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Biomedical subjects

Mark Davenport

Publications and source records attributed to Mark Davenport.

30 records · Page 2Linked to original sources

Antenatal presentation of biliary atresia.

OBJECTIVES: To investigate the incidence, presence of associated anomalies, type of malformation, and natural history of children with biliary atresia (BA) who presented antenatally. Study design The database of 194 infants diagnosed with BA at our tertiary referral unit between 1991 and 2002 was reviewed for infants in whom routine antenatal ultrasound had revealed biliary cystic malformations (BCMs). A retrospective analysis of their medical records was undertaken. RESULTS: Nine infants (six girls) with BCM were identified (4.6% of total referred patients with BA); all were born after 37 weeks' gestational age and presented to us at a median age of 4 weeks (range, 1-14 weeks). The presence of the BCM was confirmed on postnatal ultrasonography. Median age at corrective surgery was 5 weeks (range, 2-16 weeks). At surgery, six patients had type III BA, including one with the biliary atresia-splenic malformation syndrome; two had type II BA; and one had type I BA. Postoperatively, the infants were followed for a median of 2 years (range, 6 months to 11 years). All infants successfully cleared their jaundice. CONCLUSIONS: Improved techniques in routine antenatal ultrasonography can allow early recognition of BCM. This study suggests that BCM noted antenatally may represent early presentation of both biliary atresia-splenic malformation and nonsyndromic BA.

Abnormalities, Multiple↗

Current outcome of antenally diagnosed cystic lung disease.

BACKGROUND/PURPOSE: The natural history of parenchymal lung lesions such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration (PS) has been altered by the advent of antenatal ultrasonography. Initial reports were characterized by a high (about 30%) incidence of adverse features (eg, hydrops) and a poor outcome and did not accord with our recent experience. The authors have reviewed the outcome of fetuses that had been diagnosed in a large tertiary referral fetal medicine unit with the aim of delineating current experience. The term cystic lung disease was used throughout to avoid unjustifiable histologic precision. METHODS: The scans of all fetuses that had been diagnosed with cystic lung disease between January 1995 and July 2001 were reviewed. The outcome of each pregnancy was established, and, where possible, all infants underwent appropriate investigations, including thoracic computed tomography (CT) scans. RESULTS: Sixty-seven fetuses had a cystic lung abnormality diagnosed from January 1995 to July 2001. The median (range) age at diagnosis was 21 (19 to 28) weeks. The lesion was right sided in 29 (43%), left in 36 (54%), and bilateral in 2 (3%); it was characterized as dominantly macrocystic in 27 (40%), microcystic in 35 (52%), and mixed in 5 (8%). Mediastinal shift was present in 30 fetuses (45%). Severe signs of fetal distress (eg, hydrops) were present in 5 fetuses (7%). Antenatal intervention was performed in 4 fetuses (thoraco-amniotic shunts [n = 3] and percutaneous intrauterine laser therapy [n = 1]). Sixty-four (96%) of the fetuses were born alive. There was 1 termination of pregnancy and 2 intrauterine deaths (all severe microcystic lesions). Forty-two infants (63%) underwent thoracotomy and appropriate excisional surgery at a median of 7.5 months (range, 1 day to 34 months). Two infants (which included the fetus having intrauterine laser therapy) died early in the postnatal period. Both were large microcystic lesions and had antenatal features of severe fetal distress. Twelve infants were investigated in the postnatal period but did not undergo surgery. Ten infants were not appropriately investigated or were lost to follow-up. Histologic examination showed definitive diagnostic features of CCAM (n = 25) or PS (n = 6). Other lesions with hybrid features of both were also seen (n = 11). There was a degree of correlation between antenatal ultrasound features (size of cyst [P =.03], in-utero behavior [P =.06], mediastinal shift [P = 0.05]) and the need for surgery but not with the final histologic diagnosis. Surgical excision was required in 45% of lesions showing late-gestation "resolution." CONCLUSIONS: Antenatally diagnosed "cystic lung disease" has an excellent prognosis in the absence of signs of severe fetal distress. The need for surgery should be based on appropriate postnatal investigations (eg, CT scans), rather than on antenatal behavior.

