Long-term Results In Children Research Articles

Primary versus secondary intraocular lens implantation following removal of congenital/de al of congenital/developmental catar elopmental cataracts: outcomes after acts: outcomes after at least 4 years.

The aim of this study is to evaluate the long-term outcomes of primary and secondary intraocular lens (IOL) implantation following removal of congenital/developmental cataracts. One hundred and forty-four patients aged under 16 years who were followed up between 2003 and 2021 were analyzed retrospectively. The long-term results of children who underwent surgery before 2 years of age for congenital or developmental cataracts and underwent secondary IOL implantation after 2 years of age and those who underwent cataract surgery with primary IOL implantation after 2 years of age were compared. Patients with traumatic, secondary cataracts and cataracts due to ocular anomalies were not included in the study. We evaluated 64 patients (mean age 9.5 ± 4.5 years) with secondary IOL implantation and 80 patients (mean age 12.8 ± 4.1 years) with primary IOL implantation in the study. Distance and near best-corrected visual acuities were significantly better in the primary IOL group than the secondary IOL group (p < 0.001). Incidence of strabismus after primary IOL surgery was significantly lower and presence of binocular vision was more often than the secondary IOL group (p = 0.002). There was no significant difference between the two groups in terms of refraction and myopic shift (p = 0.242, p = 0.172, respectively). Mean refractive changes were significant in unilateral cases of secondary IOL group and primary IOL group (p = 0.013, p = 0.049, respectively) and myopic shift was also greater in both groups of unilateral cases than the fellow eyes (p = 0.023, p = 0.012, respectively). Visual outcomes and binocular vision were better, and the incidence of strabismus was also much less in the primary IOL group.

Pulmonary Embolism in Childhood: A Multicenter Experience from Turkey

Background:Pulmonary embolism is a clinical condition caused by the obstruction of the pulmonary artery and its branches with endogenous, exogenous embolism, or local thrombus formation. It is a rare but potentially life-threatening event in the pediatric population. Pediatric pulmonary embolism has many unknown characteristics.Aims:To evaluate clinical features, genetic and acquired risk factors, diagnostic imaging, and treatment strategies with long-term results in children with pulmonary embolism.Study Design:A retrospective multicenter clinical trial.Methods:Patients aged 0-18 years who were diagnosed with pulmonary embolism with computed tomography pulmonary angiography (CTPA) findings (intraluminal filling defect in the lobar or main pulmonary artery) in 3 university hospitals between 2006 and 2021 were included in the study. A form was created for data standardization, and variables were collected retrospectively through medical record review. In addition to the features given above, we also evaluated in situ pulmonary artery thrombosis (ISPAT) and patients’ Wells scores. Follow-up CTPA results were evaluated for patient response to treatment. Complete recovery means that there were no lesions, incomplete recovery if there was still embolism, and no response if there was no change.Results:Twenty-four patients (female:13, male:11) were included in the study. The mean age was 13.5 years. All patients but one had at least one or more genetic or acquired risk factors. Factor V Leiden mutation (16.6%) was the most common genetic risk factor. Six of 16 patients with Doppler ultrasonography were diagnosed with ISPAT because there was no sign of thromboembolic thrombosis. Nine (41.6%) patients had a Wells score of >4 (pulmonary embolism clinically strong), and 15 (58.4%) patients scored <4 (pulmonary embolism clinically likely weak), indicating that an alternative diagnosis was more likely than pulmonary embolism (sensitivity %37.5). The mean follow-up period was 23 (±17) months. Complete and incomplete recovery was observed in 15 (62.5%) and 7 (29.1%) patients, respectively, among the patients who underwent follow-up evaluation. No response was obtained in 2 patients (8.3%) who died.Conclusion:The Wells scoring system seems insufficient to diagnose pulmonary embolism in children and should be improved by adding new parameters. ISPAT may be more common in children with congenital heart disease and systemic disease.

Long-term Results in Children with Henoch-Schönlein Nephritis.

