Split-graft liver transplantation from an adult donor with an unrecognized UCD to a pediatric and adult recipient.

We report the outcomes of an adult and pediatric split liver transplant from an adult male donor who died due to an unrecognized UCD, OTC deficiency. Recognizing inborn errors of metabolism can be challenging, especially in adult centers where such disorders are rarely encountered. Shortage of donors for liver transplantation has led to procedures to maximize donor utilization, such as split and live donor grafts. The cause of death should be ascertained before accepting a cadaveric donor organ.

Increasing liver transplantation waiting list mortality: a report from the Australian National Liver Transplantation Unit, Sydney.

BACKGROUND: We aimed to describe the demand for liver transplantation (LTx) and patient outcomes on the waiting list at the Australian National Liver Transplantation Unit, Sydney over the last 20 years. METHODS: We performed a retrospective analysis with the data divided into three eras: 1985-1993, 1994-2000 and 2001-2008. RESULTS: The number of patients accepted for LTx increased from 320 to 372 and 548 (P < 0.001) with the number of LTx being performed increasing from 262 to 312 and 452 respectively (P < 0.001). The median adult recipient age increased from 45 to 48 and 52 years (P < 0.001) while it decreased in children from 4 to 2 and 1 years respectively (P = 0.001). In parallel, the deceased donor offers decreased from 1003 to 720 and 717 (P < 0.001). Methods to improve access to donor livers have been used with the use of split livers, extended criteria and non-heart beating donors, resulting in increased acceptance of deceased donor offers by 65% and 115% in the second and third eras when compared with the first era (P < 0.001). However, the adult median waiting time has increased from 23 to 41 and 120 days respectively (P < 0.001). This was associated with increased adult mortality on the waiting list from 23 to 40 and 122 respectively (P < 0.001). CONCLUSIONS: Despite the increasing proportion of donor offers being used, the waiting list mortality is increasing. A solution to this problem is an increase in organ donation to keep pace with the escalating demand for LTx.

Reverse portal flow after liver transplantation--ominous or acceptable?

Reversal of portal flow or hepatofugal flow after liver transplantation is a rare complication after liver transplantation. The available reports in the literature suggest that it is an ominous condition that requires immediate operative intervention, failing which prognosis would be grim. We report two children from two different centers who developed hepatofugal flow in the immediate post-operative period after liver transplantation. The possible etiologies in these patients were acute rejection in one and absence of an MHV causing inadequate hepatic venous outflow in the other. Both patients were treated non-operatively with steroids and immunosuppression. Spontaneous reversal to a normal hepatopetal flow occurred in both and the patients continue to be well six months after the transplant. Our experience contradicts the viewpoint that hepatofugal flow equates to mortality in the absence of surgical intervention. It remains to be defined as to which patients with hepatofugal flow will benefit from surgical intervention.

Laparoscopic gastrostomy: the preferred method of gastrostomy in children.

We present a paediatric institutional experience with laparoscopic gastrostomies (LG) and evaluate its appropriateness as the recommended method for gastrostomy placement. We also sought to evaluate the efficacy of a simple technique for LG and collected information on long-term follow-up after LG. LG was performed in 112 children over a 6-year-period. The procedure involves visualization of the stomach through an umbilical port and a second epigastric gastrostomy site to select and anchor the stomach with sutures prior to the placement of a low profile gastrostomy feeding device (LPGD). The follow-up details of the patients were analysed. A review of literature was done to compare LG with percutaneous endoscopic gastrostomy (PEG). The median operating time for the procedure in 112 patients was 48 min. There was one open conversion. Median postoperative length of stay was 6 days. Other complications were vomiting (11%), peri- gastrostomy leak (26%), granulation tissue (42%), accidental dislodgement of the LPGD (4%), faulty device requiring replacement (10%), gastric mucosal prolapse (2%) and localized infection (2%). Follow-up ranged from 6 to 75 months with a cumulative gastrostomy usage of 2,352 months. The advantages of the described technique are virtual feasibility in all patients, primary placement of a LPGD, simplicity with requirement of minimal laparoscopic expertise and safety. Comparison with reports of PEG in the literature indicates that LG should be the preferred method of gastrostomy placement in children.