Abortion, Therapeutic↗

Diaphragmatic function in infants with surgically corrected anomalies.

Infants with surgically correctable anomalies, abdominal wall defects (AWD) or congenital diaphragmatic hernia (CDH) may have poor postnatal diaphragmatic function, because the low intra-abdominal pressure experienced by such patients in utero could result in impaired diaphragmatic development. Our objective was to compare postoperative diaphragmatic function of infants with CDH or AWD to that of gestational age-matched controls. Diaphragmatic function was assessed by measurement of the transdiaphragmatic pressure and maximum inspiratory pressure at the mouth generated during crying against an occlusion. In addition, the transdiaphragmatic pressure produced by unilateral and/or bilateral magnetic stimulation of the phrenic nerves (TwPdi) was examined. Lung volume was assessed by measurement of functional residual capacity (FRC) using a helium gas dilution technique. Ten infants with CDH, 26 with AWD infants (19 gastroschisis, seven exomphalos), and 36 gestational age-matched controls were studied. Compared with their matched controls, the eight CDH infants with left-sided defects had significantly lower left (p < 0.01) and right (p < 0.05) TwPdi and FRC (p < 0.01), and the gastroschisis infants, but not those with exomphalos, had significantly lower left and right TwPdi (p < 0.05). There were no significant differences in transdiaphragmatic pressure and maximum inspiratory pressure at the mouth between the CDH or AWD infants and the controls. Diaphragmatic function postoperatively is impaired in infants with CDH or gastroschisis.

Abdominal Wall↗

The spectrum of surgical jaundice in infancy.

BACKGROUND: Conjugated jaundice arising during infancy may be caused by a number of different surgical conditions. The aim of this study was to compare clinical features, management, and outcome of all types of surgical jaundice presenting in the first year of life. METHODS: A retrospective review was conducted of all infants born in the United Kingdom with jaundice caused by a surgical cause referred to the authors' institution from January 1992 to December 1999. RESULTS: There were 171 infants who could be separated into 3 specific groups: biliary atresia (BA, n = 137), inspissated bile syndrome (IBS; n = 14), and choledochal malformation (CM; n = 12) together with a group containing various miscellaneous conditions (n = 8). Infants with BA had higher bilirubin (P <.01) and aspartate aminotransferase levels (P <.001) and came to surgery earlier (P <.01) than infants with either IBS or CM. Infants with IBS and CM were more likely to be premature and have other malformations, respectively. Ultrasound scan was the principal investigation in the differentiation of BA from other causes of jaundice. Accurate prelaparotomy diagnosis was made by percutaneous liver biopsy in 87% of cases later shown to be BA. Currently, 88 (64%) of children with BA are alive with their native liver postportoenterostomy, 4 have died, and 45 have undergone liver transplantation (with 1 death postoperatively). A policy of primary portoenterostomy for BA followed by transplantation, if necessary, resulted in a survival rate of over 95%. All children in the other diagnostic groups are alive and anicteric after appropriate surgical intervention. CONCLUSIONS: Approximately 80% of infants presenting with surgical jaundice have biliary atresia, whereas those with inspissated bile syndrome and choledochal malformations make up most of the remainder. Mortality in this age-group is confined to infants with BA, but even on these infants an overall survival rate of greater than 95% is currently expected.

Abnormalities, Multiple↗

Prenatal diagnosis of liver and biliary tract disease.

The diagnosis and detection of some forms of liver and bile duct disease have become possible since the adoption of routine fetal ultrasound scanning in the UK, and the remarkable advances in molecular biological techniques. Nevertheless, although antenatal detection may well have improved, it remains difficult to offer a specific diagnosis, much less a prognosis, in most cases. Cystic dilatation of the biliary tree is probably the most common finding. This may be due to cystic choledochal malformations or, occasionally but crucially, biliary atresia. Postnatal investigations (such as ultrasonography, magnetic resonance cholangiography and liver histology) must take the latter possibility into account, as early surgery is the key to a successful outcome following reconstructive biliary surgery. Antenatally diagnosed liver tumours are usually of vascular origin and have an unpredictable natural history. Some may cause fetal cardiac failure and are candidates for in-utero intervention, but in all likelihood, a large proportion will resolve without specific therapy.

