Bronchopulmonary foregut malformations: embryology, radiology and quandary.

Bronchopulmonary foregut malformations (BPFM) are a heterogeneous group of pulmonary developmental anomalies that present at varying ages and with overlapping symptoms, signs and radiology. This article discusses the embryology of these lesions with reference to possible common origins and the link between aetiology and radiological appearance. The radiology of each lesion, both antenatally and postnatally, is described and illustrated. A number of quandaries exist in the prediction of prognosis and subsequent treatment of BPFM. We discuss the radiological features that may help to elucidate an individual prognosis and aid in the planning of treatment. The treatment options available for BPFM are briefly discussed. Finally, the link between BPFM, in particular cystic adenomatoid malformations and malignancy, is discussed. We aim to provide a comprehensive overview of the embryology, radiology, prognosis and treatment highlighting contentious issues of BPFM.

Extracranial vascular malformation: value of antenatal and postnatal MRI in management.

We report a case where fetal MRI using a low-field-strength magnet (0.5 T) accurately confirmed a large extracranial vascular malformation, which was suspected on antenatal US. Fetal MRI enabled better counselling of the parents and allowed suitable plans to be made regarding method of delivery and early management of the neonate. To our knowledge this is the first case of an extracranial vascular malformation imaged using fetal MRI.

Persistent acquired lobar overinflation complicating bronchopulmonary dysplasia.

UNLABELLED: Persistent acquired lobar overinflation (PALO) may complicate bronchopulmonary dysplasia (BPD). From infants admitted to the regional neonatal intensive care unit or who had been followed up at the chronic lung disease clinic in Liverpool over a 6.5-year period, 11 children with BPD and PALO were identified and details of their neonatal and subsequent outcome obtained. Their median gestational age was 29 weeks (range 24-33) and median birth weight was 1317 g (range 676-1968 g). All had received ventilatory support for severe neonatal respiratory distress syndrome for a median of 26 days (range 5-86). The median age the acquired lobar overinflation was detected was 82 days (range 45424 days). Nine patients required continued neonatal or paediatric intensive care re-admission for deteriorating respiratory function. Six children have subsequently died at a median age of 9.5 months (range 6.5-20). Five patients underwent bronchoscopy, four suggesting the presence of bronchomalacia. Three patients had ventilation-perfusion scans all showing that the overinflated lobe had no mismatch defect unlike other areas of the lung. CONCLUSION: The place of specific therapies for persistent acquired lobar overinflation is unclear. Surgery to remove the overinflated lobe in such cases may be inappropriate and the outcome of this complication of bronchopulmonary dysplasia appears to be poor.

Outcome of congenital lung abnormalities detected antenatally.

To determine the outcome of congenital lung abnormalities, data were collected retrospectively between January 1991 and December 1996 on any foetus found to have a lung lesion on antenatal ultrasound. A total of 23 foetuses had lung lesions on antenatal ultrasound. In two foetuses the antenatal ultrasound showed bilateral enlarged "bright" echogenic lungs with evidence of hydrops. Both pregnancies were terminated and tracheal atresia was confirmed. In 15 foetuses the antenatal ultrasound appearance was of a unilateral "bright" echogenic lung. There was one case of bronchial atresia and two cases of congenital lobar emphysema, which all had surgery. In nine cases there was a reduction in the size of the lesion on serial antenatal ultrasounds and no lesion was detected after birth. In three cases a small lesion was present after birth on chest radiography. In six foetuses the antenatal ultrasound appearance was of unilateral cystic or mixed cystic and echogenic lung lesions. Two pregnancies were terminated; both had congenital cystic adenomatoid malformation. Four pregnancies were continued and three infants had surgery soon after birth and were confirmed to have had congenital cystic adenomatoid malformation. One infant has been managed conservatively. In conclusion, a definitive diagnosis cannot usually be made antenatally. A large lesion on initial scan does not necessarily predict a poor outcome. The natural history of small asymptomatic postnatal lesions is unknown and a long-term prospective study is needed to determine the outcome of these lesions.

Interpretation of the neonatal chest X-ray.

Most neonatal X-rays are seen initially by a paediatrician without formal training in interpretation of chest X-rays. This article aims to help improve the information obtained from these X-rays which are often complex. Many factors affect accurate interpretation of the neonatal chest X-ray, including good quality radiographs, appropriate viewing conditions and thorough education.