Routine contralateral orchiopexy for children with a vanished testis.

AIM: The aim was to determine whether routine contralateral orchiopexy is justified in a child with a vanished testis from intrauterine torsion. METHODS: A retrospective study of thirty-one consecutive boys with a vanished testis who underwent contralateral orchiopexy with the sutureless technique over an eight-year (1995 - 2002 inclusive) period was carried out. Operative findings were analyzed. All patients have been followed to date and interviewed by telephone. RESULTS: Of the thirty-one patients, 22 had testicular abnormalities (71 %). Five patients (16 %) had abnormalities that could predispose them to metachronous torsion. These included bell clapper deformity (3), horizontal lie (1), and ectopic testis (1). Other abnormalities were abnormal epididymal-testicular fusion (2), hydrocele (2), and one testis did not show compensatory hypertrophy. CONCLUSIONS: Sixteen percent of patients had testicular abnormalities that could predispose them to metachronous torsion. There was no morbidity from the operation and no post-orchiopexy torsion on follow-up. Therefore, routine contralateral orchiopexy in a boy with a vanished testis appears to be safe and effective. A large multicenter trial should be done to investigate these preliminary findings. If confirmed, contralateral orchiopexy should be advocated.

Pulmonary hypertension--a new manifestation of mitochondrial disease.

Mitochondrial respiratory chain (RC) abnormalities in children can present as multiorgan disease, including liver failure, usually within the first year of life. Cardiorespiratory complications have previously been described in association with RC defects; however, to our knowledge no cases of pulmonary hypertension have been described. We discuss two patients with proven mitochondrial RC liver disease who developed severe pulmonary hypertension, one subsequent to cadaveric orthotopic liver transplantation, the second in the neonatal period. It is our contention that pulmonary hypertension should now be included as another potential manifestation of paediatric mitochondrial disease.

Biliary dyskinesia: is the problem with Oddi?

We report our experience of cholecystectomy for treating symptoms suggestive of biliary disease in association with a decreased gallbladder ejection fraction (GBEF) but without evidence of cholelithiasis. Five children with normal biliary ultrasounds were evaluated between January 1990 and December 2000 for recurrent upper abdominal pain. Based on a cholecystokinin (CCK)-provoked GBEF of less than 50% and the absence of any other gastrointestinal pathology, patients underwent cholecystectomy with operative cholangiography for presumed biliary dyskinesia. Pathological examination demonstrated chronic inflammation in all cases. Two patients had complete resolution of their symptoms, but three had persistent pain following surgery. Biliary dyskinesia seems an uncommon cause of persistent abdominal pain in childhood. Cholecystectomy was not always effective in relieving symptoms. Biliary scintigraphy with CCK provocation should not be used as the sole criterion for cholecystectomy. Sphincteric manometry may be valuable in the assessment of this small group of patients to avoid inappropriate intervention. The future perhaps lies in better understanding of the physiological action and pharmacological control of the sphincter of Oddi.

Pancreatic trauma in children.

BACKGROUND/PURPOSE: The aim of this study was to determine the etiology, associated injuries, and outcome of children with pancreatic injuries. METHODS: A retrospective review was conducted of children under 16 years with pancreatic trauma admitted to the Children's Hospital at Westmead (CHW) from January 1983 to September 2002. Deaths were reported to the New South Wales Paediatric Trauma Death Registry (State Registry) from January 1988 to September 2002. RESULTS: Sixty-five cases were identified: 46 patients were admitted to CHW, and 22 deaths were reported to the State Registry (including 3 deaths at CHW). The median age was 6 years (range, 1 to 14 years). Boys accounted for 60% (n = 40) of cases, decreasing to 50% (n = 11) of those that died. Motor vehicle injuries (MVI) were the most common mechanism, accounting for 40% of survivors and 77% of those who died. Children were restrained incorrectly in 48% of all cases and in 67% of those who died. Significantly more children who died had head, chest, and abdominal visceral injuries, compared with those who survived. Death occurred as a result of head injuries in 68%, with only 3 deaths directly attributed to pancreatico-duodenal injuries. CONCLUSIONS: Pancreatic injuries remain uncommon in children. The most frequent mechanism was MVI, with incorrect use of passenger restraints an important contributing factor. Whereas mortality was mainly caused by other injuries, 3 deaths were directly attributable to pancreato-duodenal trauma.

