The role of IDA scintigraphy in the follow-up of liver disease in patients with cystic fibrosis.

The main aim of this study was to investigate the role of N-(2,4,6 trimethyl-3-bromophenylcarbamoylmethyl) iminodiacetic acid (IDA; Mebrofenin) scintigraphy in follow-up assessments of the biliary system in patients with cystic fibrosis associated liver disease. Fourteen patients from a study published in 1996 were re-examined after a mean interval of 4.7 years from their initial study, in which diisopropylphenyl carboxymethyl iminodiacetic acid (DISIDA) was used. The results of ultrasound, liver function tests and clinical examination were also compared. Twelve of the patients had been treated with ursodeoxycholic acid and taurine in the interim. Five subjects' IDA examinations showed a slight improvement on follow-up, six deteriorated, two were unchanged, whilst one demonstrated a 'mixed picture'. Overall, nine patients deteriorated in one or more of the tests. No patient showed a decline in all four investigations and only two in three. There was poor correlation between the various follow-up examinations, with different patients showing a decline in some tests but not others. This may be due to the mixture of functional and anatomical studies utilized, their differing sensitivies, and the fact that deterioration in one did not necessarily affect another. In conclusion, follow-up of hepatobiliary disease in patients with cystic fibrosis cannot be encompassed by one method alone. If early detection of disease progression would affect management, patients will continue to require a number of investigations rather than a single test.

Venous patency after open central-venous cannulation.

To investigate the value of Doppler ultrasound scan (USS) assessment of internal jugular vein (IJV) patency after previous open central-venous cannulation (CVC), a prospective study of 66 consecutive children (median age 4.5 years; range 4 months-17 years) who had previously undergone open insertion of at least one indwelling IJV line and required further CVC for completion of therapy was undertaken. All underwent Doppler USS examination prior to surgery. Where patency of the previously cannulated vein was suggested ultrasonographically, the accuracy of this finding was confined at open surgical exploration. Initial CVCs were in situ for a median of 9 months (1 month-4 years) prior to removal. The median interval to repeated CVC was 11 months (3 weeks-45 months). In 79 Doppler USS, 70 (88.6%) veins appeared patent, 3 (4.2%) stenosed, and 6 (7.6%) obliterated. Of the 70 "USS patent" veins, 66 were explored. Patency was confirmed surgically in 59 (89.4%) and a new CVC successfully inserted. Seven (10.6%) apparently patent veins on USS were found to be obliterated at open exploration. Review of USS images in these cases suggested that enlarged collateral veins were usually responsible. Overall, successful recannulation was possible in 74.6% of all previously accessed veins. In children requiring repeated CVC, Doppler USS of neck veins is a valuable but not entirely reliable guide to the presence of underlying vessel patency and should be interpreted with caution. At least three-fourths of previously cannulated IJVs remain patent after catheter removal and can be reused for CVC.

Colonic wall thickness, pancreatic enzyme dose and type of preparation in cystic fibrosis.

Increased colonic wall thickness has been reported in patients exposed to large doses of high strength pancreatic enzyme preparations who did not develop fibrosing colonopathy. This has been interpreted as evidence for a spectrum of subclinical disease. The relation between sonographically measured colonic wall thickness and pancreatic enzyme preparation and dose was studied in 86 children with cystic fibrosis (CF). Colonic wall thickness of a control group was also measured. The average thickness in all colonic regions was higher in the CF group (overall average range 0.7-2.5 mm v 0.6-1.4 mm in the control group). There was no significant relation between colonic wall thickness and age, sex, total dose of lipase, or copolymer. Apart from one patient with an early colonic stricture, none of those exposed to high doses of lipase, or the methacrylic acid copolymer Eudragit L30 D55, showed evidence of subclinical damage to the colon. The reproducibility of the sonographic measurements was poor.

Gastroschisis: a radiological and clinical review.

