The Diagnostics and Management of Bronchopulmonary Sequestration: An International Survey among Specialized Caregivers.

BACKGROUND: Our objective was to explore the treatment preferences for bronchopulmonary sequestration (BPS) among an international group of specialized caregivers. METHODS: Sixty-three participants from 17 countries completed an online survey concerning the diagnostics, treatment, and follow-up. Recruitment took place among members of the Collaborative Neonatal Network for the first European Congenital Pulmonary Airway Malformation Trial Consortium and through the Association for European Pediatric and Congenital Cardiology working group database. RESULTS: Most of the 63 participants were pediatric surgeons (52%), followed by pediatric pulmonologists (22%), and pediatric cardiologists (19%). The majority (65%) treated more than five cases per year and 52% standardly discussed treatment in a multidisciplinary team. Half of the participants (52%) based the management on the presence of symptoms, versus 32% on the intralobar or extralobar lesion localization. Centers with both surgical and interventional cardiac/radiological facilities (85%) preferred resection to embolization in symptomatic cases (62 vs. 15%). In asymptomatic cases too, resection was preferred over embolization (38 vs. 9%); 32% preferred noninterventional treatment, while 11% varied in preference. These treatment preferences were significantly different between surgeons and nonsurgeons (p < 0.05). Little agreement was observed in the preferred timing of intervention as also for the duration of follow-up. CONCLUSIONS: This survey demonstrates a variation in management strategies of BPS, reflecting different specialist expertise. Most centers treat only a handful of cases per year and follow-up is not standardized. Therefore, management discussion within a multidisciplinary team is recommended. Recording patient data in an international registry for the comparison of management strategies and outcomes could support the development of future guidelines. LEVEL OF EVIDENCE: Level IV.

Long-term neurodevelopment in children with resected congenital lung abnormalities.

To determine whether children who underwent resection of a congenital lung abnormality (CLA) are at higher risk for neurodevelopmental impairments than peers in the general population. The study population consisted of children born between 1999-2018 who underwent resection of a symptomatic CLA. Neurocognitive development (intelligence, memory, attention, visuospatial processing, executive functioning) and motor function of this population are monitored through our structured, prospective longitudinal follow-up program at the ages of 30 months, 5, 8, and 12 years. We compared study population scores with Dutch norm values using one-sample t-tests and one-sample binominal proportion tests. Forty-seven children were analyzed. The 8-year-olds showed significant impairments in sustained attention through the Dot Cancellation Test (mean z-scores -2.4; [-4.1; -0.8], p = 0.006 and -7.1; [-12.8; -1.4], p = 0.02 for execution speed and fluctuations respectively). Visuospatial memory was impaired at 8 years, though only in 1 out of 3 assessment tools (Rey Complex Figure Test z-scores (-1.0; [-1.5; -0.5], p < 0.001). Further neurocognitive outcomes were unimpaired at all tested ages. Regarding motor function outcomes, mean z-scores of total motor functioning were unimpaired across assessed ages. However, at 8 years, significantly more children than expected had definite motor problems (18% vs 5%, 95% CI [0.052; 0.403], p = 0.022).   Conclusion: This evaluation reveals impairment in some subtests of sustained attention, visuospatial memory and motor development. However, globally, normal neurodevelopmental outcomes were found throughout childhood. We recommend testing for neurodevelopmental impairments in children who underwent surgery for CLA only if associated morbidities are present or if caregivers express doubts about their daily functioning. What is Known: • In general, surgically managed CLA cases seldom suffer from long-term surgery-related morbidity and show favorable lung function. What is New: • Long-term neurocognitive and motor function outcome appear unimpaired within surgically managed CLA cases. We recommend testing for neurodevelopmental impairments in children who underwent surgery for CLA only if associated morbidities are present or if caregivers express doubts about their daily functioning.

Prediction of postnatal outcome in fetuses with congenital lung malformation: 2-year follow-up study.

OBJECTIVES: To identify, in fetuses with a congenital lung malformation (CLM), prenatal predictors of the need for postnatal respiratory support and the need for surgery by calculating the CLM volume ratio (CVR), and to evaluate the concordance between the prenatal appearance and the postnatal type of CLM. METHODS: This was an analysis of prenatal, perinatal and postnatal data from fetuses diagnosed with a CLM at the Erasmus University Medical Center - Sophia Children's Hospital in Rotterdam, The Netherlands, between January 2007 and December 2016. For all included fetuses, CVR was measured retrospectively on stored ultrasound images obtained at 18 + 1 to 24 + 6 weeks (US1), 25 + 0 to 29 + 6 weeks (US2) and/or 30 + 0 to 35 + 6 weeks' gestation (US3). Postnatal diagnosis of CLM was based on computed tomography or histology. Primary outcomes were the need for respiratory support within 24 h and surgery within 2 years after birth. RESULTS: Of the 80 fetuses with a CLM included in this study, 14 (18%) required respiratory support on the first postnatal day, and 17 (21%) required surgery within 2 years. Only the CVR at US2 was predictive of the need for respiratory support, with a cut-off value of 0.39. Four of 16 (25%) fetuses which showed full regression of the CLM prenatally required respiratory support within 24 h after birth. The CVR at US1, US2 and US3 was predictive of surgery within 2 years. Overall, the prenatal appearance of the CLM showed low concordance with the postnatal type. Prenatally suspected microcystic congenital pulmonary airway malformation (CPAM) was shown on computed tomography after birth to be congenital lobar overinflation in 15/35 (43%) cases. Respiratory support within 24 h after birth and surgical resection within 28 days after birth were needed in all cases of macrocystic CPAM. CONCLUSIONS: CVR can predict the need for respiratory support within 24 h after birth and for surgery within 2 years. Regression of a CLM prenatally does not rule out respiratory problems after birth. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. - Legal Statement: This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

