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Mark Davenport

Publications and source records attributed to Mark Davenport.

At least 19 recordsLinked to original sources

Staged resection for a ruptured hepatoblastoma: a 6-year follow-up.

Hepatoblastoma (HB) is a rare germ cell tumour of childhood usually presenting with progressive abdominal distention. However, presentation as acute abdomen is a rare occurrence and is secondary to spontaneous rupture. This presentation carries high mortality. To our knowledge, six cases of ruptured hepatoblastoma have previously been reported, although the long-term outcome has not been clear. We report a case of ruptured HB who was managed by initial control of haemorrhage by laparotomy followed by chemotherapy with high-risk hepatoblastoma protocol as per SIOPEL 2 (cisplatin, carboplatin and doxorubicin) and a staged hepatectomy 5 months later. Patient is currently disease free at 6-year follow-up. Staged hepatectomy after initial control of haemorrhage does not preclude a curative resection.

Antineoplastic Combined Chemotherapy Protocols↗

Management of congenital lung lesions.

Antenatally diagnosed lung lesions are most commonly congenital cystic adenomatiod malformations (CCAMs) or bronchopulmonary sequestrations (BPS). Congenital lobar emphysema and bronchogenic cysts occur rarely. CCAMs and BPS can only be truly distinguished on histopathological assessment. CCAMs can be defined using either Stocker's histological classification, or the clinically more useful 'macrocystic' and 'microcystic' subtypes introduced by Adzick. Adverse pathophysiological effects (e.g. lung hypoplasia, mediastinal shift, caval obstruction and hydrops) can occur with those of larger volume in the developing fetus and are associated with a poor outcome ( approximately 5%). In centres with appropriate expertise, antenatal intervention (e.g. thoracoamniotic shunting) should be considered for such cases. In the postnatal period, early radiological evaluation (e.g. CT scan) is essential as many cases which apparently 'regress' antenatally are still detectable and may be a cause of later morbidity. Early surgical excision is required for symptomatic cases and should also be considered for asymptomatic antenatally diagnosed lesions, because of the risks of later infection and the possibility of malignant transformation. An excellent outcome following surgery can be expected.

Humans↗

Idiopathic biliary perforation in a 10-year-old boy.

A 10-year-old boy of African origin with AIDS and Stage IV Hodgkin's lymphoma presented with a short history of abdominal distension and bile ascites shown to be due to a perforation of his common bile duct. This was treated initially by laparotomy, external peritoneal drainage and endoscopic biliary stenting although without success. He then underwent a laparotomy and biliary diversion (Roux-en-Y hepaticojejunostomy) with satisfactory resolution of his bile duct pathology. Although the co-morbid conditions are likely to be contributory factors, biliary perforation in either disease has not been reported before.

Child↗

Biliary atresia: outcome and management.

Untreated, biliary atresia remains a fatal condition of the newborn. Most present within four to six weeks of conjugated jaundice and acholic stools and, although still a challenging diagnosis to make, therein lies the opportunity of changing the course of this otherwise inexorable disease. The aim of surgery is to restore bile flow, alleviate jaundice and abbreviate the cholangiodestructive process within the liver. The Kasai portoenterostomy, introduced almost 50 years ago in Japan, aims to expose microscopic biliary ductules within the fibroinflammatory mass at the porta hepatis and restore bile drainage into a mobilised Roux loop. About 50% of infants with BA will be able to clear their jaundice following Kasai alone, given appropriately experienced surgeons and if performed prior to the onset of overt cirrhosis. They have a reasonable expectation of long-term survival to adulthood with a good quality-of-life. The remainder may be candidates for liver transplantation (where available) although donor organ shortage and immunosuppresion-related complications remain significant problems.

Biliary Atresia↗

The biliary atresia splenic malformation syndrome: a 28-year single-center retrospective study.

We carried out a retrospective review of infants with biliary atresia splenic malformation (BASM). We found that 56 infants (10.2%) met the criteria for inclusion from a series of 548 infants (from January 1977 to December 2004). Syndromic infants were more likely to be female (P = .04) and to have a higher incidence of antenatal pathology (specifically maternal diabetes; 12.5% vs 1.2%; P < .0001). Situs inversus was noted in 21 (37%) and cardiac abnormalities in 25 (45%) infants. There were no differences in liver histology (eg, degree of liver fibrosis) or in the HLA genotype between BASM and nonsyndromic infants. Five-year and 10-year estimated native liver survival were 46% and 32%, respectively. There were 7 long-term survivors with their native liver and a follow-up of more than 10 years; all were anicteric. BASM is a distinct subgroup, with an implied onset during the embryological phase of organ development.