Objective:Henoch-Schönlein purpura (HSP) is a small vessel vasculitis and palpable purpura, with arthritis, gastrointestinal as abdominal pain, and renal involvement as typical clinical findings. The most important prognostic factor for HSP vasculitis is renal involvement. This study aimed to investigate the relationship between clinical, laboratory, and histopathologic findings of children with HSP nephritis with long-term renal prognosis.Methods:This retrospective study included children with HSP nephritis between January 2010 and December 2019. Initial clinical presentation, laboratory findings, and kidney biopsy results were obtained, and treatment modalities were recorded and classified using the Meadow classification and grouped into mild and severe cases. Additionally, data at the last follow-up were analyzed and classified.Results:A total of 90 children (59 male) with a mean age of 8.8±3.2 years were included. According to initial clinical findings, 18 children were in the Meadow’s severe group. Fifteen (15/72) children in the mild group and all children in the severe group had undergone kidney biopsy. The severe group had higher histopathologic stages compared to the mild group (p=0.022). Immunosuppressive treatments were used in 44.4% of mild cases and 100% of severe cases (p<0.01). On follow-up, only four children (two in the mild group) had persistent proteinuria.Conclusions:Severe clinical findings in the initial presentation were related to more intensive immunosuppressive treatment. Additionally, renal histopathological stages were higher in the severe group. Long-term follow-up for proteinuria is mandatory for all children with HSP nephritis, even with mild initial clinical findings.

Отдаленные итоги лечения детей с интраокулярной ретинобластомой

Отдаленные итоги лечения детей с интраокулярной ретинобластомой

Outcome of posterior urethral valve in 64 children: a single center's 22-year experience.

Posterior urethral valve (PUV) is the most serious form of congenital anomalies of kidney and urinary tract (CAKUT) in boys with significant risk of progression to chronic kidney disease (CKD). We present our long-term results in children with PUV. Retrospective chart review of 113 children with PUV followed within the years of 1996-2018 was performed. Clinical, laboratory and epidemiologic parameters were analyzed for their impact on renal outcome. The median age of diagnosis was 1.00 month (1.00-132.00) and the median follow-up period was 70 months (60.00-216.00). Antenatal diagnosis was present in 33 patients (51.5%) mainly with bilateral hydronephrosis and oligohydramnios. The most common postnatal presentation was recurrent urinary tract infection (UTI) in 14 cases (21.9%) and incontinence in three cases (4.7%). Vesicoureteral-reflux (VUR) was present in 31 cases (48.4%). All patients had surgery and urinary diversion was needed in 18 (28.2%). Varying stages of chronic kidney disease (CKD) developed in 23 cases (35.9%) and rise in serum creatinine was especially prominent after the 4th year of follow-up. Of 23 CKD patients, seven (10.9%) were in ESRD and on dialysis. Mortality occurred in one (1.5%) patient. Hypertension, proteinuria and high initial serum creatinine (>1.28 mg/dL) were statistically significant risk factors for CKD, as expected. Surprisingly VUR and UTI did not show such a significant impact on CKD development. Antenatal detection was with significantly less risk for CKD. Our results confirm that PUV has a considerable risk for CKD development. Antenatal diagnosis, management of proteinuria and hypertension may modify this progression. But already injured kidneys still have a potential risk. The need for further research to evaluate the impact of any intervention on long term renal outcome is obvious.

САНАТОРНО-КУРОРТНОЕ ЛЕЧЕНИЕ ДЕТЕЙ С ЭКОЛОГООТЯГОЩЁННЫМ ПИЕЛОНЕФРИТОМ, АССОЦИИРОВАННЫМ С НАРУШЕНИЯМИ ПУРИНОВОГО ОБМЕНА

Objective: To study the effectiveness of the treatment on the basis of drinking mineral waters, carbon dioxide baths and rational pharmacotherapy in children with environmental burdened chronic pyelonephritis, associated with violations of purine exchange, in the conditions of the Zheleznovodsk resort. Methods: Fifty children with chronic pyelonephritis with an environmental burdened anamnesis of 11-14 years were under observation. The method of simple randomization was formed two groups: control (25 people), which received traditionally accepted treatment at the Zheleznovodsk resort (dietary nutrition, therapeutic gymnastics, internal intake of the little mineralized mineral water of the Smirnov spring, carbon dioxide mineral baths), and the main one (25 people), where children received additional drug therapy with herbal medicine Canefron N. All patients were prescribed: ultrasound examination of the kidneys, clinical and biochemical blood and urine tests. Results: Comparative analysis showed that children of main group had a reduction of pain and dysuric syndromes, and in control group pain syndrome by the end of treatment persisted in 8% of cases, slightly more often preserved dysurian (16%) and asthenonneurotic (16%) syndromes. In children of main group, there was a significant decrease in daily oxaluria (by 39.2%; р&lt;0,01), an increase in the rate of glomerular filtration (by 11.93%), which was statistically significant in relation to the data in control group, where there was only a tendency to improve kidney function. According to the data of the long-term results in children of main group, the number of relapses decreased by 2.9 times (p&lt;0.01), while in patients of control group there was only a tendency to decrease this indicator. Conclusion: Comprehensive balneo- and drug therapy in children with environmental burdened pyelonephritis, associated with purine metabolism disorders, contribute to statistically significant to increase clinical effectiveness, ensures the long-term preservation of the achieved therapeutic effect. Keywords: Children, environmental burdened pyelonephritis, mineral waters, resort treatment.

