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Liver transplantation (LT) for small infants6 months old is rare but becoming common as perioperative care improves. In Taiwan, living donor LT (LDLT) has expanded indications but is rarely performed for this age group because of unfavorable outcomes in the literature. We evaluated LDLT outcomes of patients6 months old.We identified infants6 months old undergoing LDLT between 2004 and 2019 at our hospital. Variables related to recipients, donors, surgeries, and outcomes were analyzed.Nine patients were identified. Indications for LT were biliary atresia (n = 2), Alagille syndrome (n = 1), protein C deficiency (n = 1), and acute liver failure (n = 5), including two patients with neonatal hemochromatosis, one with herpes simplex hepatitis, one with giant cell hepatitis with autoimmune hemolytic anemia, and one with hemophagocytic lymphohistiocytosis. Median age and weight at LT were 129 days and 4.8 kg, respectively. Graft types included left lateral segment (LLS, n = 4), hyper-reduced LLS (n = 4), and monosegment (n = 1). The median graft-to-recipient weight ratio was 4%. The median follow-up period was 14 months (range, 8 days to 127 months) with two mortalities, and two patients were totally weaned off immunosuppressants. Adjuvant therapies were required for patients with giant cell hepatitis and hemophagocytosis. Preoperative reconstructive imaging for estimating graft thickness facilitated surgical planning.Although LDLT is difficult to perform for small infants, outcomes are favorable and mainly dependent on underlying causes in addition to technical innovations.

Using "large-for-size" liver graft, graft-to-recipient weight ratio (GRWR) ≥4%, has been debated in pediatric liver transplantation due to possible graft compartment after abdomen closure. Meticulous preoperative evaluation with three-dimensional (3D) techniques may prevent these problems. This study compared the safety of large-for-size grafts in pediatric living donor liver transplantation (PLDLT) during the eras with or without 3D planning.We defined the 3D era was after November 2017 due to our first implication of 3D printing for surgical planning and subsequently developing a 3D simulation implanting model. From November 2004 to July 2021, we enrolled 30 PLDLT patients with body weight (BW)10 kg and categorized them into conventional group: GRWR ≥4% before the 3D era (n = 9), 3D group: GRWR ≥4% in the 3D era (n = 8), and control group: GRWR4% (n = 13). We followed and compared their clinical outcomes.The 3D group had the lowest BW and the highest graft volume reduction rate, with all receiving modified left lateral segments (LLS), such as reduced LLS (n = 2), hyperreduced LLS (n = 5), and segment 2 monosegment (n = 1). Overall postoperative complications were similar in conventional and control groups but significantly lower in the 3D group (OR 0.06, 95% CI 0.006-0.70, p = 0.025). However, all groups had similar graft and patient survival at 1, 2, and 4 years.Advanced preoperative 3D planning can decrease post-transplant complications and increase the safety of large-for-size grafts in PLDLT.Type of study: Retrospective comparative study; Evidence level: Level III.

Background Congenital tracheobronchial stenosis (CTBS) is a rare congenital condition characterized by complete cartilage rings covering varying lengths of the major airway. In this study, we reviewed the outcomes of patients with CTBS receiving surgical tracheoplasty in our institute. Methods We retrospectively analyzed the outcomes of consecutive patients with CTBS operated between 2006 and 2017 when extracorporeal membrane oxygenation (ECMO) was used perioperatively. Results In total, 11 patients (median follow-up period, 4.2 years; interquartile range, 1.6–5.4) were included. Seven were symptomatic in the neonatal period, 10 had cardiorespiratory anomalies, 7 required preoperative bronchoscopic balloon dilatation, and 1 required preoperative stent placement. Slide tracheoplasty (STP) was performed in 9 patients, and 2 underwent pericardial patch tracheoplasty. Seven patients required postoperative balloon dilatation, and 6 required postoperative stent placement. Early stenting provided immediate ventilatory improvement in all patients and facilitated successful extubation in a median of 4 days after stenting in 80% of the patients. Conclusions Under ECMO, severe CTBS could be successfully treated through a combination of tracheoplasty and bronchoscopic management. STP provided excellent results for solitary trachea stenosis with a minimum diameter of ≥ 3 mm. In selected patients, postoperative tracheobronchial stent placement was crucial in minimizing the ECMO duration and facilitating extubation. Level of Evidence IV