Biliary Tract Diseases↗

Long-term survival following Kasai portoenterostomy: is chronic liver disease inevitable?

OBJECTIVES: Extrahepatic biliary atresia (EHBA) is the most common indication for liver transplantation in childhood. Most children who do not undergo transplant are reported to have chronic liver disease and its complications. The aim of this single-center study was to identify children with normal laboratory indices and no clinical evidence of chronic liver disease 10 or more years after Kasai portoenterostomy (KP). METHODS: A retrospective analysis of the medical notes of all children surgically treated at the authors' center between 1979 and 1991 was undertaken. Criteria for inclusion were absence of surgical complications, unremarkable clinical examination, and normal bilirubin, aspartate aminotransferase, albumin, international normalized prothrombin ratio, and platelet count. RESULTS: Of 244 children surgically treated during the observation period, the authors identified 28 (11%) adolescents (14 male) who fulfilled the entry criteria. Their median age was 13.4 years (range, 10.2-22.2 years). Twenty-six with type 3 EHBA had conventional KP, whereas 2 underwent modified operations. The corrective surgery was performed at a median age of 58 days (range, 20-99 days). Median time of complete clearance of jaundice from the date of KP was 75 days (range, 21-339 days). Twelve (43%) patients had history of cholangitis at a median age of 3.4 years. The liver histologic findings were suggestive of mild to moderate fibrosis in 54.2% and cirrhosis in 40.7% of the patients who underwent biopsy. No child had gastrointestinal bleeding during follow-up. Thirteen (46%) patients had an elective esophagogastroduodenoscopy, which was normal in all. Twenty-six (93%) patients were in mainstream education, whereas the remaining two (7%) attended special school because of reasons unrelated to liver disease. CONCLUSIONS: A sizable proportion of children with EHBA avoid significant chronic liver disease and its complications 10 years or more after conventional surgical correction and have an excellent quality of life. Their good outcome is not hampered by isolated episodes of ascending cholangitis. Whether or not the residual histologic damage will become symptomatic during their lifetime remains to be established.

Biliary Atresia↗

Laparoscopic surgery in children.

A review of laparoscopy as used in paediatric surgery with special reference to cholecystectomy, appendicectomy, fundoplication, the undescended testicle, inguinal hernia and hydroceles.

Adolescent↗

Congenital mesenterico-portal (Rex) shunt.

Congenital anomalies of the portal and umbilical-venous system are rare, with few reported cases. We describe a case where the native umbilical vein had acquired mesenteric venous outflow to bypass an in-utero portal-vein (PV) obstruction and restore normal venous return to the liver. This is analogous to the recently described Rex shunt procedure used in some cases of extrahepatic portal hypertension due to PV occlusion.

Hernia, Umbilical↗

Acute and chronic pancreatitis.

Pancreatitis is under appreciated during childhood although its diagnosis is simple and management straightforward in most cases. There is a range of possible causes, which is quite different to the situation in adults. The commonest underlying problems are probably structural abnormalities of the pancreatic and biliary ducts such as choledochal malformation, common pancreatobiliary channel and pancreas divisum. Other causes, which can be important in certain groups and geographical areas, are those due to drug reactions, viral infection and parasitic infestation, and blunt abdominal trauma. The diagnosis is established by showing a significantly raised plasma amylase level. Other diagnostic tools such as ultrasound, computed tomography (CT) scanning and endoscopic retrograde cholangiopancreatography (ERCP) have a major role in determining possible underlying causes, and hence selecting out those who require definitive corrective surgery. The pathophysiology of pancreatitis remains to be fully elucidated and, in the acute phase can affect other organs such as the renal and respiratory systems. Later complications include sepsis, pancreatic abscess and typically pseudocyst formation. Most of these can be treated using minimally invasive techniques such as percutaneous aspiration although open surgical techniques such as cystgastrostomy may be required in a few.

Acute Disease↗