O157:H7 HC (P < 0.047). However, MBL concentrations <0.5 micro g/ml, which have been associated with MBL deficiency in relation to increased susceptibility to infections, were noted at comparable rates between the different groups (P = NS). Our data does not support that MBL deficiency may predispose to O157:H7 infections nor than the development of diarrhoea associated HUS.

Is the angle of needle insertion influencing the created defect in human fetal membranes? Evaluation of the agreement between specialists' opinions and ex vivo observations.

OBJECTIVE: We sought to evaluate the agreement between the opinions of specialists in fetal medicine with ex vivo observations on the potential influence of the angle of needle insertion on the fluid leak through the created defect in human fetal membranes. STUDY DESIGN: Membranes from placentas of women who were delivered by elective cesarean were harvested, cut in pieces, and secured to the bottom of plastic tubes filled with Hartmann solution. They were punctured with 18-, 20-, or 22-gauge needles, with an angle of insertion of 90 degrees (group 1) or 45 degrees (group 2), and the flow rate (in milliliters per minute) through the created defect at a constant pressure of 150 mm H(2)O was measured. Fifty physicians performing amniocentesis at fetal medicine reference centers were interviewed about their impression and clinical attitude with respect to the angle of needle insertion at the time of amniocentesis. RESULTS: In the ex vivo study, puncture with a 45 degrees angle was associated with a significantly lower flow of fluid through the membrane defect for all needle sizes tested. Regarding survey answers, 82% of physicians try to perform amniocentesis with a given angle (ie, 90 degrees in the vast majority of cases). Among the reasons for doing so, minimizing membrane damage was mentioned in more than half of cases. CONCLUSION: These results provide evidence that the angle of needle insertion influences the type of defect and therefore the flow rate through human membranes. Membrane damage is a concern of a proportion of specialists while performing amniocentesis, but ex vivo observations do not agree with clinical assumptions on the potential influence of the angle of insertion.

Fetoscopy in the pregnant rabbit at midgestation.

OBJECTIVE: To develop a small animal model for fetoscopy. METHODS: In 12 time-dated pregnant rabbits at 22 days' gestational age (term 32 days) one amniotic sac in each uterine horn (n = 24) was used for a fetoscopic procedure. After laparotomy, a 2- to 3-mm microsurgical myometrial incision was made to expose the chorionic and amniotic membrane. Under microscopic control, a 2-mm needle was inserted into the amniotic sac. Through this a 1.2-mm endoscope was passed to carry out fetoscopy during maximally 10 min, using 5-10 ml saline amnioinfusion. Mean outcome measurements were ability to visualize the placenta, umbilical cord and the different fetal elements during fetoscopy, as well as fetal survival and weight at second-look operation at 30 days. The untreated amniotic sacs served as negative controls. RESULTS: In all cases, fetoscopy could be carried out successfully, and all fetuses survived till delivery without significant influence on fetal birth weight. CONCLUSION: The midgestational rabbit can be used to perform fetoscopy.

Molecular characterization of a pediatric pheochromocytoma with suspected bilateral disease.

An 11-year-old boy with hypertension was suspected of having bilateral adrenal pheochromocytomas and hyperplasia. Molecular analysis of specific tumor suppressor genes and oncogenes excluded the familial syndromes, von Hippel-Lindau (VHL) disease and multiple endocrine neoplasia (MEN) type 2A. Further evaluation identified a unilateral adrenal pheochromocytoma with a VHL heterozygous somatic mutation (G695A) and loss of the maternal allele at 11p15.5-11p14 exclusively in the tumor tissue. Both reverse-transcriptase polymerase chain reaction and immunohistochemistry confirmed increased expression of IGF2 within the tumoral tissue, relative to a normal control adrenal gland. These results ruled out familial syndromes and suggested that the VHL mutation and the loss of maternal allele on chromosome 11 could have contributed to tumor development.