Long-term results in children with head and neck rhabdomyosarcoma: A report from the Italian Soft Tissue Sarcoma Committee.

Rhabdomyosarcoma (RMS) occurring at nonparameningeal head and neck (NPM-HN) sites carries a better prognosis than parameningeal RMS and some other sites. We analyzed the treatments administered and results obtained in patients with localized NPM-HN RMS, included in the protocols coordinated by the Italian Soft Tissue Sarcoma Committee (STSC), in an effort to identify prognostic factors that could facilitate the tailoring of treatment. Sixty-six patients up to 18 years of age with previously untreated, localized NPM-HN RMS were prospectively registered in three consecutive protocols: RMS79, RMS88, and RMS96. Primary surgery was recommended when complete tumor resection was deemed feasible without mutilations. In other cases, only a biopsy was performed, followed by chemotherapy and delayed surgery and/or radiotherapy (RT). NPM-HN RMS showed favorable characteristics: 72.7% were<5cm, 72.7% were T1, and 80.3% were N0. With a median follow-up of 16 years (range 7-27), the 10-year progression-free survival and overall survival for the whole group were 65.1% (confidence interval [CI]: 52.3-75.3) and 74.2% (CI: 61.8-83.1). Progressive improvement has been seen in the successive protocols. Age and RT emerged as independent prognostic factors. The group of young children (age <lyear) not irradiated was at high risk of failure. The experience of the Italian STSC confirms that NPM-HN RMS has a favorable prognosis, which has improved over the years due to a better, multimodal treatment approach. RT has an important role, but different modalities such as proton therapy and brachytherapy should be explored in systematic ways in very young children.

Techniques Used to Create Continent Catheterizable Channels: A Comparison of Long-term Results in Children

To compare long-term results of 3 different techniques used to create continent catheterizable channels (CCCs) in children in a single institution. Data were retrospectively collected from 112 children who had a CCC (appendicovesicostomy [APV], tubularized bladder flap [TBF] or Monti) created between December 1995 and December 2013. Primary outcome was revision-free survival. Secondary outcomes were channel stenosis, incontinence, and false channel requiring revision. Time-to-event survival was analyzed using a Kaplan-Meier survival curve. A total of 117 CCCs were identified with median follow-up of 85 months. Surgical revision was required in 52%, with no significant difference between the different techniques. Major revision was required in 27% of the cases and significantly more often in Monti channels. Complete revision was required in 7%. Stenosis requiring revision was seen in 33%. A false channel was formed in 9% of the cases. Incontinence with a low leak point pressure was seen in 12%. A time-to-event analysis with a Kaplan-Meier curve showed no significant differences between the 3 techniques. Although CCC is an effective solution for children who cannot perform urethral catheterization, 52% requires surgical revision. Because no differences were found in outcome between APV, TBF, and Monti, we conclude that a TBF CCC is a good alternative for the APV. If the appendix is not available and bladder volume is sufficient, a TBF channel is preferred to a Monti channel.

