Outcome of prenatally diagnosed congenital lung anomalies in the North of England: a review of 228 cases to aid in prenatal counselling.

OBJECTIVE: To describe data on congenital lung anomalies identified on prenatal ultrasound from two centres in the North of England. METHOD: This retrospective case series includes all cases notified to the Northern Congenital Abnormality Survey from 1990 to 2010 and to Leeds Regional Fetal Medicine Unit 2000-2015. RESULTS: There were a total of 228 cases, 101 from Northern Congenital Abnormality Survey and 127 from Leeds Regional Fetal Medicine Unit. Eight-five per cent were unilateral congenital pulmonary airway malformations (CPAMs), 2% bilateral CPAMs and 11% bronchopulmonary sequestrations. Mediastinal shift was present in 50% of cases, occurring more frequently in macrocystic CPAMs and larger lesions. Polyhydramnios was evident in 28%, and fetal hydrops occurred in 9%. Prenatal regression occurred in 54%, and lesions were no longer visible on ultrasound in 27% at a later gestation. Prenatal intervention was performed in 5% of cases, and postnatal surgical intervention was required in 12% due to ongoing respiratory symptoms. Regression of fetal hydrops occurred in five cases. CONCLUSION: The outcome was favourable in 83% cases. Prenatal regression was common, and macrocystic lesions were less likely to regress than microcystic lesions. No specific prenatal features predicted the need for early surgical intervention. The data analysed in this prenatal series will help when counselling prenatal cases. © 2017 John Wiley & Sons, Ltd.

Renal lymphangiomatosis, interrupted IVC with persistent primitive hepatic venous plexus and multiple anomalous venous channels: parts of an overlap syndrome?

A 5-year-old girl with cutis marmorata telangiectasia congenita (CMTC) and congenital glaucoma, who had previously presented with seizures, transient hemiplegia, upper gastrointestinal bleeding and hemihypertrophy, developed a large pleural effusion. Subsequent imaging revealed renal lymphangiomatosis, multiple anomalous intra-abdominal venous channels, an interrupted inferior vena cava with a persistent primitive hepatic venous plexus (PPHVP) and meningeal angiomas. To the best of our knowledge, the CT findings of PPHVP and the combination of the demonstrated abnormalities have not been previously reported. They may represent an overlap syndrome of CMTC, Sturge-Weber syndrome and Klippel-Trenaunay syndrome. The complexity and degree of overlap highlights the importance of an accurate clinical and anatomical description and good communication among clinicians.

Extubation strategies after esophageal atresia repair.

BACKGROUND/PURPOSE: Early extubation following repair of esophageal atresia (EA) is desirable unless the anastomosis is under tension, in which case paralysis and post-operative ventilation may reduce the risk of anastomotic leakage. However, complications from emergency reintubations do occur with either strategy. We aim to examine the risk/benefit balance of early and delayed extubation following EA repair. METHODS: A seven-year retrospective review of all babies that underwent EA repair was performed. Babies extubated within 24 h of surgery were classified as early extubation (EE). Babies intubated beyond the first 24 h were classified as delayed extubation (DE). The EE group was subdivided into babies extubated in operating room (EIOR), and babies who returned to the neonatal intensive care unit (NICU) intubated but extubated within 24 h (EW24). RESULTS: Forty-six babies were analyzed, and overall 15 (32.6%) required 24 reintubation episodes. Eight (28.6%) babies in the EE group required reintubation. The EIOR group (n = 12) had significantly increased risk of requiring reintubation (OR:7, 95%CI:1.08 to 45.16:p = 0.04) compared to the EW24 group (n = 16). Seven (38.9%) babies in the DE group required reintubation. The complication rate from reintubation after EA repair was 17%. CONCLUSIONS: Extubation on the NICU within 24 h of surgery carried the lowest risk of reintubation. For babies with a tight anastomosis, elective postoperative ventilation appeared to confer a protective benefit without incurring a high risk of complications from reintubation.

Prenatally diagnosed congenital lung malformations-A long-term outcome study.

AIM: To report the natural history of a cohort of children with prenatally diagnosed congenital lung malformations (CLM) which we set out to manage expectantly. METHODS: Retrospective review of children born between 1995 and 2013 with a CLM identified on prenatal ultrasound. Prenatal ultrasound data were analyzed along with patient medical records, radiology, and pathology. RESULTS: One hundred fifty-nine newborns with a fetal lung lesion were identified during the study period. All infants were live born. Twenty-eight (18%) newborns were symptomatic, 17 with transient symptoms, and 11 with persistent symptoms. The latter all underwent lobectomy in the neonatal period with two postoperative deaths. One hundred thirty-one asymptomatic newborns plus the 17 babies with transient symptoms (148 total) were followed during childhood for a median of 6.0 years (0.1-19.2 years). Twenty-one children (13% of the original cohort of 159) became symptomatic at a median age of 2.5 years (9 months-5 years 8 months) with infection and underwent CLM resection. No child became symptomatic after the age of 5 years 8 months. One hundred twenty-seven children remained symptom free during follow-up for a median of 5.75 years (1 month-19 years). We saw no instance of malignancy in the resected specimens. CONCLUSIONS: This study adds further evidence that most children born with CLM identified prenatally are asymptomatic at birth and the majority will remain asymptomatic during childhood. We recommend follow-up to the age of 10 years.

Tracheal Replacement Therapy with a Stem Cell-Seeded Graft: Lessons from Compassionate Use Application of a GMP-Compliant Tissue-Engineered Medicine.