Small bowel perforation in the premature neonate: congenital or acquired?

To determine the potential aetiological factors of small bowel perforation in the premature neonate, we performed a retrospective chart review of those neonates with spontaneous intestinal perforation (SIP) of the small bowel seen in our tertiary paediatric hospital between January 1980 and December 2000. Data were collected on gestational feto-maternal health, medical interventions prior to perforation and the subsequent operative and laboratory findings. There were 23 patients with SIP of the small bowel over the 21-year review; 65% were male. There were 7 twin pregnancies but no cases linked to maternal drug abuse. The median gestational age was 27 weeks, the median birth weight 973 g, 19 neonates required ventilation, 15 steroids and 13 indomethacin. The median age at diagnosis was 7 days, heralded by rapid development of abdominal distension in 22 patients. Surgical intervention in addition to insertion of a peritoneal drain was required in 19 patients. Positive microbiological cultures of blood or peritoneal fluid at operation were documented in 8 patients; 5 grew Staphylococcus epidermidis and 4 Candida species. Perforations were located in the ileum in 20 and the jejunum in 1. Deficiency of the muscularis propria was found in 6 patients. Of the 6 deaths, 2 neonates had significant co-morbidity in addition to extreme prematurity. Small bowel SIP occurs in the premature neonate after the first week of life and usually presents with abdominal distension. Putative risk factors identified included twin gestation, neonatal ventilation, use of steroids and indomethacin, infection with Staphylococcus epidermidis and Candida species and deficiency of enteric smooth muscle.

A new technique of anastomosis to avoid stricture formation in oesophageal atresia.

Anastomotic stricture is a common problem following repair of oesophageal atresia (OA). We describe a technique of oesophageal anastomosis that may prevent this problem. A horizontal incision is placed on the anterior hemicircumference of the upper pouch approximately 0.5 cm proximal to its blind ending to raise a flap. A corresponding vertical incision is made at the open end of the lower pouch to spatulate it. The flap from the upper pouch is laid into the open V of the lower pouch. This creates a wide anastomosis, and the suture line is not restricted to one plane. In 11 cases of OA, oesophageal continuity was established with this technique over a period of 10 years. Only 1 child developed an anastomotic stricture, which responded to a single dilatation. Two patients required Nissen's fundoplication for a distal oesophageal stricture. In neither of the patients did the anastomosis become stenotic. The technique described here is simple and effective. A suture line is created that is long and not in a single plane. This minimises the risk of stricture formation.

Intra-operative pulse oximetry can help determine intestinal viability.

The intra-operative assessment of intestinal viability when dealing with ischaemic bowel remains a challenge. Reliable healing of small-bowel anastomoses, using pulse oximetry to exclude critical ischaemia, has been shown in the canine model. In this study, intra-operative pulse oximetry (PO) was used to help determine intestinal viability 48 h after de-torsion of a volvulus. Approximately one-half of the ischaemic, volved bowel was able to be preserved. Intra-operative PO can thus help preserve bowel of doubtful viability.

Inferior liver allograft survival from cadaveric donors >50 years of age?