Abnormalities, Multiple↗

Outcome of antenatally diagnosed sacrococcygeal teratomas: single-center experience (1993-2004).

AIMS: Sacrococcygeal teratomas (SCTs) are the commonest neonatal tumors with an incidence of approximately 1:30,000. There are few large single-center series and even fewer describing both their antenatal and postnatal course. We report the outcome of all fetuses investigated at a tertiary fetal medicine center with this diagnosis. METHOD: Demographic details were obtained from a prospectively maintained database. Patient records were examined for additional data including antenatal and postnatal interventions. Data were described as median (range). RESULTS: Forty-one SCTs were diagnosed antenatally during the period 1993 to 2004. Twelve were excluded from subsequent analysis (single antenatal visit or attending for second opinion [n = 6] and termination of pregnancy [n = 6]). Twelve underwent fetal intervention (laser vessel ablation [n = 4], alcohol sclerosis [n = 3], cyst drainage [n = 2], amniodrainage [n = 2], vesicoamniotic shunt [n = 1]) for fetal hydrops and polyhydramnios to aid in delivery and to prevent obstructive uropathy developing in the fetus. Of these, 3 died in utero and 9 survived to be born (median gestational age, 33 weeks [27-37 weeks]). A further 3 died in the neonatal period. There are 6 long-term survivors (50%) from this group. Seventeen infants, without intervention, were born at median gestational age 38 weeks (26-40 weeks). One infant with severe cardiac anomalies died on the day of birth. All surviving infants had definitive excisional surgery at a median of 2 days (1-16 days). Current median follow-up of survivors is 39 months (8-86 months). There have been no recurrences. One child has mild constipation, and 3 are awaiting cosmetic revision of their scars. CONCLUSIONS: The overall survival of antenatally diagnosed SCT is approximately 77%, with the development of hydrops and others requiring in utero intervention carrying a poor prognosis. Otherwise, the outcome after surgical excision is excellent.

Adult↗

Late-presenting cholangitis and Roux loop obstruction after Kasai portoenterostomy for biliary atresia.

BACKGROUND/PURPOSE: Late-onset cholangitis is an uncommon complication after the Kasai operation for biliary atresia. Experience with 3 recent patients illustrates appropriate management. METHODS: Retrospective review of patients with late unexplained deterioration in liver function attributed to cholangitis. All underwent quantitative radioisotope hepatobiliary scans to identify the Roux loop obstruction and confirm postoperative resolution. The role of percutaneous transhepatic cholangiography and computed tomography scanning was reviewed. RESULTS: Three patients aged 8, 13, and 17 years were identified. All 3 had had a successful Kasai portoenterostomy for type 3 biliary atresia and presented after several virtually trouble-free years with late-onset cholangitis. The radioisotope scans in all 3 patients showed poor tracer uptake by the liver and pooling of the radioisotope at the porta hepatis. Laparotomy confirmed a proximal Roux loop obstruction at the level of the mesocolic window in all. This required mobilization and adhesiolysis and, in 1, correction of an actual stenosis within the wall of the intestine at this level. All patients had normalization of their liver function postoperatively. All are now well and anicteric at a follow-up of 6 to 12 months. CONCLUSIONS: Long-term survivors of the Kasai portoenterostomy for biliary atresia with immediate deterioration in liver function warrant investigation for possible Roux loop obstruction. Resolution of the obstruction allows preservation of their native liver.

Adolescent↗

Current outcome of antenatally diagnosed exomphalos: an 11 year review.