Tracheal replacement for the treatment of end-stage airway disease remains an elusive goal. The use of tissue-engineered tracheae in compassionate use cases suggests that such an approach is a viable option. Here, a stem cell-seeded, decellularized tissue-engineered tracheal graft was used on a compassionate basis for a girl with critical tracheal stenosis after conventional reconstructive techniques failed. The graft represents the first cell-seeded tracheal graft manufactured to full good manufacturing practice (GMP) standards. We report important preclinical and clinical data from the case, which ended in the death of the recipient. Early results were encouraging, but an acute event, hypothesized to be an intrathoracic bleed, caused sudden airway obstruction 3 weeks post-transplantation, resulting in her death. We detail the clinical events and identify areas of priority to improve future grafts. In particular, we advocate the use of stents during the first few months post-implantation. The negative outcome of this case highlights the inherent difficulties in clinical translation where preclinical in vivo models cannot replicate complex clinical scenarios that are encountered. The practical difficulties in delivering GMP grafts underscore the need to refine protocols for phase I clinical trials. Stem Cells Translational Medicine 2017;6:1458-1464.

The great debate: open or thoracoscopic repair for oesophageal atresia or diaphragmatic hernia.

Controversy exists over the best method and technique of repair of oesophageal atresia and diaphragmatic hernia. Open surgical repairs have a long established history of over 60 years of experience. Set against this has been a series of successful thoracoscopic repairs of both congenital anomalies reported over the past decade. This review was based upon a four-handed debate on the merits and weaknesses of the two contrasting surgical philosophies and reviews existing literature, techniques, complications, and importantly outcome and results.

Percutaneous endoscopic gastrostomy. Procedure in practice.

Percutaneous endoscopic gastrostomy (PEG) is a relatively common procedure used in the treatment of children. As with all procedures, it must be used with care and only after taking careful account of circumstances and likely responses of the individual patient. This first of two articles on the procedure discusses the indications and contraindications for PEG insertion in children and then focuses on the procedure itself. The second article will discuss the role of the nurse as surgeon's assistant in the application of this procedure to children.

Nurse-assisted PEG in children.

The procedures reviewed in this article comprised 83 PEG insertions and 39 PEG changes in children aged six weeks to 19 years (median three years) with weights between 4.7 kg and 80 kg (median 13.5 kg). The complication rate was lower than previously reported paediatric series performed exclusively by medical practitioners. Four children required open conversion and one gastro-colic fistula was discovered five months later.

Vascular ring diagnosis following respiratory arrest.

Vascular rings can present with non-specific respiratory and/or oesophageal symptoms. Early diagnosis requires a high index of suspicion. This case report describes an uncommon acute presentation of a vascular ring. We report a thriving 14-month-old child with a long history of recurrent wheeze and 'noisy breathing'. He presented acutely with food bolus impaction in the oesophagus which led to a respiratory arrest. Oesophagoscopy and bronchoscopy suggested vascular ring anomaly. A contrast-enhanced CT scan demonstrated a right-sided aortic arch with left ligamentum arteriosum encircling the oesophagus and airway. The ligament was ligated and divided. At follow-up 6 months later, the infant had mild persistent stridor but was otherwise well.

Congenital cytomegalovirus infection associated with development of neonatal emphysematous lung disease.

A 26-week-gestation infant developed cystic lung changes which required lobar resection at 6 weeks of age. Lung histology showed cytomegalovirus (CMV) inclusion bodies. The authors present the radiology and histology images of this case and review the literature regarding congenital CMV infection and cystic lung disease. Lung disease caused by CMV is typically a diffuse pneumonitis. This is the first reported case of congenital CMV infection causing emphysematous lung disease to develop in the neonatal period. The case raises awareness of CMV as a possible cause of cystic lung lesions in newborns.

A simulation to evaluate screening for Helicobacter pylori infection in the prevention of peptic ulcers and gastric cancers.

A discrete event simulation model has evaluated a screening programme for Helicobacter pylori infection (H. pylori) in which individuals under the age of 50 years would be screened once. Eradication of H. pylori would reduce the peptic ulcer risk immediately and the gastric cancer risk after a fixed delay. The data were derived from published databases and peer reviewed papers. The simulation model, using variance reduction techniques, predicted that a screening programme would reduce morbidity and deaths but could cost around 19 million pounds for England and Wales in the first year of screening. A factorial design analysis showed the sensitivity of key variables. An increase in the opportunistic testing was found significantly to reduce the impact of screening.

A prospective study of neonatal inguinal herniotomy: the problem of the postoperative hydrocele.

Previous studies have reported an increased incidence of complications following neonatal inguinal herniotomy (IH) in boys. The incidence and natural history of postoperative hydrocele in such cases has not been described. A prospective follow-up study of a consecutive series of male infants weighing less than 3 kg at the time of IH was undertaken. Regular follow-up examinations were scheduled for at least 1 year. Thirty-eight boys weighing less than 3 kg underwent IH during an 18-month period. One subsequently died from complications of prematurity. Complete follow-up data were available for 29/37 (78%) patients, yielding a total of 46 IHs. There were 2 recurrent hernias (4%), 2 unequivocally atrophic testes (4%), and 1 iatrogenic testicular ascent (2%). Five ipsilateral hydroceles complicated the postoperative course of 4 boys (14% of patients, 11% of herniotomies). Two of these were explored, but in neither case was a recurrent/residual patent processus vaginalis found. One hydrocele was aspirated without recurrence, and the remaining 2 resolved spontaneously. The complication rate in small infants undergoing neonatal IH is significantly higher than in older boys. The presence of a hydrocele after neonatal IH may simply reflect the accumulation of fluid in the distal hernia sac and, provided there is no evidence of a recurrent inguinal hernia, an expectant approach is recommended.