Spinal deformity changes in children with long-term vertical expandable prosthetic titanium rib treatment

Background ContextIn several studies, vertical expandable prosthetic titanium rib (VEPTR) implants have shown good scoliosis control in children with the longest reported follow-up of 3.6 years. For growing rods, recent studies suggest a decreased efficiency of correction starting just after that time. To our knowledge, no long-term results of children with VEPTR treatment are available. PurposeThis study aimed to evaluate spinal deformity in scoliotic children and to investigate correction potential of VEPTR implants at several time points of treatment, particularly after long-term follow-up. Study Design/SettingWe performed a retrospective case series of 32 children with spinal deformity and VEPTR treatment with analysis of clinical and radiological data pre- and post-VEPTR implantation and every 2 years during the follow-up period. Patient SampleThirty-two patients with spinal deformity and VEPTR treatment comprised the patient sample. Outcome MeasuresPatients had a primary VEPTR implantation due to spinal deformity and thoracic insufficiency syndrome and repeated lengthening procedures every 6 months. Clinical data were assessed and radiological parameters were analyzed. The main thoracic scoliotic curve and associated curves as well as kyphosis, lordosis, pelvic obliquity, and spinal length were measured in all radiographs until the end of VEPTR treatment or the last available examination. MethodsDevelopment of the different parameters during follow-up was evaluated and statistical analysis was performed with Statistica version 13.0. No funding was obtained for this study. The authors have no conflicts of interest to declare. ResultsDirectly after VEPTR implantation, thoracic and lumbar curves corrected significantly, were stable at 2.8-year follow-up, and increased at 5.5-year follow-up, whereas cervical scoliosis was not affected by the treatment. The sagittal profile was initially improved both in kyphosis and lordosis. However, at 5.5-year follow-up, hyperkyphosis had deteriorated beyond the initial deformity. Pelvic obliquity was significantly restored especially in neuromuscular patients, and increasing spinal length was achieved within the 5.5-year follow-up. ConclusionIn children with spinal deformity, implantation of the VEPTR device sufficiently corrected the deformity in all planes. During long-term follow-up, scoliosis increased slightly and was rather well controlled, whereas the implant system was not able to prevent deterioration of hyperkyphosis. Pelvic obliquity was well balanced and spinal lengthening was achieved during long-term follow-up.

Long-term follow-up of children and adolescents with primary sclerosing cholangitis and autoimmune sclerosing cholangitis

Sclerosing cholangitis (SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate long-term results in children with SC according to the types of SC. We retrospectively followed up 25 children with SC over a period of 4-17 years (median 12). The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis (ASC); other patients were included in a group of primary sclerosing cholangitis (PSC). The incidence of the following complications was studied: obstructive cholangitis, portal hypertension, advanced liver disease and death associated with the primary disease. Fourteen (56%) patients had PSC and 11 (44%) had ASC. Patients with ASC were significantly younger at the time of diagnosis (12.3 vs 15.4 years, P=0.032) and had higher IgG levels (22.7 vs 17.2 g/L, P=0.003). The mentioned complications occurred in 4 (16%) patients with SC, exclusively in the PSC group: one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, two other children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications. In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.

Pediatric Coronary Artery Revascularization Surgery: Development and Effects on Survival, Cardiac Events and Graft Patency for Children With Kawasaki Disease Coronary Involvements.

Pediatric coronary artery bypass surgery gained wide acceptance with the introduction of internal thoracic arteries (ITAs) for bypass operations for post Kawasaki disease (KD) lesions. The technique is now established as the standard surgical choice, and its safety even in infancy, graft patency, growth potential, graft longevity and clinical efficacy have been well documented. In this article the author reviews the development of pediatric coronary bypass as the main indication for the treatment of coronary lesions due to KD. I believe that coronary revascularization surgery in pediatric population utilizing uni- or bilateral ITAs is the current gold-standard as the most reliable treatment, although percutaneous coronary intervention with or without a stent has been tried with vague long-term results in children.