Background The medial segment as a mono-segmental graft was proposed to increase the donor pool for pediatric liver transplantation, but to date, there has been no published case. This study aims to revisit the feasibility of procuring the medial segment graft (MSG) by three-dimensional (3D) printing and ex vivo procedures performed on explanted diseased livers to overcome the gap between theory and clinical implementation. Methods From October 2004 to December 2016, we retrospectively analyzed preoperative computed tomography, magnetic resonance cholangiopancreatography, and intraoperative cholangiography images of our previous live liver donors and identified the indicated anatomy for the MSG, then materialized by 3D printing models to simulate the engraftment. Furthermore, we practiced the procurement procedures on selected explanted diseased livers. Results Among 291 analyzed livers, 96 livers (33%) met the arterial criteria for MSG, and two-thirds of them had ideal portal branches for reconstruction. The proposed right border of the MSG was the Cantlie's line, and the left edge was the right side of the umbilical fissure. The mean estimated volume of the MSG was 234 ± 54 ml. Besides, we suggest implanting the MSG as an auxiliary partial graft in an inverted vertical position or a standalone graft with right-side rotation in the right subphrenic space. Conclusion The procurement of the MSG is feasible based on our results. However, due to the novelty of the procedure, we suggest that the first attempted case of MSG should be implanted as an auxiliary partial graft to maximize patient safety. Level of Evidence Type of study: Case series with no comparison groups Evidence level Level IV.

Background Antifibrosis therapy may prevent progressive liver fibrosis after successful Kasai portoenterostomy (KPE) in biliary atresia (BA) patients. The aim of this study is to evaluate the efficacy of antifibrosis therapy in a rat model of BA and KPE. Methods BA model was created on three-week-old Sprague–Dawley rats by intrabiliary alcohol injection as previously described, and KPE was performed at postoperative week (POW) 5 by cystoenterostomy. Liver biopsies were performed at the time of BA creation, during KPE, POW 9, and at sacrifice (POW 17). Prednisolone (0.1 mg/100 g/day, group 1, n = 20), Vitamin A (0.5 mg/100 g/day, group 2, n = 20), and ursodeoxycholic acid (UDCA, 1.5 mg/100 g/day, group 3, n = 20) were respectively given to three groups after KPE and continued daily until sacrifice. Histological evaluation of fibrosis and immunohistochemistry stains for 8 fibrosis markers were compared to the control group (without medication, n = 10). Results Among the four markers, namely ɑ-smooth muscle actin (ɑ-SMA), glial fibrillary acidic protein (GFAP), tumor growth factor β1 (TGFβ1) receptors 1 and 2, which showed persistently high expression after successful KPE in the examined 8 markers, only the expression of ɑ-SMA was significantly reduced in all treatment groups at POW17. However, the fibrosis grade at POW 17 was only significantly reduced in group 2 in comparison with the control group (Vitamin A vs. control group, Ishak score 3 vs. 1.8, p Conclusion In our rat model of BA with KPE, Vitamin A was effective in reducing liver fibrosis, and the mechanisms deserve further study. Level of evidence Basic science.

Background Endovascular intervention with stent placement to treat portal vein (PV) and hepatic vein (HV) stenosis after pediatric liver transplantation (LT) is still controversial in small children owing to the potential risk of functional stenosis after growth. The aim of this study is to evaluate the safety and efficacy of stent placement in this population. Methods Between 2004 and 2016, 6 children (all n = 2) and PV ( n = 4) stents placement among 46 pediatric LT patients at our institution. The clinical outcome and patency rate were followed. Morphologic changes of stents were assessed from plain films by a new index: the stent diameter ratio (SDR). Results The median age of the patients at LT was 8.9 months. The patency rate was 100% without functional stenosis during a median follow-up period of 65.5 months. The "stent growth" phenomenon was demonstrated by SDR with significant resolution of hourglass deformity 2 years after stent placement ( p for trend Conclusion Vascular stent placement is a safe and effective method for the management of PV and HV stenosis following pediatric LT because these stents will enlarge as children grow. Type of study Case Series with no Comparison Group Level of evidence Level IV.

