Percutaneous Transcatheter Retrieval of Central Venous Port Fragments in Pediatric Patients; A Single-center Experience From the Pediatric Cardiology Department.

BACKGROUND: Split/fracture and embolization of central venous/shunt catheters are rare but serious complications in children. Percutaneous retrieval of intravascular foreign bodies is an important minimal invasive treatment. This study is aimed to represent our largest pediatric sample experience till now of 17 years from a single institution. Another aim is to compare the results regarding the removal or leaving in place of embolized or ruptured intravascular or cardiac venous catheter parts in children. PATIENTS AND METHODS: A total of 26 cases were included in this study. Any pediatric patient with normal coagulation parameters and a fractured catheter fragment was included in this study. Other intravascular foreign bodies related to interventional devices and/or pacemaker/implantable cardiac defibrillator leads were excluded from this study. RESULTS: Twenty-six patients, of whom 25 had oncologic diseases and 1 had a ventriculoatrial shunt, were included. The median age was 83.5 months (between 20 mo and 18 y) at treatment.Superior vena cava (9 cases), followed by the right atrium (5 cases), were the most two common sites of embolization for cardiovascular foreign bodies. The success rate of percutaneous retrieval was 92.3% in all patients. There were neither complications nor deaths. The retrieval technique revealed a predisposition for extraction through the femoral vein (96.1%) and using snare techniques (100%). Additional catheters like pigtail, National Institutes of Health, or ablation catheters were used for stabilization in selective cases in which the permanent central venous fragments stuck to the vessels. A tractional maneuver and capturing the ruptured material in the middle were other trick points for successful retrieval. Patients were asymptomatic in 76.9% of cases (20/26). CONCLUSION: Percutaneous retrieval of cardiovascular foreign bodies is a reasonable, safe, and effective way in children when the catheter fragments are free and mobile. It should be considered the preferred treatment option instead of surgery. In patients where catheter fragments are stuck and are adherent to vessels, it could be left, and followed up by anticoagulation. Novel techniques accompanied by an experienced team could be helpful in difficult cases.

Favourable outcomes of conservatively managed acute abdomen associated with multisystem inflammatory syndrome in children during the COVID-19 pandemic.

BACKGROUNDS: Abdominal pain is one of the most common symptoms of multisystem inflammatory syndrome in children (MIS-C). Abdominal pain can vary from mild to severe and may present as acute abdomen. Severe abdominal pain in patients with MIS-C should be differentiated from surgical causes of acute abdomen to prevent unnecessary surgery. METHODS: The diagnosis of MIS-C was based on WHO and CDC recommended criteria. Records of children treated for MIS-C between September 2020 and January 2021 were reviewed retrospectively. RESULTS: In a short time, we encountered seven patients who were diagnosed with MIS-C and showed acute abdomen findings. There were four male and three female patients. The median age was 9 years. Fever, abdominal pain and distension were present in all. The median duration of symptoms was 4 days. Five patients had general abdominal tenderness mimicking acute abdomen. Three patients had right lower quadrant tenderness mimicking acute appendicitis. After the initiation of immunomodulatory therapy and antibiotics, the physical examination findings were improved step by step in all. The median time to initiate oral feeding was 2 days. The median length of hospitalization time was 8 days. CONCLUSION: Serial abdominal examinations performed by the same surgeon enabled us to follow these patients conservatively and thus avoid unnecessary surgical intervention.

Giant cell tumor arising from the anterior arc of the rib: an extremely rare site in an adolescent girl.

BACKGROUND: Giant cell tumor is a rare and locally aggressive neoplasm of the long bones in children. Rib is the least frequently affected site, seen in less than 1% of all cases and most of them occur at the posterior arc. CASE: A 12-year-old girl presented with swelling and slight pain on the left inferior-anterior chest wall for two years. Physical examination revealed a giant, hard and fixed mass on the left chest wall. Hematological and biochemical test results were in normal limits but slight elevation of alkaline phosphatase level. Computed tomography of the chest showed a large expansive mass and lytic lesion with internal calcification arising from the anterior part of the 7th rib. En-bloc resection was performed including the 6th-8th ribs and a small part of the diaphragm. The pathological evaluation revealed giant cell tumor of bone. CONCLUSIONS: Herein, we aim to emphasize that giant cell tumor should be considered in the differential diagnosis of chest wall tumors in childhood whereby en-bloc resection and close follow up would be paramount.