A retrospective clinico-radiological review was undertaken of 66 consecutive cases of gastroschisis managed at our institution between August 1982 and February 1993. The condition's morbidity and mortality were reviewed, as were its radiological features and their impact upon management. All patients underwent surgery in the first 24 h of life, and the overall survival rate was 92 %. The finding of bowel atresia at operation was associated with a particularly poor outcome, with only two out of five infants surviving. A minority of infants developed serious complications including necrotizing enterocolitis, short-bowel syndrome, persistent small-bowel dysfunction and cholestatic jaundice. Investigation by plain films, contrast studies and ultrasound examinations was necessary and helpful in these patients. Plain film radiography commonly revealed bowel-wall thickening and luminal dilatation, frequently accompanied by generalised abdominal distension. Small-bowel enema was considered to be superior to the conventional follow-through in distinguishing mechanical from functional obstruction in infants with persistent bowel dilatation.

Case report: the sonographic appearance of neonatal herpes simplex encephalitis.

This case report describes the sonographic appearances in a proven case of neonatal Herpes Simplex encephalitis. Both early and late phase appearances are discussed, and are compared with other reports of imaging in similar disease processes. A role for sonography in the management of neonatal Herpes Simplex encephalitis is suggested.

Case report: central venous catheter fracture due to compression between the clavicle and first rib.

A central venous catheter inserted via the subclavian route may be compressed by the clavicle and adjacent first rib. This appearance on a radiograph has been previously described as the 'pinch-off sign' and indicates the need to remove the catheter, due to a significant risk of subsequent fracture. A case and its management are presented, followed by discussion of the radiographic appearances and their cause.

Is the appendix where you think it is--and if not does it matter?

For over 100 years the recognized surface marking for the appendix has been McBurney's point (the junction of the lateral and middle thirds of a line joining the umbilicus with the right anterior superior iliac spine (ASIS)). In order to test its validity, the relationship between McBurney's point and the appendix was determined on 275 double contrast barium enemas (DCBE). A wide spread of distribution of the site of the appendix base was seen. Only 35% of appendix bases were found to lie within 5 cm of McBurney's point, and 15% were more than 10 cm distant. Seventy-five per cent of appendix bases were below and medial to a line joining the umbilicus with the right ASIS. These findings are in agreement with world-wide studies conducted by the World Organisation of Gastroenterology which showed that less than half of all patients with appendicitis have tenderness maximal over McBurney's point. A record was also made in 93 cases of the position of the appendix in relation to the caecum. The importance of these results in the diagnosis and management of patients with suspected appendicitis is discussed.

Oxygen tension on the skin of the gaiter area of limbs with venous disease.

We examined the levels of oxygen on the skin of the gaiter areas of limbs with venous disease using a Roche Transcutaneous pO2 Monitor to determine whether hypoxia contributes to the skin changes and ulceration associated with severe venous disease. We studied patients with superficial varicose veins and no skin changes (Group 1: n = 25), patients with skin changes and healed ulcers (Group 2: n = 15), and an age matched normal control group (controls: n = 25). TcpO2 measurements were taken 5 cm above the medial malleolus with the limb both horizontal and vertical using a Roche Transcutaneous pO2 Monitor. A standard heel-raising test using a Medisonics Photoplethysmograph indicated the degree of venous insufficiency present. Results show that Group 1 patients and controls have similar skin oxygen levels (Group 1: TcpO2 66.48 + 14.12 mmHg; controls: TcpO2 61.60 +/- 11.02 mmHg) whereas those with skin changes and ulceration have significantly lower oxygen levels (Group 2: TcpO2 46.57 +/- 9.70 mmHg, t = 4.29, P less than 0.001). Group 2 patients did not show a significant rise in TcpO2 levels in the standing position whereas Group 1 patients and controls did (t = 2.6, P less than 0.02). PPG post-exercise recovery times confirmed differences between the three groups. These findings show that significant skin hypoxia occurs on the gaiter area of limbs with severe venous disease and support the concept of an oxygen diffusion block.