[Palmar and axillary hyperhidrosis in children: endoscopic thoracic sympathectomy using clips for an underestimated problem]. / Hyperhidrosis palmaris en axillaris bij kinderen.

BACKGROUND: Palmar and axillary hyperhidrosis are defined as perspiration exceeding the body's 'normal' physiological need for thermal regulation. This condition affects about 1% of adolescents and children and may lead to psychosocial problems and poor school performance. CASE DESCRIPTION: We carried out an endoscopic thoracic sympathectomy (ETS) with clips in a 14-year-old girl and a 16-year-old girl who suffered from debilitating palmar and axillary hyperhidrosis. They were able to quickly resume their general daily activities and both were free of symptoms during an outpatient visit after 6 weeks. CONCLUSION: ETS using clips is a safe, relatively simple and effective technique, which may provide a permanent solution. ETS can be an option in case of therapy-resistant debilitating hyperhidrosis. This surgical procedure should be carefully considered, possible causes should be excluded and ETS should not be offered as the first treatment option for children.

A comparison of hemiarthroplasty with total hip replacement for displaced intracapsular fracture of the femoral neck: a randomised controlled multicentre trial in patients aged 70 years and over.

The aim of this study was to analyse the functional outcome after a displaced intracapsular fracture of the femoral neck in active patients aged over 70 years without osteoarthritis or rheumatoid arthritis of the hip, randomised to receive either a hemiarthroplasty or a total hip replacement (THR). We studied 252 patients of whom 47 (19%) were men, with a mean age of 81.1 years (70.2 to 95.6). They were randomly allocated to be treated with either a cemented hemiarthroplasty (137 patients) or cemented THR (115 patients). At one- and five-year follow-up no differences were observed in the modified Harris hip score, revision rate of the prosthesis, local and general complications, or mortality. The intra-operative blood loss was lower in the hemiarthroplasty group (7% > 500 ml), THR group (26% > 500 ml) and the duration of surgery was longer in the THR group (28% > 1.5 hours versus 12% > 1.5 hours). There were no dislocations of any bipolar hemiarthroplasty than in the eight dislocations of a THR during follow-up. Because of a higher intra-operative blood loss (p < 0.001), an increased duration of the operation (p < 0.001) and a higher number of early and late dislocations (p = 0.002), we do not recommend THR as the treatment of choice in patients aged ≥ 70 years with a fracture of the femoral neck in the absence of advanced radiological osteoarthritis or rheumatoid arthritis of the hip.

An emergency visceral hybrid procedure for ruptured thoraco-abdominal aortic aneurysms.

Rupture of a thoraco-abdominal aortic aneurysm (TAAA) is usually lethal. Patients with contained ruptures, who reach the hospital, have traditionally been subjected to open reconstructive surgery. However, especially in older patients, open surgery has a high mortality and morbidity rate. Visceral hybrid procedures (VHPs) can provide an alternative in this high-risk patient group. We present a literature review of VHPs with a focus on acute TAAAs.

Bryant traction in paediatric femoral shaft fractures, home traction versus hospitalisation.

AIM: To evaluate Bryant traction at home in terms of feasibility, morbidity, effect on length of hospital stay, outcome and costs. PATIENTS AND METHODS: Retrospective analysis of 54 children treated for femoral shaft fracture (1991-2004). Results of 'home traction' (A, n=38) and 'hospital traction' (B, n=16) were compared. Data were collected regarding demographics, length of hospital stay, traction period, various aspects of the fractures, co-morbidity, morbidity, and follow-up. The parents' experience was evaluated by telephone questionnaire. RESULTS: Two early treatment failures occurred. One patient stopped home traction at 8 days due to skin problems and had a spica cast applied at another hospital, with subsequent development of a compartment syndrome. The other patient was placed in a hip spica after 2 weeks of hospital traction because of difficulty in getting satisfactory X-rays. At follow-up, one unacceptable shortening of 3 cm had occurred in the home traction group. There was only a significant difference in hospital stay (A 7.0 days versus B 22.5 days), total traction period (A 28.0 days versus B 22.5 days) and costs (group B 3x versus group A). The parents were overall pleased with traction at home. Complications occurred as much in the home traction group as in the control group and fracture position at union was equal in both groups. CONCLUSIONS: Treatment with home traction is feasible, simple and effective; it reduces hospital stay to 1 week, and costs to one-third. Good patient selection and instructions of the parents are mandatory.