Renal Outcome after Combined Liver-kidney Transplantation in Children

Background: Combined Liver and Kidney Transplantation (CLKT) is a recognized treatment option for a select group of paediatric patients with severe liver and kidney disease. The aim of this study is to report Indication and outcome of CLKT in our center and to compare the results with the Isolated Kidney Transplantation (IKT). Patients and method: We retrospectively reviewed children who underwent CLKT between 1997 and 2009. Data analyzed include age, sex, cause of kidney/liver disease, pre-transplant dialysis, donor age, cross-match, and immunosuppression regimen. Additionally, cold ischemia time, Acute Rejection (AR) episodes and patient and graft survival were compared with patients who had IKT in the same period of time. Results: 9 children (5 females, 4 males) underwent CLKT and 127 IKT. In CLKT group, mean patient age was 10.5 ± 3.9 years. Indications for CLKT were: primary hyperoxaluria (n=3), hemolytic uremic syndrome (n=1), Alagille Syndrome (n=1), nephronoptisis (n=1) and polycystic hepatorenal disease (n=3). Five patients were on dialysis before transplant. In all patients, both grafts were obtained from the same donor and cross-matches were negative. Immunosuppression was induced with Basiliximab and maintained with triple therapy (Tacrolimus+Mycophenolate mofetil+Prednisone). Mean cold ischemia time was shorter in CLKT (10.9 ± 3.5 vs. 16.8 ± 4.6 hours) and hospital stay was longer (35.5 ± 10 vs. 13.9 ± 5.3 days) compared to IKT. Long-term mean Glomerular Filtration Rate (GFR) and cystatin levels were similar in both groups. Rejection tended to be less frequent in CLKT (11 vs. 25%) than in IKT. Patient survival was lower (P=0.008) in CLKT. One patient with chronic liver and kidney rejection due to noncompliance died during re-transplantation 5 years after the first transplant. There were no deaths in the IKT group Mean follow-up time is 5.27 ± 2.9 years the IKT group Mean Follow-up time is 5.27 ± 2.9 years. Conclusions: Most common indications for CLKT in our children are primary hyperoxaluria and polycystic disease. Long-term results in children receiving CLKT are comparable to those with isolated kidney transplantation.

Matched Sibling Versus Matched Unrelated Allogeneic Hematopoietic Stem Cell Transplantation in Children with Severe Acquired Aplastic Anemia: Experience of the Polish Pediatric Group for Hematopoietic Stem Cell Transplantation

In the study, 48 children with severe acquired aplastic anemia (SAA) transplanted from matched sibling donor (MSD) between 1991 and 2009, and 38 children with SAA transplanted from matched unrelated donor (MUD) between 2000 and 2009 were evaluated. Engraftment was achieved in 45 (93.75%) patients after MSD-hematopoietic stem cell transplantation (HSCT) and in 33 (86.8%) after MUD-HSCT. Transplant-related mortality rate after MSD-HSCT was 8%, while 37% after MUD-HSCT. After MSD-HSCT 44 (91.7%) patients are alive for 1-216months (median: 85months), while after MUD-HSCT 24 (63.2%) patients for 1-84months (median: 16months). The 5-year probability of event-free survival after MSD-HSCT and MUD-HSCT was 87 and 53%, respectively, while 5 years of overall survival was 91 and 64%, respectively. It was concluded that MSD-HSCT as the first line treatment for children with SAA is a safe therapeutic approach with a low rate of treatment failures and excellent outcome. Results of MUD-HSCT in pediatric patients with SAA who failed to respond to immunosuppressive therapy are still inferior than those of MSD-HSCT. Treatment failures of MUD-HSCT are mainly related to infectious complications and graft failure. It seems, however, that HLA-matching of unrelated donors at allelic level along with early MUD-HSCT after FCA (FLUDA, low-dose cyclophosphamide, and anti-thymocyte globulin) conditioning, perhaps using lower Thymoglobulin dose could enable further improvement of long-term results in children with SAA who lack MSD.

Homeopathy in paediatric atopic diseases: long-term results in children with atopic dermatitis

To study the socio-demographic features, the prescribed remedies and the outcome of atopic diseases in children treated with homeopathy at the Homeopathic Clinic of Lucca (Italy), and the long-term outcome of children suffering from atopic dermatitis (AD) after an approximate 8-year period (range 5-10 years). Our data derive from an observational longitudinal study carried out on 213 children (38.6%) with atopic diseases out of 551 children consecutively examined from September 1998 to December 2008. We used the Glasgow Homeopathic Hospital Outcome Score to evaluate the results that were classified on the basis of a Likert scale. Eighty-three (39%) children were affected by asthma, 51 (24%) by allergic rhinoconjunctivitis, 76 (36%) by AD and 3 (1%) by food intolerance. Follow-up patients were 104 (48.8%), and 65 (62.5%) of them reported a major improvement or resolution. The parents of paediatric patients suffering from AD, who had started homeopathic treatment at <4.9 years of age were invited to follow-up assessment 8 years later and 40 children (mean age 12.9) were examined; 28/40 (70%) had a complete disappearance of AD, 12/40 children (30.0%) were still affected by AD; 8/40 (20%) had asthma and 8/40 patients had, or developed, allergic rhinitis. These preliminary results seem to confirm a positive therapeutic effect of homeopathy in atopic children. Furthermore, according to the data from the literature paediatric patients treated with homeopathy seem to show a reduced tendency to maintain AD and develop asthma (and allergic rhinitis) in adult age.