We aimed to minimize the dose of tacrolimus in pediatric patients undergoing liver transplantation prospectively.Pediatric liver transplant recipients with stable graft function1year (transplant at1year of age), or 2years (transplant at1year of age) post transplant were screened. After baseline graft biopsy, patients were enrolled into our protocol for elective tacrolimus dose reduction. Patients were assessed by liver function test and protocol biopsy during and after tacrolimus dose reduction.From January 2011 to December 2012, 16 patients were recruited, of whom 15 completed follow-up at a mean 40.75±5.98months. Six patients were preliminarily weaned off tacrolimus, and five remained tacrolimus-free for more than 2years. Of the 10 patients who were not weaned off tacrolimus, six experienced seven episodes of clinical rejection. Five patients had a reduction in tacrolimus dosage to an undetectable trough level, another five to a trough level4ng/ml, including one patient who was off the study. At the last patient visit, all of the patients had normal liver function test results with no graft loss. Three patients had low-grade graft fibrosis. The patients with metabolic liver disease (p=0.039) and who were recruited earlier after transplantation (p=0.028) were more likely to be weaned off tacrolimus.Tacrolimus withdrawal is feasible in select pediatric liver transplant recipients, and long-term follow-up for these patients is suggested.

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.

Background 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. Methods 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). Results 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. Conclusions 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.

The contralateral internal inguinal rings of 333 children were examined with a laparoscope during repair of unilateral inguinal hernia. The nonpuncture laparoscopy technique was used so that no extra wound was created. The morphology of the rings was classified as follows: type 1, flat and covered tightly with peritoneum; type 2, shallow with a visible base under an elevated peritoneal fold; type 3, deep ring that represented a patent processus vaginalis. The incidence of types 1, 2, and 3 rings was 45%, 23%, and 32%, respectively. The incidence of type 1 internal rings was similar for different age groups. The incidence of type 2 rings increased with age, and that of type 3 decreased with age. This observation is compatible with autopsy studies and the experience of contralateral exploration of childhood inguinal hernias.

The authors studied the preoperative Technetium 99m-dimercaptosuccinic acid renal scan (DMSA) of patients undergoing unilateral vesicoureteral antireflux surgery to compare the amount of renal scarring between the refluxing and the contralateral renal units. They sought to determine whether postoperative contralateral vesicoureteral reflux was preexistent or new onset.Sixty-eight patients who underwent unilateral vesicoureteral antireflux surgery and had preoperative DMSA and postoperative voiding cystourethrography (VCUG) examinations were studied. Preoperative DMSA results were analyzed to determine the amount of renal scarring in each kidney.Sixty-four (94.1%) ipsilateral refluxing renal units had renal scars. Of the 68 contralateral renal units, scars were noted in 28 (41.2%). The rate of nonscar was 4 of 68 (5.9%) in reflux kidneys, which was significantly lower than 40 of 64 (62.5%, excluding 4 with a history of resolved reflux) in nonreflux kidneys (P.001). Of 40 contralateral nonscarred kidneys, 1 of 40 (2.5%) had subsequent reflux, which was significantly lower than 5 of 28 (17.9%) of scarred kidneys (P.005). Six patients (8.8%) had contralateral reflux, and 1 of them had a history of resolved reflux. Of the 6 contralateral kidneys with severe scarring involving 3 poles or contracted, 4 of 6 (66.7%) had subsequent reflux.Scar in the contralateral kidney seen on DMSA scan seems to predict contralateral reflux after unilateral antireflux surgery. The contralateral reflux may be preexistent. Postoperative VCUG should be performed routinely for patients who have contralateral renal scars. In patients with a history of contralateral reflux or severe contralateral renal scar, simultaneous contralateral ureteral reimplantation should be considered.

Purpose: The authors describe the experience of a modified technique to correct primary vesicoureteral reflux in children. Methods: Twenty children (28 renal units) with primary vesicoureteral reflux (grade III to grade IV) were treated with this technique. The authors incised the bladder mucosa and muscle along the direction of the ureter using a right-angled probe as a guide. About 2 to 3 cm of extravesical ureter was freed and pulled into the bladder via the incision. The bladder muscle was closed under the ureter, and the mucosa was repaired over the ureter. Results: One patient had residual reflux. All other patients, who underwent follow-up with sonogram and voiding cystourethrogram from 14 to 42 months after the operation, had neither recurrent reflux nor urinary tract obstruction. Conclusions: The advantages of this technique are (1) anastomosis of the ureteral orifice to the urinary bladder is not needed, so that the risk of orifice stenosis is minimized; (2) the ureteral orifice remains unchanged after the procedure, so that ureteroscopic procedures are easier to perform in future if required; and (3) pelvic dissection is not needed as in extravesical detrusorrhaphy. However, this procedure may not be suitable for patients with severe hydroureter.