The growing imbalance between the number of cadaveric organ donors and recipients has led to an increasing use of high-risk donors as an option to expand the donor pool. The aim of this study was to evaluate our experience with the use of older liver (donor>50 yr of age) allografts. The medical records, postreperfusion biopsies and laboratory results were reviewed of the 393 patients who underwent orthotopic liver transplantation between 1986 and 1997. The outcome of the 61 patients who received older livers (OL) was compared to that of the other 332 recipients. Increasing use of OL was evident from 1992 onwards. Recipients of OL were older than recipients of younger livers (YL, p<0.001) and more commonly had underlying chronic viral hepatitis (CVH) or fulminant hepatic failure (p<0.05). Patient and allograft survival were only slightly less in recipients of OL versus YL (p=NS). Although postperfusion biopsies showed more damage in OL than YL allografts (p<0.05), this was not associated with increased primary graft failure. OL allografts can be transplanted with acceptable results into recipients without the concern of early allograft loss. SUMMARY OF ARTICLE: This report of one centre's experience with 61 recipients of older donor liver allografts identifies recipient factors that may also have a negative impact on allograft outcome. These factors include a diagnosis of either CVH or fulminant hepatic failure at the time of transplantation. Postreperfusion biopsies of older donor allografts tend to show more damage, but this is not associated with primary non-function.

Gallstones and common bile duct calculi in infancy and childhood.

BACKGROUND: Gallstones and common bile duct calculi have been increasingly diagnosed in recent years in infants and children. The present study aims to review the spectrum of this disorder in the last two decades. METHODS: During the period 1979-96 a total of 102 consecutive infants and children were diagnosed in Royal Alexandra Hospital for Children with gallstones or common bile duct calculi. A detailed retrospective analysis and follow-up of these children form the basis of the present report. RESULTS: The median age at presentation was 10 years. Recurrent right upper quadrant pain was the most common clinical presentation. The male-to-female ratio was 3:2 and this male predominance was noted in all the age groups. Aetiologically three identifiable groupings were noted: idiopathic disease (n = 66), haematological diseases (n = 23) and specific non-haematological disease (n = 13). The incidence of idiopathic and haematological stones had increased two-fold in the second half of the study. The majority of children (86%) underwent surgical correction. Choledocholithiasis (CDL) was noted in 18 children (18%). Jaundice was commonly associated with abdominal pain in this group. A higher incidence of common bile duct calculi was noted in females and children less than 5 years of age (P < 0.01). Common bile duct calculi were accurately diagnosed by pre-operative imaging in all 18 children. Surgical correction was required in all except two. CONCLUSIONS: The present study suggests an increasing incidence of gallstones in children. Cholelithiasis in children occurs commonly in boys, is idiopathic in aetiology and presents with a vague right upper quadrant pain. Choledocholithiasis is not uncommon in children, occurs more commonly in girls aged < 5 years and presents with jaundice or abnormal liver function tests.

Diverticular rectal duplication with heterotopic gastric mucosa in a child: a rare cause of rectal bleeding.

This report describes a rare case of rectal bleeding and proctalgia in a child due to diverticular duplication of rectum associated with heterotopic gastric mucosa.

Unusual biliary scan appearance in a child with a transplanted liver with hepatic arterial thrombosis: a case report.

A 5-year-old girl with biliary atresia and a subsequent Kasai procedure is described. She had clinical symptoms suggestive of rejection after a recent orthotopic liver transplant A hepatobiliary scan showed partial hepatic infarction and a biloma in the infarcted area.

Adverse outcome of congenital diaphragmatic hernia is determined by diaphragmatic agenesis, not by antenatal diagnosis.

BACKGROUND/PURPOSE: The authors studied their congenital diaphragmatic hernia (CDH) cases retrospectively to ascertain if classical CDH and diaphragmatic agenesis (DA) have separate clinical manifestations, whether antenatally diagnosed cases behave differently from those not diagnosed antenatally, and if antenatal diagnosis before 25 weeks carries a worse prognosis. METHODS: The authors performed a retrospective review of 23 infants with CDH treated at their institution between January 1996 and March 1999. The patients were divided into 3 groups that were analyzed: DA and classical CDH, antenatally diagnosed and nonantenatally diagnosed, and antenatally diagnosed before 25 weeks and after 25 weeks. RESULTS: There were 8 cases of DA and 11 cases of classical CDH. Four infants died without operation and could not be classified. Neonates with DA had significantly longer mean duration of preoperative stabilization compared with classical CDH (5.25+/-2.76 days v 1.36+/-1.0 days) and postoperative mechanical ventilatory support (15.7+/-3.0 days v 4.9+/-3.0 days). Fifty percent of DA patients died; all classical CDH patients survived. Twelve cases were diagnosed antenatally, 6 before 25 weeks' gestation. Antenatally diagnosed cases had no statistically significant difference in mortality rates from those not diagnosed antenatally; 50% of those diagnosed before 25 weeks survived. CONCLUSIONS: DA cases require more preoperative preparation and postoperative ventilation and have a bad prognosis compared with classical CDH. Antenatal diagnosis of CDH does not convey a different prognosis. Fifty percent of CDH patients with antenatal diagnosis before 25 weeks survive.