Long-term results in children with standard risk acute lymphoblastic leukaemia treated with 5.0 g/m2 versus 3.0 g/m2 methotrexate i.v. according to the modified ALL-BFM 90 protocol. The report of Polish paediatric Leukemia/lymphoma study group

PURPOSE: A non objective randomised, retrospective study was performed to assess and compare the efficacy of two different high doses of methotrexate (HD-MTX) in combination with intrathecal therapy in children with standard risk (SR) ALL as central nervous system (CNS) preventive therapy in a place of cranial radiation. PATIENTS AND METHODS: Between July 1993 and April 2002, in 650 children standard risk (SR) ALL were diagnosed and treated in Poland according to the modified ALL-BFM 90 protocol. Methotrexate was used in the dose of either 3.0 g/m2 (group A) or 5.0 g/m2 (group B) four times in two week intervals during consolidation (protocol M). RESULTS: Probability for 10-year event-free survival (pEFS) and relapse-free survival (pRFS) for all evaluated patients (n = 611) was 0.77 ± 0.02 and 0.82 ± 0.02, respectively. EFS probability for group A (n = 411) was 0.73 ± 0.03 and for group B (n = 200) – 0.85 ± 0.04, whilst RFS probability for group A was 0.79 ± 0.04 and for group B – 0.89 ± 0.03, and these differences proved statistically significant (P = 0.0050 and P = 0.0012). In multivariate analysis, T-cell immunophenotype (P = 0.0046) and age older than 10 years (P = 0.0250) had a significantly adverse impact on the outcome. CONCLUSIONS: HD-MTX used in dose 5.0 g/m2 has been found to be more effective in the treatment of SR ALL in children than HD-MTX in dose 3.0 g/m2.

Long-term Results in Children With Massive Bone Osteoarticular Allografts of the Knee for High-grade Osteosarcoma

Reconstruction of distal femur or proximal tibia in growing patients is a challenge for the high rate of complications and limb length discrepancy at the end of growth. The purpose of this study was to evaluate the long-term outcome of children affected by high-grade osteosarcoma of the knee region, reconstructed by osteoarticular bone allograft of distal femur, and proximal tibia. We retrospectively reviewed 25 patients treated for high-grade osteosarcoma, 13 in the distal femur and 12 in the proximal tibia. The mean follow-up was 124 months. Clinical and radiologic evaluation was carried out in the 20 long-term survivors with a minimum follow-up of 7 years from surgery. The rates of survival of the implants were estimated with use of the Kaplan-Meier method. Functional and radiographic evaluation was done according to the Musculoskeletal Tumor Society scoring system at the time of the latest follow-up in all the patients that still had the allograft. Five patients died during the first 2 years of follow-up for disease-related causes. Of the remaining 20 osteoarticular allografts (10 of the distal femur and 10 of the proximal tibia), 12 failed: 4 in the distal femur and 8 in the proximal tibia. All the failures were related to a graft fracture, but in 4 patients with subchondral collapse the graft was maintained and converted into an allograft prosthetic composite. No deep infection of the primary reconstruction was observed. The overall rate of allograft survival was 70% at 5 years and 58% at 10 years in the distal femur, and 45% at 5 years and 20% at 10 years in the proximal tibia. At final follow-up, 8 patients still walked on the primary implant, 6 in the distal femur, and 2 in the proximal tibia. The functional outcome of these patients was evaluated as good in 5 patients (3 with distal femoral and 2 with proximal tibial allograft), and poor in 3. Although mechanical complications significantly affect the outcome, osteoarticular allografts may represent a viable option for reconstruction in children older than 8 with high-grade sarcomas about the knee. Level IV.

