Evaluation of surgical strategies and long-term outcomes in pediatric hepatocellular carcinoma.

PURPOSE: Hepatocellular carcinoma (HCC), the second most common pediatric malignant liver tumor after hepatoblastoma, represents 1% of all pediatric tumors. METHODS: A retrospective study was conducted on children with HCC treated at our center from March 2002 to October 2022, excluding those with inadequate follow-up or records. Demographic data, initial complaints, alpha-fetoprotein (AFP) values, underlying disease, size and histopathological features of the masses, chemotherapy, and long-term outcomes were analyzed. RESULTS: Fifteen patients (8 boys, 7 girls) with a mean age of 11.4 ± 4.1 years (0.8-16.4 years) were analyzed. The majority presented with abdominal pain, with a median AFP of 3.9 ng/mL. Hepatitis B cirrhosis in one patient (6.6%) and metabolic disease (tyrosinemia type 1) in two patients (13.3%) were the underlying diseases. Histopathological diagnoses were fibrolamellar HCC (n:8; 53.3%), HCC (n:6; 40%). Four of the 15 patients underwent liver transplantation, and 9 underwent surgical resection. Due to late diagnosis, two patients were considered inoperable (13.3%). The survival rate for the four patients who underwent liver transplantation was found to be 75%. CONCLUSION: Surgical treatment of various variants of HCC can be safely performed in experienced centers with a multidisciplinary approach, and outcomes are better than in adults.

A rare complication of pediatric liver transplantation: Post-transplant diaphragmatic hernia.

AIM: Acquired post-transplant diaphragmatic hernia (PTDH) is a rare complication of liver transplantation (LT) in children. We aimed to present our experience in PTDH, and a possible causative background is discussed. METHODS: Medical records of patients who had undergone diaphragmatic repair following LT between 2015 and 2023 were reviewed. Demographic information, details of primary diseases necessitating LT, transplantation techniques, and clinical findings associated with PTDH were evaluated. RESULTS: There were seven patients with PTDH. Median age at transplantation was 69 (range: 9-200) months. Five patients received a left lateral sector, one patient had a right lobe, and one had a left lobe graft. Time between LT and PTDH was 9 (2-123) months. One patient who was diagnosed in the postoperative 10th year was asymptomatic. Respiratory distress and abdominal pain were the main symptoms among all. All patients underwent laparotomy, and primary repair was performed in six patients, and one patient required mesh repair because of a large defect. Small intestine herniated in most cases. There were two complicated cases with perforation of the stomach and colonic volvulus. There is no recurrence or long-term complications for the median 60 (20-119) month follow-up period. CONCLUSION: PTDH is a rare but serious complication. Majority of symptomatic cases present within the first postoperative year, whereas some late-presenting cases may not be symptomatic. Inadvertent injury to the inferior phrenic vasculatures due to excessive use of cauterization for control of hemostasis may be a plausible explanation in those cases.

Transanal endorectal or transabdominal pull-through for Hirschsprung's disease; which is better? A systematic review and meta-analysis.

AIM: Hesitations concerning the long-term results of transanal endorectal pull-through (TEPT) due to prolonged anal stretching and resultant stricture and continence problems has been started to be questioned. This meta-analysis intended to compare long-term results between TEPT and transabdominal (TAB) pull-through techniques in the surgical management of Hirschsprung's disease. METHODS: All publications between the years 1998-2021 in the PubMed, Medline, Google Scholar, Cochrane databases were reviewed. Retrospective and prospective comparative studies for TEPT, TAB as well as Laparoscopic-assisted TEPT (LTEPT) were included. Data included age at operation, postoperative constipation, enterocolitis, incontinence, stricture, and soiling rates. RESULTS: Eighteen publications met the inclusion criteria for TAB and TEPT, and six for TEPT and LTEPT. Patients who underwent TEPT had significantly younger operation age than patients with TAB (SMD - 1.02, 95%Cl - 1.85 to - 0.18, p: 0.0168). Postoperative constipation (OR 0.39, 95% Cl 0.25-0.61 p < 0.0001) and enterocolitis (OR 0.65, 95% Cl 0.46-0.90, p: 0.0108) rates were significantly lower in TEPT groups. Postoperative incontinence (OR 1.06, 95% Cl 0.56-2.01, p: 0.8468), stricture (OR 1.97, 95% Cl 0.81-4.80, p: 0.1352) and soiling rates were similar between the two groups. Furthermore, when TEPT and LTEPT results were compared, incidence of incontinence (OR 7.01, 95% Cl 0.75-65.33, p: 0.0871), constipation (OR 1.95, 95% Cl 0.70-5.37, p: 0.199), enterocolitis (OR 3.16, 95% Cl 0.34-29.55 p: 0.3137), stricture (OR 1.33, 95% Cl 0.29-6.15, p: 0.7188) and soiling (OR 1.57, 95% Cl 0.57-4.31, p: 0.3778) were similar for both techniques. DISCUSSION: TEPT is superior to TAB in terms of constipation and enterocolitis. Contrary to concerns, postoperative incontinence rates are not statistically different. However, further publications about long-term LTEPT results are necessary for more reliable conclusions.