Early postoperative complications in pediatric abdominal solid tumor surgery according to Clavian-Dindo classification.

PURPOSE: Postoperative period after abdominal solid tumor surgery is critical regarding complications. This study aimed to detect incidence and treatment of complications. METHODS: Single center retrospective study including years 2010-2019 for early postoperative complications were documented and graded according to Clavian-Dindo classification. RESULTS: The overall complication rate was 10% and they were chylous leak (n = 8, 2%), collection in surgical field (n = 8, 2%), acute renal failure (n = 7, 1.7%), hemorrhage (n = 5, 1.3%), intestinal obstruction (n = 4, 1%), surgical site infection (n = 3, 0.7%), thromboembolism (n = 3, 0.7%) and peripheric neuronal event (n = 2, 0.5%). Distribution of complications according to Clavian-Dindo classification was as follows: 2 grade I, 23 grade II, 9 grade IIIb, 5 grade IVa and 1 grade V. While age and percentage of benign or malignant disease were not different in groups with or without complications (p = 0.11, p = 0.24), males had more complications than female patients (p = 0.008). Having more than one surgery aiming tumor resection was associated with increased postoperative complications (p = 0.002). Incidence of complications were highest after extragonadal germ cell tumor (35%) and hepatic tumors (29%). CONCLUSIONS: Postoperative complications of abdominal tumor surgery are diverse in children. They are related with prolonged hospital stay and need for medical or surgical interventions. Number of surgeries, organ of origin and tumor type have an influence on risk of complications.

An unusual complication of tumor surgery: chylous leakage.

PURPOSE: This study was designed to evaluate the effectiveness of conservative treatment for chylous leak after tumor surgery and to propose a management algorithm. METHODS: The data of patients with postoperative chylous leak after tumor surgery in our institution between 2010 and 2019 were retrospectively reviewed. In this study, 469 laparotomies, 89 thoracotomies, and 57 cervical excisions were performed for tumor surgery in our institution. RESULTS: Twelve patients with a median age of 4 (IQR, 3-8) years had postoperative chylous leak. All patients received total parenteral nutrition for a median of 13 days. Five patients had intravenous somatostatin for a median of 14 days (IQR, 9-16) to decrease chyle production. Eventually, chylous leak ceased in all patients with conservative treatment and surgical drains were removed after no leak was observed with enteral feeding. CONCLUSIONS: The incidence of chylous leak in childhood tumor surgery is approximately 2%. Extended tumor resection and lymph node dissection lead to the injury of the delicate structures that drain chyle. Conservative treatment with total parenteral nutrition and somatostatin seems to be effective. In particular, somatostatin may be used in resistant cases. Conservative treatment can take up to 1 month. The algorithm consists of how to manage postoperative chylous leak in childhood.

Imaging findings of primary adrenal tumors in pediatric patients.

Apart from neuroblastomas, adrenal tumors are rarely seen in children. The most common adrenal tumors are adrenocortical carcinoma and pheochromocytoma. Adrenocortical carcinoma is usually a large heterogeneous, well-marginated mass with solid/cystic areas and calcifications, with poor prognosis. Most of the pheochromocytomas are benign tumors and usually show intense contrast enhancement, the pattern of which may be diffuse, mottled, or peripheral on computed tomography and magnetic resonance imaging. The purpose of this article is to evaluate primary nonneurogenic adrenal tumors.

Extraovarian juvenile granulosa cell tumor in a prepubertal child: novel location of a rare tumor.

Juvenile granulosa cell tumor (JGCT) is the most common type of sex cord stromal tumor arising from gonadal structures of children and young adults. We present a 3.5-year-old girl with JGCT located in retroperitoneum without ovarian involvement. Extragonadal occurrences of other sex cord stromal tumors have been rarely reported, but this is the first case of JGCT in an extragonadal location. We speculate the possible underlying mechanism of sex cord stromal tumor formation in extragonadal locations. Furthermore, clinical presentation, differential diagnosis and management of this tumor in childhood are discussed.

Approach to pheochromocytoma and paraganglioma in children and adolescents: A retrospective clinical study from a tertiary care center.