Duodenum-Sparing Technique of Head Resection in Solid Pseudopapillary Tumor of the Pancreas in Children

Aim of the study was to assess the complications and long-term results in children operated on for solid pseudopapillary tumor of the pancreas (SPTP) between 1993-2008 at the authors' institution with a focus on a novel duodenum-sparing technique to treat tumors of the head of the pancreas. Retrospective analysis was performed of patient data including demographics, diagnostic measures, the operative technique focusing on tumor of the head of the pancreas, complications and long-term results. There were 13 patients (12 girls and one boy) with an average age of 14 years (9-17.5 years) at operation. In 7 patients the tumor was localized in the head of the pancreas, in 4 patients in the tail, and in 2 patients both the body and tail were involved. Patients with body and tail involvement underwent distal pancreatic resection. In 6 patients with head involvement a duodenum-sparing resection of the head and end-to-end anastomosis of the excluded jejunal loop either to the corpus or tail of the pancreas were performed. One girl underwent a modified Whipple operation. She developed a biliary fistula which closed after three weeks with endoscopic stenting. One patient with head resection developed a biliary fistula which closed after two weeks of stenting. One patient who underwent resection of the pancreatic head complained of recurrent abdominal pain one year postoperatively. All patients are alive without tumor recurrence at 6 months to 16 years after operation. SPTP is a rare pancreatic tumor with a low degree of malignancy. No perioperative chemotherapy is necessary. Therefore duodenal resection in cases of SPTP in the head of the gland seems too invasive and mutilating. The authors consider the duodenum-sparing technique to be more appropriate for the developing organism of a child. Favorable short and long-term results support this opinion.

Aortic root replacement in children: a word of caution about valve-sparing procedures

Evaluate the results of various surgical procedures used for aortic root replacement in children with aortic root aneurysm. Between 1986 and 2007, 23 children (less than 16 years of age, mean age: 8.1+/-5.1 years) underwent elective aortic root replacement for aortic root aneurysm (with associated aortic insufficiency in 10 patients). All had connective tissue defect syndromes. Nine patients underwent composite valve graft repair using a mechanical valve. Fourteen children underwent valve-sparing aortic root replacement (remodeling procedure in 11, reimplantation procedure in 3). Mean follow-up (100% complete) was 7.3+/-5.5 years (range 6 months-21 years). There was one early death (4.3%)(after valve-sparing remodeling) and no late mortality. Following valve-sparing remodeling operation (10 patients, mean follow-up: 7.8+/-3.0 years), there were 9 reoperations in 6 patients and only 5 patients retained their native aortic valve. In patients who underwent valve-sparing reimplantation operation (three patients, mean follow-up: 2.1+/-0.3 years), one underwent reoperation for endocarditis. Fifteen patients had composite valve graft replacement either as a primary operation (nine cases) or at reoperation for valve-sparing failure (six cases); mean follow-up was 8.2+/-6.2 years; there was no thrombo-embolic or hemorrhagic event and one reoperation for patient-prosthesis mismatch. (1) composite valve graft aortic root replacement provides excellent long-term results in children with aortic root aneurysm due to connective tissue disorder. This remains the first choice procedure in patients with more than minimal aortic insufficiency, with distorted aortic leaflets or needing concomitant mitral valve replacement. (2) Valve-sparing remodeling surgery yields disappointing results and should probably be abandoned in the pediatric population. (3) Valve-sparing reimplantation repair may achieve superior outcome but needs further evaluation.

Living related liver transplantation in children

Living related liver transplantation (LRLT) has become established for treating children with end-stage liver disease. The aim of this study was to review a single-centre experience of left lateral segment liver transplants from living donors in children. Fifty left lateral segment LRLT procedures have been performed since 1993. There were 17 girls and 33 boys, of median age 1.5 years (range 0.5 to 13 years), with a median weight of 10 (range 0.7-44) kg. Donors included 23 mothers, 26 fathers and one uncle, with a median age of 33 (range 19-46) years. At a median follow-up of 86 months, there was no donor mortality and low morbidity. Patient and graft survival rates were 98, 96 and 96 per cent, and 98, 96 and 93 per cent at 1, 3 and 5 years respectively. Three children had a second transplant at a median of 9 years after the first. The incidence of hepatic artery thrombosis, portal vein thrombosis and biliary complications was 6, 4 and 14 per cent respectively. Living related liver transplantation has good long-term results in children.

Long term results of the right ventricle-to-pulmonary artery shunt in Norwood procedure for children with hypoplastic left heart syndrome

Purpose: The hypoplastic left heart syndrome (HLHS) is still a challenge for pediatric cardiac surgeons. The aim of this study was to present the multistage surgical treatment long term results of children with HLHS with application in the first stage palliation of the right ventricle-to-pulmonary artery shunt (RV-PA).