ABO blood group incompatibility in liver transplantation: a single-centre experience.

BACKGROUND: For most organ transplantation (Tx), ABO blood group incompatibility (ABOI) is an absolute contraindication because of the high incidence of hyperacute rejection (HAR). While HAR occurs in ABOI liver Tx (LTx), it is known that some liver grafts can be accepted. METHODS: ABO-incompatible (ABOI) liver allografts were used in seven of 355 orthotopic LTx operations performed at our institution over a 10-year period. All seven recipients were in fulminant hepatic failure (FHF) prior to Tx. RESULTS: Following Tx, all grafts functioned immediately. One patient died without recovering consciousness. Six patients recovered consciousness following Tx but three patients subsequently required re-transplantation (with ABO-compatible grafts (ABOC)) because of severe acute rejection (2) and chronic rejection (1). Hyper-acute rejection did not occur. All six patients are now well, with a mean survival of 61.5 months. When compared to 36 other FHF patients who received ABOC grafts, graft survivals were 3/7 (43%) for ABOI versus 23/36 (64%) for ABOC (P = not significant (NS)). Patient survivals were 6/7 (85.7%) for ABOI patients and 23/36 (64%) for ABOC (P = NS). The re-transplantation rate was significantly higher in the ABOI group (P = 0.001). CONCLUSIONS: The results confirm that ABOI liver grafts should be used in urgent circumstances when compatible grafts are not available. Some grafts function indefinitely, while those that fail may function for sufficient time to allow successful retransplantation with ABOC grafts.

Portosystemic shunting for paediatric portal hypertension.

The records of 22 patients who received portosystemic shunting for portal hypertension from 1985 to 1995 inclusive at the Royal Alexandra Hospital for Children (RAHC) were retrospectively reviewed. There were 11 girls and 11 boys. The average age at operation was 8 years, 3 months (range, 2 years, 3 months to 16 years, 7 months). The aetiology was idiopathic portal cavernomatous transformation (n = 9), billiary atresia (n = 4), cystic fibrosis (n = 3), documented neonatal portal vein thrombosis (n = 3), congenital hepatic fibrosis (n = 2), and portal vein obstruction after liver transplant (n = 1). The major presenting problem was upper gastrointestinal haemorrhage. Two patients had recurrent melaena from Roux-en-Y jejunal loop and caecal varices, respectively. Before receiving shunts, 12 patients had endoscopic sclerotherapy, 1 had gastric transection, and 2 had gastric varices oversewn. Portal pressure at preoperative splenoportogram averaged 28 mm Hg (range, 20 to 41). Urgent shunts were performed on 13 patients. Two disadvantaged patients had prophylactic shunts for severe hypersplenism. The types of shunts used were reversed splenorenal (n = 13), splenoadrenal (n = 6), inferior mesenteric renal (n = 1), portocaval (n = 1), inferior mesenteric caval (n = 1), and superior and inferior mesenteric caval (n = 1). In all, 22 patients had 23 shunts. The patency rate was 96% on 6 months to 10 years follow-up (average, 5.8 years). No spleen was lost. There were 2 late deaths. Two cystic fibrosis patients and one child with extrahepatic portal hypertension experienced post-shunt encephalopathy. Three patients rebled in the early postoperative period despite a patent shunt. Two patients subsequently received liver transplantation without any additional difficulties. Thus, portosystemic shunting using a method appropriate for the patient is a reliable option for treating children with portal hypertension in whom variceal sclerotherapy is inappropriate or has failed.