RESUMO
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.
Assuntos
Transplante de Fígado , Doença da Deficiência de Ornitina Carbomoiltransferase/diagnóstico , Complicações Pós-Operatórias/diagnóstico , Adulto , Criança , Evolução Fatal , Feminino , Humanos , Transplante de Fígado/métodos , Masculino , Doença da Deficiência de Ornitina Carbomoiltransferase/etiologiaRESUMO
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.
Assuntos
Síndrome de Alagille/cirurgia , Atresia Biliar/cirurgia , Circulação Hepática , Transplante de Fígado/métodos , Veia Porta/fisiopatologia , Anastomose em-Y de Roux , Feminino , Humanos , Lactente , Doadores Vivos , Veia Porta/diagnóstico por imagem , UltrassonografiaRESUMO
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.
Assuntos
Transplante de Fígado/mortalidade , Listas de Espera/mortalidade , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Austrália/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Hepatopatias/mortalidade , Hepatopatias/cirurgia , Transplante de Fígado/tendências , Masculino , Pessoa de Meia-Idade , New South Wales/epidemiologia , Estudos Prospectivos , Estudos Retrospectivos , Doadores de Tecidos , Adulto JovemRESUMO
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.
Assuntos
Criptorquidismo/cirurgia , Testículo/anormalidades , Procedimentos Cirúrgicos Urológicos Masculinos , Criança , Pré-Escolar , Humanos , Lactente , Masculino , Técnicas de SuturaRESUMO
Carbon monoxide (CO) inhalation leads to cerebral, cardiac, and, more rarely, liver damage. The use of liver allografts from CO poisoned donors with evidence of liver damage has not previously been reported. In this report we describe two recipients, both in fulminant hepatic failure, who received liver grafts from such donors. One donor had markedly abnormal liver function tests (LFTS), and in the other LFTS were mildly abnormal. In both, the liver appeared normal at procurement. There was satisfactory early function of both allografts, although marked patchy necrosis was seen on the postreperfusion biopsy (case 1), and on a 10 day postoperative biopsy (case 2). In both cases the changes were considered to be related to damage sustained from CO inhalation. Both allografts soon achieved normal function and both recipients are well. We conclude that CO poisoning can cause liver damage that can recover completely following liver transplantation.
Assuntos
Intoxicação por Monóxido de Carbono/complicações , Transplante de Fígado/estatística & dados numéricos , Adulto , Cadáver , Feminino , Humanos , Doadores de TecidosRESUMO
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.
Assuntos
Hipertensão Portal/cirurgia , Derivação Portossistêmica Cirúrgica , Adolescente , Criança , Pré-Escolar , Varizes Esofágicas e Gástricas/etiologia , Feminino , Hemorragia Gastrointestinal/etiologia , Humanos , Hipertensão Portal/complicações , Masculino , Auditoria Médica , Seleção de Pacientes , Derivação Portossistêmica Cirúrgica/efeitos adversos , Complicações Pós-Operatórias , Estudos RetrospectivosRESUMO
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.
Assuntos
Diafragma/anormalidades , Hérnia Diafragmática/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas , Ultrassonografia Pré-Natal , Diafragma/diagnóstico por imagem , Feminino , Hérnia Diafragmática/mortalidade , Hérnia Diafragmática/cirurgia , Humanos , Masculino , Gravidez , Prognóstico , Estudos Retrospectivos , Sensibilidade e Especificidade , Análise de Sobrevida , Fatores de Tempo , Resultado do TratamentoRESUMO
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.
Assuntos
Sistema Biliar/diagnóstico por imagem , Artéria Hepática , Transplante de Fígado/diagnóstico por imagem , Trombose/diagnóstico por imagem , Bile , Atresia Biliar/cirurgia , Pré-Escolar , Feminino , Humanos , Cintilografia , Trombose/etiologiaAssuntos
Transplante de Fígado , Doadores de Tecidos/provisão & distribuição , Obtenção de Tecidos e Órgãos , Acidentes de Trânsito/mortalidade , Adolescente , Adulto , Idoso , Austrália , Morte Encefálica , Causas de Morte , Transtornos Cerebrovasculares/mortalidade , Criança , Pré-Escolar , Hepatectomia/métodos , Humanos , Pessoa de Meia-Idade , Nova Zelândia , Obtenção de Tecidos e Órgãos/estatística & dados numéricos , Ferimentos e Lesões/mortalidadeAssuntos
Transplante de Fígado/métodos , Transplante de Fígado/fisiologia , Derivação Portossistêmica Transjugular Intra-Hepática , Adolescente , Adulto , Varizes Esofágicas e Gástricas/cirurgia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de TempoRESUMO
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.