AIM: Pheochromocytoma (PCC) and paraganglioma (PGL) are rare tumors in childhood. They are catecholamine secreting tumors and present with signs or symptoms related to their excess. Most common signs and symptoms are hypertension, headache and diaphoresis. The management of children usually depend on experience of adulthood. This study is conducted to present the clinical characteristics, surgical management and outcome of childhood PCC and PGL in a tertiary care center. MATERIAL AND METHODS: We reviewed clinical records of all patients operated for PCC and PGL between 2000 and 2020 retrospectively. RESULTS: There were 18 children operated for PCC and PGL in the study period. The female to male ratio was 1:1. The median age at diagnosis was 13 (IQR, 9-15) years. The most common presenting symptoms were headache and diaphoresis. Hypertension was the most common sign. Three patients had von Hippel-Lindau (VHL). Tumors of two patients with VHL were detected during routine follow-up. Three patients had multifocal disease. Medical preparation for surgery was carried out in all patients. Antihypertensive treatments were administered preoperatively. Since the patients are at risk for postoperative hypotension due to chronic vasoconstriction and blood volume contraction, high salt diet was recommended. Intravenous normal saline at a rate of 3000 ml/m2 body surface area per day was started for intravascular volume expansion preoperatively. The mean duration for preoperative medication to achieve normal blood pressure was 22 days (range, 16-30). Twenty-five tumors were excised in eighteen patients. One patient who had bone metastases on diagnosis and is on I131MIBG therapy. The median follow-up time was 5.6 years (range, 1 months - 21 years). Five patients reached adulthood during the study period. Four of these had recurrent metastases (n = 2) and new tumors (pancreatic neuroendocrine tumor, n = 1 and pancreatic neuroendocrine tumor and renal cell carcinoma, n = 1) after the age of 18. CONCLUSION: Multidisciplinary approach is necessary to achieve safe surgical treatment and surveillance of PCC and PGL. Detection of associated familial cancer susceptibility syndromes and long-term follow-up is essential to detect late recurrences and new tumors.

Adrenocortical tumours in children: a review of surgical management at a tertiary care centre.

BACKGROUND: Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management. METHODS: Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively. RESULTS: The median age of 24 children was 78 months. Fourteen patients had adrenocortical carcinoma, nine had adrenocortical adenoma and one had neuroendocrine differentiation of ACT. Endocrine dysfunction was noted in 79% of the patients. Five patients had preoperative chemotherapy but none had a decrease in tumour size. Transabdominal approach was used in all but two patients who had thoracoabdominal incision for excision of giant tumours and ipsilateral lung metastases. Two patients had visceral excision to achieve R0 resection. Five patients, four of whom had spillage and one with partial resection died of widespread disease. Two patients with stage 4 adrenocortical carcinoma are still on chemotherapy. All patients with stage I-III disease who had total excision without spillage (n = 17) are disease-free for 2-170 months. CONCLUSIONS: Our results show the importance of excision in ACT without spillage for survival. However, multicentre prospective studies should enhance the knowledge of children about ACT and develop alternative therapies for stage III and IV cases.

Anteroposterior Duplicated Exstrophy: A Case Report.

Duplicated bladder exstrophy is an extremely rare variant of the exstrophy/epispadias complex. Duplicated exstrophy defines an exstrophic mucosal plate in hypogastric area with a normal closed bladder. We present a unique case of an anteroposterior duplicated exstrophy in a female newborn.

Isolated thoracoschisis: Case report.

Ardiçli B, Karaman A, Özyazici A, Zenciroglu A, Okumus N. Isolated thoracoschisis: Case report. Turk J Pediatr 2017; 59: 217-220. Thoracoschisis is a rare congenital anomaly that refers to a congenital fissure of the chest wall. It is frequently accompanied with other congenital defects of the limbs and the abdominal wall as part of the limb-body wall complex, which is exencephaly/encephalocele and facial clefts, thoracoschisis and/or abdominoschisis and limb defects. Isolated thoracoschisis is a rare entity. We present a case of isolated thoracoschisis. A 24-week gestational age boy presented with a 3 cm chest wall defect in the left lateral 10th intercostal space and intestines herniating through the defect. There was no history of maternal drug use during pregnancy. Birth weight was 500 g. He underwent surgery. The intestines were reduced via the thoracic wall defect.

