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ABSTRACT: Methylmalonic acidemia (MMA) is a very rare genetic disease of metabolism that progressively leads to neurological and renal sequelae. This report describes an unusual case of a patient with MMA who developed severe hyperkalemia and severe dysrhythmia during anesthesia. A 13-month-old male infant with MMA underwent urgent insertion of a port-a-cath under general anesthesia. A life-threatening arrhythmia suddenly occurred, with severe hyperkalemia (up to 7.4 mmol/L), immediately following induction of anesthesia. Emergent resuscitation was successfully carried out, with a complete neurological recovery after 7 days after surgery. Although MMA is a rare complication, the possibility of severe hyperkalemia should be considered in the differential diagnosis of patients with MMA presenting with wide QRS complex tachycardia. The management and intraoperative complications of this disorder are reported here, and the available literature is reviewed.
Journal of the Chinese Medical Association 05/2012; 75(5):243-5. · 0.79 Impact Factor
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ABSTRACT: Although the lateral segment (LS) from the split-liver of a deceased donor or a live donor can increase the organ pool of pediatric patients awaiting liver transplantation, the shortage of organ donation in Asia countries is still serious and results in high death rates of pediatric patients. The medial segment (MS) of the liver is sacrificed during the standard technique of splitting a whole liver into an LS and an extended right liver because the cutting sites of portal vein, hepatic artery and bile duct are all in the bifurcation of the liver hilum to have adequate length of vascular and biliary pedicles for easier grafting. However, the surgical techniques of vascular and biliary reconstructions for liver transplantation, particularly from the experiences of living donor liver transplantation, have been much improved in the last decade. Therefore it may be possible for an additional MS of the liver to be an isolated graft for a small recipient on the premise that grafts of right lobe (RL) and LS are minimally injured. In light of detailed reviews of anatomies of hepatic arteries, hepatic veins, portal veins and bile ducts, the dissection and reconstruction of vessels and bile ducts for the MS can possibly be performed if the extra-hepatic length of the artery to the MS is long enough. As the artery for the MS, middle hepatic artery (MHA), usually derives from a branch of the left hepatic artery and often in the liver parenchyma, the length is usually too short to be reconstructed. If the MHA for the MS is isolated and its extra-hepatic length is more than 1cm, triple liver grafts from a deceased whole liver, consisting of the RL, MS and LS may be possible. The anatomies of the hepatic artery in abdominal computed tomography (CT) or magnetic resonance imaging (MRI) for live liver donors in our institution were retrospectively analyzed. The results showed that three types of hepatic arterial anatomies could be considered for possible recovery of triple segments: type I is an accessory left gastric artery to feed the lateral segment; type II is an isolated MHA; type III is an early bifurcation of the left hepatic artery and MHA.
Medical Hypotheses 03/2012; 78(5):668-71. · 1.39 Impact Factor
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ABSTRACT: DJS is an autosomal recessive disorder that causes an increase in conjugated bilirubin without elevation of liver enzymes. Most patients are asymptomatic and have normal life spans, but to the best of our knowledge, their livers have never been reported to be grafts in liver transplantation. Herein, we report an infant patient with MMA that received a partial liver graft from his mother, who had DJS. A biliary anastomosis stricture was noted five months after transplantation and was successfully treated with radiological interventions. Otherwise, the patient's liver functions were normal, and a liver biopsy showed a pathognomonic picture of DJS nine months after the transplantation. The patient was followed for one yr, and the results were satisfactory for an increase in oral intake and protein uptake, no recurrence of metabolic stroke and there was a gradual catch-up with regard to physical development despite having a persistently abnormal profile of amino acid analysis. From the experience of our case, we suggest that a liver from a donor with DJS can be used as a graft for liver transplantation, although long-term follow-up is mandatory to examine the grafted liver under the use of immunosuppressive medications.