Fiberoptic endoscopic evaluation of swallowing (FEES) study: the first report in children to evaluate the oropharyngeal dysphagia after esophageal atresia repair.

BACKGROUND: The aim of this study was to evaluate the swallowing problems by fiberoptic endoscopic evaluation of swallowing (FEES) study in both short- and long-gap patients after esophageal atresia (EA) repair. METHODS: Hospital records of patients who had undergone surgery for EA were reviewed retrospectively. Patients were divided into two groups as short-gap (SG) group (n:16) and long-gap (LG) group (n:10) to compare the swallowing problems. FEES study was performed, and the results were discussed in detail. RESULTS: There were twenty-six (16 M/10 F) patients with a mean age at evaluation was 7.52 ∓ 3.68 years. Mean follow-up period was 75.35 ∓ 44.48 months. In FEES study, pharyngeal phase abnormalities were detected in 10 patients (38.4%). Pharyngeal phase abnormalities were detected significantly higher in LG group (p:0.015). Laryngeal penetration/aspiration was seen in four patients on FEES study (15.3%). All of them was in LG group (40%). Laryngeal penetration/aspiration was seen significantly higher in LG group (p:0.014). CONCLUSION: This is the first study to conduct FEES study in children after esophageal atresia repair to evaluate their swallowing conditions. Even though our sample is small, swallowing problems are more common than expected in the cases of LG when compared to SG.

Diffusion tensor imaging in anorectal malformations: a pilot study for the evaluation of lumbosacral plexus.

INTRODUCTION: Neurogenic bladder with anorectal malformations (ARM) is a well-known yet understudied topic. Diffusion tensor imaging (DTI) is a special usage of MRI that can evaluate peripheric nerves. The aim of this study is to evaluate the lumbosacral plexuses of patients with ARM using DTI. PATIENTS AND METHODS: Patients with ARM willing to participate were included. Patient files were reviewed, a questionnaire was made, and DTI was performed. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were computed for the right and left lumbosacral plexuses. RESULTS: There were 18 patients and 12 controls. Groups were similar in terms of sex (p = 0.232) and age (p = 0.853). There was a significant difference only for ADC of the left plexus. There was visible asymmetry in tractographies and DTI parameters of two patients with severely deformed pelvises (image 1) but also in another patient with a normal sacrum. In addition, despite asymmetry, FA and ADC were similar to controls also in these patients. CONCLUSION: This is the first study presenting quantitative data about the lumbosacral plexus in ARM. We think the most interesting finding of this study was observing the normal values even in the patients with severely malformed sacrum which is yet to be validated with further studies.

Long-term outcome and efficiency of symptom-selective approach to assess gastroesophageal reflux prior to gastrostomy in neurologically impaired children.

PURPOSE: To evaluate the necessity of preoperative screening for gastroesophageal reflux (GER) prior to gastrostomy in neurologically impaired children. METHODS: Medical records of neurologically impaired children, who have undergone laparoscopic gastrostomy between January, 2004 and June, 2018, were retrospectively reviewed. Before the year of 2014, all patients who required gastrostomy had been routinely screened for GER pre-operatively, but after the year of 2014, only the ones with GER-related symptoms were tested. The characteristics and outcomes of Routine Screening (RS) and Selective Screening (SS) periods were compared. RESULTS: There were 55 and 54 patients in the RS and SS periods, respectively. Demographics, primary pathologies, and mean follow-up durations (> 2 years) were similar. The rate of GER screening was significantly lower in the SS period (29.6% vs. 63.6%). The rate of Laparoscopic Nissen Fundoplication (LNF) combined with gastrostomy was significantly lower in the SS period (14.8% vs. 38.2%). During follow-up, the rates of new-onset GER symptoms (13% vs. 11.7%) and LNF requirement later on (6.5% vs. 8.8%) were statistically similar between the two periods. CONCLUSION: Routine screening for GER is not necessary prior to gastrostomy in neurologically impaired children. Symptom-selective screening algorithm is safe and efficient in the long term.

Intracellular localization of beta-catenin expression plays a possible prognostic role on the outcome of hepatoblastoma patients.