The clinical significance of fetal right heart enlargement with a structurally normal heart.

Isolated right heart enlargement of the fetus without cardiac structural abnormalities appears to be associated with growth retardation or abnormal perinatal outcome. We report the outcome of eight fetuses with subjective enlargement of the right heart. All cases were diagnosed antenatally from a standard four-chamber view on an ultrasound scan. All fetuses had otherwise normal cardiac anatomy. Seven out of the eight fetuses ultimately had outcomes which deviated from the norm. An isolated finding of fetal right heart enlargement warrants close antenatal fetal surveillance.

Ovarian ultrasound.

Ovarian ultrasound is applicable to all ages, from the fetus through childhood and adolescence to the premenopausal and finally postmenopausal women. Indications include the chance finding of an ovarian lesion in the fetus, the diagnosis of ovarian pathology resulting in symptoms and the uses of follicle monitoring in infertility and screening for ovarian cancer in high-risk groups.

Early antenatal ultrasound diagnosis of fetal intracranial teratoma.

The commonest fetal intracranial tumour is a teratoma. The prognosis is poor with death usually occurring shortly after birth. Modern high resolution ultrasound scanners facilitate examination of the cranial contents, allowing earlier diagnosis. We report a case where an intracranial teratoma was identified at 21 weeks gestation, the earliest gestational age that this has been reported. The ultrasound appearances are discussed.

Congenital lobar emphysema in congenital cytomegalovirus infection.

We report a case of congenital lobar emphysema diagnosed antenatally in an infant of 32 weeks' gestation. Histology and serology confirmed infection with cytomegalovirus (CMV). CMV pneumonitis leading to inflammation and obstruction in the bronchial tree may have resulted in the development of congenital lobar emphysema.

Antenatal and neonatal renal vein thrombosis: new ultrasonic features with high frequency transducers.

The ultrasonic appearances of two infants with renal vein thrombosis (RVT) are described. One infant presented in the neonatal period and one antenatally. Consistent with previous reports, one or both kidneys were enlarged, with a generalised increase in echogenicity and vascular or perivascular streaking compatible with interlobular and arcuate vessel occlusion. Using high frequency transducers (7. 0-10 MHz), we identified new features during the course of the disease. The apex of the renal papilla became hypo-echoic and there was a ring of reduced echogenicity around the affected pyramids 1-2 weeks after the acute phase. These features correlate with the known pathophysiological course of RVT, but have not been previously described using ultrasound. The features are illustrated and the mechanism is discussed.

Fetal echogenic lung lesions: prenatal ultrasound diagnosis and outcome.

The differential diagnosis of echogenic areas in the fetal chest include congenital diaphragmatic hernia (CDH), cystic adenomatoid malformation (CAM), sequestrated lung and tracheal or bronchial atresia. The purpose of this study was to evaluate the accuracy of prenatal diagnosis and document outcome in fetuses with echogenic chest lesions. Seventeen fetuses with echogenic chest masses were seen in our unit between 17 and 36 weeks' gestation over a 5-year period. We reviewed these cases retrospectively for prenatal diagnosis, postnatal diagnosis and outcome. Prenatal diagnosis was correct in 13 fetuses, with CDH in 8, sequestrated lung in 4 and tracheal atresia in 1. Four fetuses had incorrect or uncertain prenatal diagnoses. In three fetuses CDH and CAM could not be differentiated. After delivery two of these had CDH and one had sequestrated lung. One fetus with bilateral lesions had prenatal diagnosis of bilateral CAM. Post-mortem examination revealed tracheal atresia as part of Fraser syndrome. All five babies with sequestrated lung are well and none required surgery. Ten fetuses had CDH, two pregnancies were terminated, one died in utero, five died as neonates and two babies survived following surgery. The study reveals that in a minority of fetuses CDH and CAM could not be differentiated prenatally. We agree with recent reports of fetal sequestrated lung describing sonographic improvement in utero. A large lesion on initial scan does not necessarily predict a poor neonatal outcome in this condition. This, together with the poor outcome in fetuses with echogenic CDH and tracheal atresia, has important implications for prenatal counselling.

Intrathoracic rib.

The intrathoracic rib is a rare anomaly. Despite this, it has distinctive radiological features which should enable a confident diagnosis to be made from the chest radiograph alone. Its importance lies in recognising it as a benign lesion and limiting further investigation appropriately. We present an 11-year-old girl with an intrathoracic rib found incidentally on a chest radiograph.