Pediatric Transplantation 02/2012; 16(1):E25-9. · 1.48 Impact Factor
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ABSTRACT: Retrograde arterial flush (RGAF) of liver grafts, which prevents arterial intima injuries in living donor liver transplantation (LDLT), seemed to reduce post-transplantational cholestasis by our preliminary study. The aim of this study was to further confirm this effect of RGAF of grafts in LDLT by a prospective randomized study. The enrolled cases were randomly divided into two groups, RGAF (portal flush plus RGAF) and non-RGAF (portal flush only). The outcome measures included intra-operative hemodynamic changes, one-month post-transplantational liver function tests and acute cellular rejection, vascular and biliary complications, the length of postoperative hospital stay, and graft and patient survivals. The significant findings of results were lower postoperative serum bilirubin in the RGAF group until three wk after transplantation (days 1, 3, 7, 14, and 21 serum total bilirubin, mg/dL: 5.82±5.46, 3.95±4.28, 4.87±5.01, 3.62±5.32, and 3.63±6.91 vs. 8.41±5.49, 7.47±5.61, 10.0±10.1, 9.06±11.6, and 6.02±9.84, respectively, in RGAF and non-RGAF, p=0.017, 0.002, 0.014, 0.011, 0.109) and shorter postoperative hospital stay in the RGAF group (31.1±17.6 vs. 44.8±32.3 d, respectively, in RGAF and non-RGAF, p=0.035). In conclusion, the RGAF of liver grafts on a back table may ameliorate postoperative functional cholestasis in LDLT, which possibly reduces postoperative hospital stay.
Clinical Transplantation 08/2011; 26(2):305-13. · 1.67 Impact Factor
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World Journal of Surgery 08/2011; · 2.36 Impact Factor
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ABSTRACT: This study was designed to evaluate the possibility of avoiding small-for-size syndrome (SFSS) in living donor liver transplantation (LDLT) by increasing the donor's body weight (BW) before liver donation.
Nineteen participants, including 15 volunteers and 4 liver donors, were enrolled in this study to increase their BW by 1.5-5 kg within 3 months by eating a high-protein and high-carbohydrate diet according to a flexible formula to increase calorie intake.
Fifteen participants, including 12 volunteers and 3 live liver donors, successfully increased their BW by 1.5-5 kg within 3 months (failure rate, 21%). The actual liver weight (LW) gain was more than the expected LW gain using the formula for calculating standard liver volume (2.77- to 8.94-fold; median, 4.49-fold; mean, 4.45-fold, P < 0.005) and using the ratio of liver weight to body weight (1.36- to 4.49-fold; median, 2.01-fold; mean, 2.06-fold, P < 0.005). The enlargement of the livers was symmetrical without significant fatty change. The graft-versus-recipient weight ratio increased 0.17%, 0.07%, and 0.08%, respectively, for the three live liver donors and successful LDLTs were performed.
By having liver donors eat a high-protein, high-carbohydrate diet to increase their BW in a short period of time, the actual LW may increase more than the expected LW gain without fatty change. This method of increasing LW may be used in selected cases of LDLT to avoid SFSS.
World Journal of Surgery 10/2010; 34(10):2401-8. · 2.36 Impact Factor
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ABSTRACT: Haddad syndrome is characterized by congenital central hypoventilation syndrome (Ondine's curse) associated with segmental distal gut aganglionosis (Hirschsprung's disease). The prognosis of Haddad syndrome is very poor, and survival is often less than 2 years. Treatment of Hirschsprung's disease is usually influenced by the association with Ondine's curse. We report the case of a girl with Haddad syndrome who underwent redo pull-through with Duhamel's method because of persistent obstruction after primary transanal pull-through surgery. After 7 years of follow-up, the patient is alive and does not suffer from recurrent partial intestinal obstruction. She performs her daily activities with the support of a portable ventilator. Thus, aggressive surgical treatment for Hirschsprung's disease could have good outcome in terms of long-term survival in Haddad syndrome patients.
Journal of the Chinese Medical Association 08/2010; 73(8):438-40. · 0.79 Impact Factor
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Liver Transplantation 11/2009; 15(11):1655. · 3.39 Impact Factor
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ABSTRACT: HFH is a metabolic disease caused by a defect in the gene that encodes the synthesis of the cellular receptor for LDL-Rs. A high plasma level of cholesterol is present from birth and leads to severe atherosclerosis in childhood, and death from myocardial infarction usually occurs before the age of 20 yr. The liver contains approximately 50-75% of the total body LDL-Rs; therefore, liver transplantation has been carried out to treat this metabolic disorder effectively. The rationale for using an HFH liver for a domino graft is that the absence of functional LDL-Rs in the liver may be compensated for by the extra-hepatic LDL-Rs. Therefore, an HFH liver can possibly be used as a domino graft for a recipient with a normal plasma cholesterol level before transplantation. We herein report a domino liver transplantation using an HFH liver as a domino graft with successful results. To expand the donor pool, especially in Asian countries, domino grafts from HFH should be encouraged by careful selection of the domino recipient.