PURPOSE: The aim of this study was to determine the outcome of antenatally diagnosed exomphalos. METHODS: The database of a tertiary referral Fetal Medicine Centre was searched for all cases of antenatally diagnosed exomphalos between January 1991 and December 2002. Patients, general practitioners, and hospitals were contacted for outcome details. RESULTS: In total, 445 cases of exomphalos were identified. In 250 (56%) cases, the fetal karyotype was abnormal (group A), in 135 (30%) cases, the karyotype was normal (group B), and in 60 (14%) cases, karyotyping was declined (group C). In group A, there were 248 (99%) terminations of pregnancy (TOP) or fetal deaths and 2 live births. In group B, 74 (54%) fetuses had other structural anomalies; 82 (61%) pregnancies resulted in TOP or fetal death, 42 (31%) in live births, and 11 (8%) were lost to follow-up. In group C, 38 (63%) fetuses had other structural anomalies; 41 (69%) pregnancies resulted in TOP or fetal death, 11 (18%) in live births, and 8 (13%) were lost to follow-up. Of the 55 live births, 11 died preoperatively and 44 had surgery. There were no postoperative deaths. CONCLUSIONS: Less than 10% of the antenatal diagnostic workload reached operative repair. In our unit, these babies are a highly selected group, which is a factor in the high postoperative survival.

Abnormalities, Multiple↗

Minimally invasive management of bile leaks after blunt liver trauma in children.

BACKGROUND: Management of bile leaks after blunt abdominal trauma remains controversial. Conventionally, open surgery has been considered necessary, but new modalities of treatment, including endoscopic biliary stenting and laparoscopy, offer a minimally invasive alternative. MATERIALS AND METHODS: A retrospective review of all cases of blunt liver trauma in children treated at our institution between May 2002 and October 2004 was performed looking for possible biliary injury. RESULTS: Five children (3 boys), median age 13 years (range, 10-15 years), were referred to our institution at a median time of 4 days (1-15 days) after the initial trauma. Mechanism of injury was motor vehicle accident (n = 3), fall from a motorbike/Quad bike (n = 2), and a scooter handlebar injury (n = 1). Two, who were hemodynamically unstable, required laparotomy within 24 hours, although their subsequent management was minimally invasive. Endoscopic retrograde cholangiopancreatography, performed at a median time of 15 days (2-28 days), demonstrated an intrahepatic biliary leak in all 5 patients. Biliary stenting was performed in each case, with 2 also having a sphincterotomy. One subsequently developed a bile duct stricture that was managed by endoscopic dilatation. Four required additional percutaneous external drainage of intraabdominal collections. Two underwent laparoscopy to facilitate peritoneal lavage and rule out bowel injuries. No child required open surgery to treat the bile leak. Median hospital stay was 43 days (range, 15-58 days). CONCLUSIONS: A minimally invasive, multidisciplinary approach to traumatic bile leaks, as an alternative to open surgery, is practical and safe. It requires flexibility, particularly if the diagnosis has been delayed, and may still involve a prolonged hospital stay.

Abdominal Injuries↗

Jejunal blind loop 10 years after neonatal necrotizing enterocolitis.

A 10-year-old girl presented with abdominal distension and vomiting due to a jejunojejunal bowel fistula, forming a blind loop. This was related to neonatal necrotizing enterocolitis (NEC) in her first few weeks of life, which was followed by apparently a full recovery. We would like to raise awareness of possible long-term gastrointestinal sequelae in children who have had NEC.

Child↗

Twitch airway pressure elicited by magnetic phrenic nerve stimulation in anesthetized healthy children.

Children with diaphragm dysfunction may be unable to maintain adequate ventilation. Accurate diagnosis is important, but can only be achieved using an appropriate test and reference range. The aim of this study, therefore, was to measure diaphragm contractility and examine the influence of age and maturation, using magnetic phrenic nerve stimulation in healthy children. Anterolateral magnetic stimulation (MS) of the phrenic nerves was performed using a 43-mm figure-eight coil in 23 children (14 male; mean age, 7.8 years; range, 1.8-15.7) anesthetized for minor surgery with sevoflurane gas. The airway was maintained with a cuffed laryngeal mask airway (LMA) which was briefly occluded during MS. Diaphragm contractility was assessed by measuring the airway pressure (TwPaw) elicited by MS. TwPaw responses were obtained in all subjects, with mean (SD) TwPaw 18.2 (6.8) cm H2O bilateral, 7.3 (3.2) cm H2O left unilateral, and 8.6 (3.1) cm H2O right unilateral. Subgroup analysis was performed in 17 of the children who were prepubertal. Their mean (SD) TwPaw was 17.3 (6.8) cm H2O bilateral, 7.1 (3.7) cm H2O left unilateral, and 8.3 (3.3) right unilateral. The mean (SD) intrapatient coefficients of variation for bilateral and left and right unilateral TwPaw were 8.4% (5.2), 6.7% (3.5), and 11.7% (10.3), respectively. Bilateral and left and right unilateral TwPaw were significantly related to age (P < 0.05). In healthy prepubertal children, diaphragm contractility is primarily influenced by age.