The relationship of pulmonary interstitial emphysema to subsequent type of chronic lung disease.

Chronic lung disease of prematurity (CLD) can be classified using the chest radiograph taken on day 28 into type 1 (homogeneous opacification) or type 2 (the presence of cystic change) and it has been suggested that the presence of pulmonary interstitial emphysema (PIE) leads to the development of type 2 CLD. To further study this relationship we examined the chest radiographs taken on days 1-7, 14, 21 and 28, of 202 infants treated at Liverpool Maternity Hospital between January 1980 and December 1989 who developed CLD. 39% (54/137) of infants who developed type 1 CLD had suffered from PIE compared with 78% (51/65) with type 2 CLD (p < 0.001). There was no difference in the incidence of PIE in infants who developed CLD and subsequently died compared with those who survived. In the infants who survived there was no significant difference in length of oxygen dependency between those who suffered from PIE (median 99 days oxygen dependent) and those who had no PIE (median 105 days). We conclude that the presence of PIE is associated with subsequent development of type 2 CLD but is not a prerequisite for this radiographic type of chronic lung disease. Of those infants who survive to develop CLD, the presence of PIE does not alter prognosis in terms of duration of oxygen dependency or mortality.

Current imaging of childhood urinary infections: prospective survey.

OBJECTIVES: To assess whether ultrasonography alone is adequate for routine screening of childhood urinary infection, whether clinical features determine the need for further investigations, and which investigations are most appropriate. DESIGN: Prospective survey of children with proved urinary infection and a preinvestigation record of clinical features. Ultrasonography and intravenous urography were routine, with choice of further studies determined by ultrasonographic findings. SETTING: A children's hospital and two district general hospitals in Mersey region. MAIN OUTCOME MEASURES: Sensitivity and specificity of ultrasonography both generally and in relation to clinical features. Accuracy of intravenous urography compared with radioisotope examinations. RESULTS: Specificity of ultrasonography was good (99% (95% confidence interval 96% to 100%)) but sensitivity modest (43% (32% to 55%)), principally with respect to detecting vesicoureteric reflux and renal scarring. Among older children (aged 2-10 years) with positive ultrasound results and fever or vomiting the sensitivity in detecting reflux (with and without renal scarring) was 78% (62% to 89%) and the specificity 69% (60% to 78%); in detecting renal scarring (with and without reflux) the sensitivity was 100% (80% to 100%) and specificity 65% (56% to 74%). Renal scarring and obstructive uropathies were better assessed by radioisotope examinations than by intravenous urography. CONCLUSIONS: Ultrasonography alone is inadequate for routine screening of childhood urinary infection. Though further investigations remain advisable in infants, in older children they can be restricted to a minority who have positive ultrasound examinations or have had fever or vomiting. Radioisotope examinations largely eliminate the need for intravenous urography.

The role of ultrasound in the management of ovarian masses in children.

A retrospective study of 64 girls with ovarian cysts and tumours, diagnosed by ultrasound over 4 years, was undertaken. Most ovarian cysts were benign and conservative management with serial ultrasound scans helped to avoid unnecessary surgery. The incidence of surgery decreased from 18 operations in the first 2 years of the study to 10 operations in the second 2 years. All children for whom surgery is considered should have a pre-operative scan to determine whether the cyst has resolved. Immediate surgery is indicated in children who have a palpable mass, a solid mass, a mass associated with calcification, or a mass associated with persistent pyrexia. Appendix abscesses may mimic ovarian masses and can be correctly diagnosed by ultrasound.

Neonatal ovarian cysts: therapeutic dilemma.

Seven cases of neonatal ovarian cysts that presented over the past seven years were studied. Complications included torsion and rupture and usually occurred in cysts more than 5 cm in diameter. Surgical removal, either oophorectomy or cystectomy, was the treatment of choice. Because even cystectomy results in loss of normal ovarian tissue, and because spontaneous regression of cysts less than 5 cm in diameter can occur, a more conservative approach is now proposed. Regular ultrasonography alone is recommended if the cysts are less than 5 cm in diameter, and aspiration of the cysts followed by regular ultrasonographs if the cysts are more than 5 cm in diameter. Operation should be reserved for recurrent cysts or for those with complications. Cysts diagnosed antenatally may be aspirated in utero if there are signs of thoracic compression.