Pediatric Transplantation 03/2009; 14(3):E30-3. · 1.48 Impact Factor
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ABSTRACT: The feasibility of D-D biliary reconstruction in pediatric LDLT using left-lobe graft has been discussed in few reports. The use of a trans-anastomotic biliary tube seemed to be the favorable method to avoid the complications according to these reports. We had performed left-lobe LDLT for seven pediatric cases and D-D was done originally. Three cases were converted to R-Y hepaticojejunostomy due to radical resection of hepatoduodenal ligament (n = 1) and severe kinking of D-D (n = 2). Four cases received D-D using 6-0 PDS interrupted sutures without external stent tube. One D-D case died of intra-cerebral hemorrhage 10 days after operation with a functioning graft. There was one biliary leakage in a D-D patient who required PTCD stent for 4 months without any sequale. From our limited experience, D-D biliary reconstruction without external stent tube in left-lobe LDLT is feasible in certain pediatric cases having normal extra-hepatic bile ducts. In smaller recipient with larger graft, the use of a trans-anastomotic biliary tube can prevent anastomotic kinking although we suggest R-Y biliary reconstruction is better for this condition.
Pediatric Transplantation 12/2008; 13(6):693-6. · 1.48 Impact Factor
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ABSTRACT: It is difficult to reconstruct the hepatic venous outflow in LDLT for the case of BCS, especially when the thrombosis extends to the retro-hepatic and supra-hepatic vena cava. We reported on a technique of diamond-shape venoplasty which successfully treated such a case. The venoplasty used autologous vein grafts to overcome the size discrepancy between the orifices of supra-diaphragmatic inferior vena cava and hepatic vein of the liver graft.
Pediatric Transplantation 10/2008; 13(1):35-8. · 1.48 Impact Factor
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Liver Transplantation 10/2008; 14(9):1378-9. · 3.39 Impact Factor
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Surgery 08/2007; 142(1):123-4. · 3.10 Impact Factor
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ABSTRACT: Although using a Kamada model with a cuff approach for vascular anastomosis shortens the anhepatic time in orthotopic rat liver transplantation (ORLT), cardiac arrest is still often encountered during the anhepatic phase. We hypothesize that low cardiac output is the reason for this. In this study, we used the supra-celiac aorta clamp (SCAC) method during the anhepatic phase in ORLT to evaluate the success rate.
Spraque-Dawley rats were used in this the study. First, we tested the time between the clamping of the supra-hepatic inferior vena cava (IVC) clamped and cardiac arrest in between two groups according to whether SCAC was performed (n = 40) or not (n = 20). Clamping of the supra-hepatic IVC was performed 30 s or 1 min after SCAC (n = 20 for each) in the SCAC group. Second, we performed the Kamada method for ORLT in two groups according to whether SCAC was performed (n = 20, 1-min SCAC) or not (n = 20). Successful ORLT was defined as a recipient rat surviving more then 5 days.
The mean time between IVC clamping and cardiac arrest was longer in the group of 1-min SCAC group than in the 30-s SCAC group or without SCAC (19.8 versus 11.45 versus 3.87 min, respectively, P < 0.001). The success rate of ORLT was higher in the SCAC group than in the group without SCAC (85% versus 15%, respectively, P < 0.001).
SCAC can effectively increase the success rate of ORLT by increasing the tolerable time during the anhepatic phase. The reason for this is the avoidance of hypovolumic cardiac arrest during the anhepatic phase.
Journal of Surgical Research 11/2006; 136(1):116-9. · 2.25 Impact Factor
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ABSTRACT: Esophageal atresia (EA), tracheoesophageal fistula (TEF), or both is a complicated problem. The purpose of this study was to evaluate the outcomes and postoperative complications in patients with EA/TEF who were admitted to our hospital.
In total, 15 patients were enrolled from 1994 to 2003, including 8 males and 7 females. Patient demographics, associated anomalies, and outcomes were analyzed.
The most common variant was EA with a distal TEF (type C), which occurred in 12 patients (80%). The latter had associated congenital anomalies, and cardiac anomalies were the most frequent, occurring in 8 patients (53.3%). Of the 6 cases who had life-threatening anomalies, 4 (66.7%) died, and of the 9 cases who had no life-threatening-anomalies, 2 (22.2%) died. Tracheomalacia and/or stenosis were diagnosed in 8 patients (66.7%) postoperatively. Though 3 of the 4 cases who suffered from dying spell received intratracheal stent implantation, 2 cases still died.
The survival rate of the patients with EA/TEF is influenced mainly by associated life-threatening anomalies. TMS combined with a history of dying spell may be the major fatal complication.