Assuntos
Gastrostomia/métodos , Laparoscopia/métodos , Gastropatias/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Tempo de Internação , Masculino , Estudos Retrospectivos , Resultado do TratamentoRESUMO
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.
Assuntos
Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/patologia , Doenças Mitocondriais/diagnóstico , Trifosfato de Adenosina/metabolismo , Transporte de Elétrons , Evolução Fatal , Fibroblastos/metabolismo , Humanos , Hipertensão Pulmonar/mortalidade , Lactente , Recém-Nascido , Fígado/metabolismo , Hepatopatias/metabolismo , Falência Hepática , Transplante de Fígado , Masculino , Mitocôndrias/metabolismo , Doenças Mitocondriais/mortalidade , Doenças Mitocondriais/patologia , Pele/metabolismo , Fatores de TempoRESUMO
A 35-year retrospective review of all surgically significant pathological lesions of the ovary was conducted. Fifty-one children had 53 ovarian lesions which required surgical intervention. Of the 53 ovarian lesions seen in this series, 21 had presented with torsion. Five of these torted ovaries were microscopically normal. Three children were castrated. One of these castrations could have been prevented if contralateral oophoropexy was done. In addition, one child, who had lost an ovary previously because of torsion, presented with major cystic disease of the remaining ovary. Torsion was aborted by ovarian cystectomy. Contralateral oophoropexy at the time of ipsilateral oophorectomy is recommended to prevent castration.
Assuntos
Infarto/cirurgia , Doenças Ovarianas/prevenção & controle , Ovário/irrigação sanguínea , Doença Aguda , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Doenças Ovarianas/cirurgia , Ovariectomia/métodos , Estudos Retrospectivos , Anormalidade TorcionalRESUMO
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.
Assuntos
Intestino Delgado/irrigação sanguínea , Complicações Intraoperatórias/diagnóstico , Intussuscepção/cirurgia , Isquemia/diagnóstico , Oximetria/instrumentação , Anastomose Cirúrgica , Feminino , Humanos , Lactente , Intestino Delgado/fisiopatologia , Complicações Intraoperatórias/fisiopatologia , Complicações Intraoperatórias/cirurgia , Intussuscepção/fisiopatologia , Isquemia/fisiopatologia , Isquemia/cirurgia , Curativos Oclusivos , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/cirurgia , ReoperaçãoRESUMO
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.
Assuntos
Anastomose Cirúrgica/métodos , Atresia Esofágica/cirurgia , Complicações Pós-Operatórias/prevenção & controle , Criança , Constrição Patológica , Esôfago/patologia , Fundoplicatura , Humanos , Estudos Retrospectivos , Técnicas de SuturaRESUMO
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.
Assuntos
Colelitíase/cirurgia , Cálculos Biliares/cirurgia , Adolescente , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Colecistectomia , Colelitíase/complicações , Colelitíase/diagnóstico por imagem , Colelitíase/patologia , Terapia Combinada , Feminino , Cálculos Biliares/complicações , Cálculos Biliares/diagnóstico por imagem , Cálculos Biliares/patologia , Humanos , Lactente , Masculino , Estudos Retrospectivos , Resultado do Tratamento , UltrassonografiaRESUMO
A prospective study to evaluate the efficacy of human amniotic membrane dressings in the treatment of chronic leg ulcers was conducted on 15 patients. Patients with two ulcers were selected. One ulcer was treated with human amnion and the other, treated with standard dressings, served as a control. The rate of healing of the two ulcers was observed and compared. Chronic ulcers of post-phlebitic, post-irradiation and post-burn origin responded to treatment with human amnion and healed more rapidly than the control ulcer. Ulcers associated with rheumatoid arthritis and vasculitis showed no improvement. It was observed that all ulcers treated with human amnion quickly became pain-free.