Adolescent↗

Under pressure: choledochal malformation manometry.

PURPOSE: The cause of choledochal (cystic or fusiform) malformation is not known. A favoured hypothesis suggests that abnormal reflux of activated pancreatic secretions via a common pancreatobiliary channel may initiate mucosal injury and mural weakness leading to bile duct dilatation, at normal intraduct pressures. However, bile duct pressures in both normal or disease states are not known in such children. METHODS: Intraoperative choledochal pressure (CP) measurements were made before any other manipulation. Bile was cultured and its amylase content measured. Biochemical liver function (bilirubin, aspartate aminotransferase, gamma-glutamyl transpeptidase, and alkaline phosphatase) was measured. Data were quoted as median (interquartile range). Statistical tests were parametric, where appropriate, and P = .05 was regarded as significant. RESULTS: Twenty-five children (age 2.5 [1.25-5.91] years) with choledochal (cystic [n = 13] and fusiform [n = 12]) malformation coming to surgery were studied. Median CP was 13 (8.5-17) mm Hg. Median bile amylase was 6722 (241-18,000) IU/L. Choledochal pressure inversely correlated with bile amylase (r = -0.60, P = .001), serum aspartate aminotransferase (r = 0.46, P = .01), and log gamma-glutamyl transpeptidase (r = 0.4, P = .04) but not with bilirubin (P = .11), alkaline phosphatase (P = .20), or age (P = .11). No difference in CP, bile amylase, or liver biochemistry could be identified between the 2 biliary phenotypes. All bile cultures were sterile. CONCLUSIONS: Increased CP is inversely related to the level of bile amylase (and hence degree of the functional common channel). This suggests that obstructive stenosis at the level of the pancreatobiliary junction (but not the ampulla) may be a causal factor in a proportion of choledochal malformations.

Alkaline Phosphatase↗

Definitive surgical management of antenatally diagnosed exomphalos.

BACKGROUND/PURPOSE: The management of exomphalos is controversial with many centers in the United Kingdom and elsewhere advocating a conservative nonsurgical approach for the larger examples. Nevertheless, this approach is not without problems or complication. The aim of the study was to ascertain the outcome of all infants with an antenatally diagnosed exomphalos treated recently at our institution using a policy of aggressive abdominal wall closure. METHODS: This is a retrospective review of all infants with exomphalos treated from January 1995 to September 2002. RESULTS: There were 35 infants, all of whom underwent surgery. These were separated into 3 groups: group A (all exomphalos minor) underwent primary closure (n = 11), group B (exomphalos major) underwent primary closure (n = 13), and group C (exomphalos major) underwent staged closure involving a silo (n = 11). Infants in group C had a lower birth weight (P = .05) and were less mature (P = .06). They required longer periods of ventilation (P < .001), a longer hospital stay (P = .001), and a longer period to achieve full enteral feeds (P < .001). Overall survival was 34 (97%) of 35 infants. One premature infant who was born with a ruptured exomphalos sac (birth weight, 862 g) died of nonsurgical complications (sepsis and respiratory failure) early after the creation of a silo. CONCLUSIONS: An aggressive surgical approach in infants with exomphalos is a safe option resulting in effective abdominal wall closure. This requires a skilled multidisciplinary approach and possibly greater resources than other options.

Abdominal Wall↗

Soluble adhesion molecule profiling in preoperative infants with biliary atresia.