Journal of the Chinese Medical Association 08/2006; 69(7):317-21. · 0.79 Impact Factor
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ABSTRACT: Stoma prolapse is a common complication of intestinal stoma. Although various surgical methods yield satisfactory results, nonsurgical treatment may be better for a temporary stoma. We report a case of a patient with a distal limb prolapse of a right transverse colostomy who received nonsurgical treatment with satisfactory results. For the treatment of a temporary transverse loop colostomy with distal limb prolapse, we designed a simple device consisting of a pediatric plastic medicine cup, which was rolled into a towel to shape the bottom of the cup into a compressor. The towel was put on the stoma outside of the colostomy bag with the compressor above the prolapsing limb of the colostomy. An abdominal binder was applied to fix the towel.
Journal of the Chinese Medical Association 04/2006; 69(3):138-9. · 0.79 Impact Factor
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ABSTRACT: Buried penis is a result of penile skin deficiency and inadequate attachment of the skin to the Buck's fascia. Prolonged postoperative edema (>1 month) has been seen in some patients after preputial unfurling. Some modifications which may reduce this complication are described.
Thirty-two boys with buried penis, aged 14 months to 12 years, underwent the surgical procedure. A circumferential incision was made at the junction of the outer and inner prepuce. The subcutaneous tissue was removed from the inner prepuce and degloved from the Buck's fascia, so that a thin inner prepuce could be sutured directly to Buck's fascia. Excessive prepuce was trimmed. Reapproximation of the outer and the inner prepuce completed the procedure. A narrow skin ring at the penile base, which may compromise venous and lymphatic flow, was avoided by dorsal or ventral incision.
Mild edema was seen in some patients but subsided within a month. Satisfactory cosmetic results were achieved.
This procedure unfurls the inner prepuce to cover the penile shaft. Ideal cosmetic results can be achieved if some details of the procedure are emphasized.
Journal of pediatric urology 10/2005; 1(5):327-9. · 1.38 Impact Factor
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ABSTRACT: A case of proximal jejunal obstruction by a congenital band is reported. The band ran from the antimesenteric wall of the proximal jejunum just distal of the Treitz's ligament to the root of the mesentery. Lysis of the band and enterotomy were performed to make sure no intrinsic obstruction cured the patient. No recurrence was found after 3 months of follow-up. To the best of the authors' knowledge, this is the second case with an anomalous congenital band causing proximal jejunal obstruction reported in the English-language literature.
Journal of Pediatric Surgery 04/2005; 40(3):E27-9. · 1.45 Impact Factor
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ABSTRACT: The Palmaz stent can relieve congenital malacia or stenosis of airway, but reports on the indications and results of stent removal are rare. The authors report their experience in removing Palmaz stents and discuss the indications for removal.
Thirteen stents in 12 patients were removed by a rigid bronchoscope for various reasons. The indications were expected recovery (n = 5), severe granulations and expected recovery (n = 2), stent collapse (n = 2), and stent migration and/or fracture (n = 4).
The course after removal was smooth in 9 patients and complicated in 3. The indications for stent removal in these 3 complicated cases were all expected recovery. One of the 3 complicated cases needed emergent cardiopulmonary bypass and tracheostomy for a collapsed stent that occluded airway, 1 failed for intractable bleeding, and 1 failed for intense vagal reflex causing cardiac arrest. At 6 months of follow-up, satisfactory results were seen in all but one case that needed further procedures for an iatrogenic tracheoesophageal fistula.
Most Palmaz stents can be removed smoothly with a rigid bronchoscope; however, lethal complications can happen. The authors suggest that indications for stent removal should be intractable airway symptoms caused by the stent rather than expected recovery. Muscle relaxants should be avoided during anesthesia to maintain spontaneous breathing, and cardiopulmonary bypass should be on standby.
Journal of Pediatric Surgery 01/2005; 39(12):1772-4. · 1.45 Impact Factor
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ABSTRACT: Buried penis is a result of penile skin deficiency and inadequate attachment of the skin to the Buck's fascia. A modified prepuce unfurling technique and the results are reported.
Thirty-two boys with buried penis, aged 14 months to 12 years, underwent the surgical procedure. A circumferential incision is made at the junction of the outer and inner prepuce. The subcutaneous tissue is dissected from the inner prepuce and degloved from the Buck's fascia, so that a thin inner prepuce can be sutured directly to Buck's fascia. Reapproximation of the outer and the inner prepuce completes the procedure.
Prolonged preputial edema was seen in two patients. No patient had skin necrosis. Most patients achieved satisfactory results.
This procedure unfurls the inner prepuce to cover the penile shaft. Ideal cosmetic results could be achieved in selected patients if some details of the procedure are emphasized.
Zhonghua yi xue za zhi = Chinese medical journal; Free China ed 10/2002; 65(9):422-5.