PURPOSE: Wnt/Beta-catenin pathway plays an essential role in liver development and regeneration. Abnormal activation in this pathway leads to development of hepatoblastoma (HB). Although its importance has invoked attention, its prognostic role is debatable. We aimed to evaluate the significance of intracellular localization of beta-catenin (BC) expression in the outcome of hepatoblastoma patients. METHODS: Medical records of HB patients between 2004 and 2018 were reviewed. Patients were grouped according to intracellular localization of BC expression by immunohistochemistry as being cytoplasmic or nuclear. Demographics, radiological images, PRETEXT classifications, vascular involvement, risk groups, chemotherapy responses, and survival rates were analyzed and compared between groups. RESULTS: There were 41 patients. Thirteen patients were excluded for unavailability of records in four, negative/unclear BC expressions in seven. Cytoplasmic expression of BC was observed in 17 patients whereas 13 patients displayed nuclear expression. Demographics were similar in both groups. Cytoplasmic BC expression was associated with poor chemotherapy response (p = 0.001) and increased vascular involvement (p = 0.0162) requiring more extensive surgeries (p = 0.039). CONCLUSION: Although the numbers are limited in our series, the intracellular localization of BC expression has been found to be a promising determining factor for hepatoblastoma prognosis. With larger patient series, more reliable results can be achieved.

Management of Childhood Thyroid Nodules: Surgical and Endocrinological Findings in a Large Group of Cases.

OBJECTIVE: The management of childhood thyroid nodules is still a big challenge for clinicians. In this study, we aimed to present our surgical and endocrinological experience in more than one hundred pediatric cases. METHODS: A retrospective analysis of patients admitted with a thyroid nodule between 2006 and 2014 was performed. Detailed ultrasonography and fine-needle aspiration biopsy (FNAB) were the cornerstones of the diagnostic approach. RESULTS: One hundred-three children (72 female, 31 male) with a mean age of 13.1±3.6 years (3-18 years) were admitted to our center. Management strategy was surgery in 58 patients and follow-up in 45 patients. Mean nodule size was 17±12.7 mm (2-45 mm). The diagnoses were listed as benign solitary nodule (48 patients), thyroid carcinoma (26 patients), multinodular goiter (23 patients), Hashimoto thyroiditis (4 patients), and Graves' disease (2 patients). Surgical procedures were nodulectomy/lobectomy (32 patients), total thyroidectomy (TT) (13 patients), or TT+ neck dissection (13 patients). The rate of malignancy was 25% in the total group and 44% in the surgery group. The malignancy rate was higher in patients younger than 12 years compared to older children (41% vs. 17%, p=0.040). Metastasis was seen in 38% of the malignant nodules. Postoperative complications were transient hypocalcemia (8%), permanent hypocalcemia (1.7%), and unilateral vocal cord paralysis (1.7%). Recurrence or mortality was not encountered in the 5.4±1.2-year follow-up period. CONCLUSION: Thyroid nodule in a child requires an aggressive diagnostic approach due to increased risk of malignancy and metastasis. Intraoperative frozen section examination must be done as a useful adjunct to determine the surgical strategy. Incidence of complications is small in thyroid surgery when performed by experienced surgeons.

Laparoscopic Treatment of Median Arcuate Ligament Syndrome: A Rare Cause of Chronic Severe Abdominal Pain.

Median arcuate ligament syndrome is a rare disorder characterized by chronic postprandial abdominal pain and weight loss caused by compression on celiac artery. A 17-year-old girl with chronic severe abdominal pain and weight loss was referred to our clinic. Other causes of chronic abdominal pain were investigated and excluded. The compression on celiac artery was detected on Doppler ultrasound and diagnosis was confirmed by computed tomography angiography. The patient underwent laparoscopic release of median arcuate ligament. There were no intraoperative complications; however, partial pain response was observed postoperatively that necessitated para-spinal ganglion blockage. The patient is symptom-free in 1-year follow-up period.

The Efficacy of Intralesional Steroid Injection in the Treatment of Corrosive Esophageal Strictures in Children.

PURPOSE OF THE STUDY: Esophageal dilatation can be insufficient in the treatment of severe corrosive esophageal strictures. In this study, we aimed to present the efficacy of intralesional steroid injection as an adjunct to dilatation therapy. MATERIALS AND METHODS: Retrospective analysis of children who underwent intralesional steroid injection between 2004 and 2014 was performed. Patients' age, type of corrosive substance, length of stricture, number of injection and dilatation sessions and complications were reviewed. The success was evaluated by the comparison of number, frequency, and requirement of dilatation therapy before and after injection. RESULTS: Intralesional steroid injection was performed to 32 children with a mean age of 3.6±2.5 years. The types of corrosive agents were alkali (24) or acid (8). Mean number of injection sessions was 2.5±1.1(1 to 6). Mean number of dilatation sessions was decreased from 10±8.8 to 5.4±4.6 after injection (P=0.003). Mean frequency of dilatations was extended from 3.6±0.9 weeks to 8.7±3.9 weeks (P=0.000). Dilatation treatment was successfully terminated in 25 of 27 children with short-segment strictures after injection (92%). Whereas all of the children with long-segment strictures could not resolved and finally required esophageal replacement (5 patients). One patient had transient cushingoid phenotype as a complication. There was not seen any major complication-like perforation. The mean follow-up period was 6±3 years. CONCLUSIONS: Intralesional steroid injection is an effective adjunct to dilatation in most of the children with short-segment strictures. It should be performed as a safe and efficient treatment option in patients with short-segment corrosive esophageal strictures resistant to dilatation therapy.