[A 26-year-old cyclist with intermittent claudication]. / Claudicatio intermittens bij een 26-jarige wielrenner.

A 26-year-old male amateur cyclist, with no risk factors for vascular disease or previous trauma, presented with left-calf claudication. Physical and additional examination revealed an occlusion of the external iliac artery. During the operation, the cause was found to be an endofibrotic lesion of the external iliac artery, probably due to mechanical trauma as a result of the non-physiological aerodynamic position held on the bicycle during many hours of training. An endarterectomy was performed and the tendon of the psoas-minor muscle was cut because of its strong impression on the psoas-major muscle, which resulted in kinking of the external iliac artery. There followed two episodes of re-occlusion which were treated with a venous interposition graft and a dacron interposition graft, respectively. Thereafter the patient was able to train without pain. Intermittent claudication of the legs in young athletes should not be underestimated; occlusive vascular disease caused by arterial endofibrosis should be considered.

Pre-operative diagnostic biopsy and surgery in paediatric liver tumours--the Amsterdam experience.

AIM: To report 24 years of pre-treatment biopsy and surgical experience in primary liver tumours in children. METHODS: Between 1979 and 2003, 53 children presented with a primary liver tumour of whom 48 who underwent surgical resection were evaluated (two died, two were unresectable, and one was transplanted). Biopsy data, per- and post-operative complications, mortality, and survival were retrospectively reviewed. RESULTS: Benign tumours were diagnosed in eight patients. Surgical resection for a malignant tumour was performed in 40 patients (26 hepatoblastomas (HB), eight hepatocellular carcinomas (HCC) (four had fibrolamellar HCC), three rhabdomyosarcomas (RMS), one neuroblastoma, one non-hodgkin lymphoma (NHL), and one teratoma). Primary resection was performed in one HB, and four HCCs. The cumulative survival without evidence of disease was 73% for HB (median 7 years) and 88% for HCC (median 3.5 years). CONCLUSION: The treatment results are comparable with those of larger international series except for HCC. The existing diagnostic pitfalls in differentiating between the various liver malignancies justify the use of a diagnostic biopsy.

Bilateral axillary metastases of occult breast carcinoma: report of a case with a review of the literature.

The case of a 47-year-old premenopausal woman is presented in whom axillary lymph node metastases occurred on both sides 3 years apart although no primary tumor was detectable in either breast is presented. An overview of the literature on this rare entity is given. Patients with occult breast carcinoma with axillary lymph node metastases should have a complete physical examination, radiologic analysis (mammography, ultrasonography, and MRI of both breasts) and screening for disseminated disease. If there is no evidence of a primary tumor and metastases other than in the axilla, an axillary dissection should be carried out. In addition, the patient should be offered the choice of irradiation of the breast or mastectomy. Postoperatively, patients should receive appropriate systemic therapy tailored to their age, menopausal status, and receptor status.

[Diagnostic image (201). A man with a swelling of a finger due to a giant cell tumor of the tendon sheath]. / Diagnose in beeld (201). Een man met een zwelling aan een vinger.

A 46-year-old man presented with a swelling of the proximal phalanx of the right index finger, due to a giant cell tumour of the tendon sheath.

[Diagnostic image (193). A man with a palpable mass in the upper abdomen. Porcelain gallbladder]. / Diagnose in beeld (193). Een man met een palpabele zwelling in de bovenbuik. Porseleinen galblaas.

In a 73-year-old man with a non-tender palpable mass in the right part of the upper abdomen and soft stool a porcelain gallbladder was observed at radiological examination.

[Femoral shaft fracture in children younger than 4 years: shorter hospital stays with the help of at home traction apparatus]. / Femurschachtfractuur bij kinderen jonger dan 4 jaar: kortere ziekenhuisopname met behulp van tractie thuis.

OBJECTIVE: Evaluation of home traction as a treatment as a treatment of femoral shaft fractures in children with the objective to shorten the hospital stay. DESIGN: Retrospective. SETTING: Paediatric Surgical Centre Amsterdam (EKZ/AMC and AZVU), the Netherlands. METHOD: In the period 1991-1995, 18 femoral shaft fractures in children younger then 4 years were treated. In ten of them traction was applied at home (in the other cases the parents refused to co-operate, the home situation was not appropriate, there were additional medical problems or there was a suspicion of child abuse). During follow-up of the group treated at home with traction, angulation, deformity and leg length discrepancy were determined with special attention to complications. The parents' experience of this method was evaluated by telephone (n = 8). RESULTS: The median age of the children was 2.4 years. The mean hospital stay was 7 days (range: 3-12), the mean follow-up 2.4 years (range: 1.0-4.3). Angulation, rotational deformities and leg length discrepancy > 1 cm did not occur. Oedema and pain were seen in 1 patient as a result of incorrect treatment at home. In one patient a compartment syndrome occurred after a switch from traction to a plaster treatment in another hospital. With exception of some small practical and informational problems, parents were very pleased with this method. CONCLUSION: Treatment at home of femoral shaft fractures in children with traction is a simple and effective method which reduces the hospital stay to one week with minimal complications. Good patient selection and instructions of the parents are mandatory.