OBJECTIVE: Intercellular adhesion molecule (ICAM) expression in liver and bile duct remnant is a feature of the inflammatory component of biliary atresia (BA). Circulating levels of such soluble adhesion molecules (SAM) should reflect intrahepatic disease and would prove a useful adjunct in the evaluation of BA. STUDY DESIGN: Serum ICAM-1 (sICAM-1), serum vascular cell adhesion molecule (sVCAM-1), and serum E selectin (sE-selectin) were measured by enzyme-linked immunosorbent assay in infants with BA at the time of portoenterostomy and stratified by outcome. Results are quoted as medians (range). Binary logistic regression analysis and actuarial survival curves were used to investigate the relationship of SAM profile to outcome. Significance was assumed at P values of < or = .05. RESULTS: Sixty-one infants with BA were treated between 1996 and 2002 and at follow-up; 39 of these were alive and jaundice-free (good outcome, n = 39); 21 had been transplanted, and 1 died (poor outcome, n = 22). Preoperative values for sICAM-1 were 1233 (400-2000) ng/mL; sVCAM-1, 1204 (517-1921) ng/mL; and sE-selectin, 71 (26-192) ng/mL. sVCAM-1 (P < .0001) and sICAM-1 (P < .0001) significantly increased compared with normal control infants, although sE-selectin did not (P = .17). There was a significant correlation of age at surgery with sICAM-1 (r = 0.33, P = .01) but not with sVCAM-1 (r = 0.16, P = .23) or sE-selectin (r = 0.05, P = .70). Binary logistic regression analysis showed that the variables, sICAM-1, sE-selectin, or age at surgery, were not significant predictors of outcome, although sVCAM-1 approached significance (P = .069). A cutoff value for sVCAM-1 of 1380 ng/mL was defined by receiver operating characteristic curve analysis, and the cohort of patients with sVCAM-1 (> 1380 ng/mL) showed a significantly worse actuarial survival (P = .05). CONCLUSION: Levels of sICAM-1 and sVCAM-1 are grossly elevated in BA, whereas sE-selectin levels are normal. Only sVCAM-1 levels have prognostic significance. SAM profiling has the potential to monitor the inflammatory process of BA and may guide more novel forms of pharmacological intervention or immunomodulation.

Biliary Atresia↗

Biliary atresia.

Biliary atresia (BA) remains a devastating disease of infants. It is still a disease of largely unknown etiology although many hypotheses such as an aberrant early bile duct development, perinatal viral infection, aberrant immune response, and abnormalities of bile acids have all been suggested as possibly etiologically important. Although recent studies, using the techniques of molecular biology and immunohistochemistry, have improved the understanding of some of the inflammatory elements of BA, there is a lack of understanding of how many such disparate elements interact and relate. Clinically, the management in the majority of cases should consist of a primary portoenterostomy (Kasai procedure) to try and restore bile flow and alleviate jaundice. Transplantation should be reserved for those who develop chronic liver disease and its attendant complications. Recent series would suggest that over 50% of infants in large centers will be able to clear their jaundice and therefore have a reasonable expectation of long-term survival with a good quality-of-life.

Biliary Atresia↗

Seamless management of biliary atresia in England and Wales (1999-2002).

BACKGROUND: Before 1999, infants born in the UK with suspected biliary atresia were investigated in regional centres, and, if confirmed, a Kasai operation was done there. Since 1999, all infants with suspected biliary atresia in England and Wales, UK, have been referred to one of three designated centres where both the Kasai operation and liver transplantation (if necessary) could be done. METHODS: We assessed clearance of jaundice (bilirubin <20 micromol/L) as an early outcome in all cases of biliary atresia referred from one of the three centres. We then estimated survival using the Kaplan-Meier method with endpoints of liver transplantation or death. FINDINGS: 148 infants with biliary atresia were treated between January, 1999, and June, 2002. A primary portoenterostomy was done in 142 (96%) infants and a primary liver transplant in five (3%). One child died before any intervention. Early clearance of jaundice after portoenterostomy was achieved in 81 of 142 (57%) infants. Liver transplantation was done in 52 (37%) of those undergoing portoenterostomy. 13 (9%) infants died. Of the 135 children who survived, 84 (62%) still have their native liver and 51 (38%) had transplantation. The median follow-up of survivors was 2.13 (range 0.5-4.1) years. The overall 4-year estimated actuarial survival was 89% (95% CI 82-94). The 4-year estimated actuarial survival with native liver was 51% (42-59%). INTERPRETATION: Our early results suggest that surgical outcome can be improved by centralisation of care to supra-regional centres.

Biliary Atresia↗