Sclerotherapy with bleomycin versus surgical excision for extracervical cystic lymphatic malformations in children.

PURPOSE: Sclerotherapy (ST) with bleomycin is an effective treatment for cervical cystic lymphatic malformations (LM) in children. However, its efficacy for treating extracervical cystic LM in children has not been investigated adequately. This retrospective study compares the efficacy of ST with surgery for treating extracervical cystic LM in children. METHODS: The subjects of this study were children treated for extracervical cystic LM at our hospital between 1970 and 2013. We evaluated retrospectively the hospital records of these children for age, gender, presenting symptoms, location of the lesion, radiological findings, treatments, complications, duration of hospitalization, and outcome. RESULTS: We analyzed the records of 70 children (M:F = 1:9) with a mean age of 52.57 ± 54.87 months (range 1-204 months). The number of children treated by surgery alone, ST alone, and surgery plus ST was 53 (77 %), 13 (18 %), and 4 (5 %), respectively. Surgery comprised total excision (n = 41), near-total excision (n = 9), partial excision (n = 6), and incisional biopsy (n = 1). The complication and recurrence rates were lower, the complete response rate was higher, and the length of hospitalization was shorter in the ST group than in the surgery group (5 vs. 15 % and 8 vs. 17 %, respectively, p < 0.05; 91 vs. 77 %, respectively, p = 0.05; and 2.42 ± 1.67 vs. 13.57 ± 16.24 days, respectively, p = 0.03). CONCLUSION: ST is as safe as surgery for extracervical macrocystic or mixed LMs in children, but is much more effective with higher success rates and lower recurrence rates. Thus, ST provides a cost-effective and appropriate mode of treatment for children with extracervical cystic LM.

An interesting fistula tract presenting with recurrent gluteal abscess: instructive case.

A fistula extending from the gluteus to penis is an extremely rare entity. In this paper, we have highlighted novel variant of congenital penile to gluteal fistula complicated with gluteal and penoscrotal abscess in a previously healthy boy. A fistulous tract extending from the gluteus to penis has been shown by fistulogram. Bleomycin has been used in fistula tract with successful results in our patient.

Composition of parenteral nutrition solution affects the time of occurrence but not the incidence of cholestasis in surgical infants.

Parenteral nutrition-associated cholestasis (PNAC) is one of the most important complications of parenteral nutrition (PN). This study was conducted to define the incidence, characteristics and precipitating factors of PNAC in infants treated with two different PN regimens in the pediatric surgery department. The records of infants who received two different PN regimens during January 2000-May 2008 (Group 1) and June 2008-May 2012 (Group 2) were reviewed retrospectively. Patients with and without PNAC comprised the study (Groups 1a-2a) and control groups (Groups 1b-2b), respectively. With Group 1 (n=109) and Group 2 (n=46) constituted as indicated, Group 1a (n=13), Group 1b (n=32), Group 2a (n=6) and Group 2b (n=8) were evaluated. The incidence of PNAC was 14.1% (n=22), and the type of PN regimen did not affect the incidence (14.6% vs. 13.0%, p=0.39). The duration of PN was longer in Group 1a than in Group 1b (34 ± 3 7 vs. 13 ± 10 days, p=0.01), and longer in Group 2a than in Group 1a or Group 2b (146 ± 148 vs. 34 ± 37 days, p=0.02, and 14.0 ± 5.9 days, p=0.002). PNAC developed later in Group 2a than in Group 1a (42.0 ± 24.6 vs. 9.0 ± 8.3 day, p=0.02). The lipid dosage was higher in Group 1a than in Group 1b (3.5 ± 0.7 vs. 3.45 ± 0.54 g/ kg/day, p=0.001) or Group 2a (2.2 ± 0.4 g/kg/day, p=0.01). The lipid dosage was also higher in Group 2a than in Group 2b (p=0.001). Two different regimens resulted in similar PNAC rates. The composition of nutrients in PN solution affects the time of occurrence but not the duration of PNAC. PNAC should be detected as early as possible and treated promptly.

Laparoscopic detorsion of isolated idiopathic fallopian tube torsion: conservative treatment in a 13-year-old girl.

Isolated fallopian tube torsion (IFTT) is a rare entity in adolescent girls. Invasive surgery, salpingectomy, was used during the initial surgery or at the time of recurrent torsion in 87% of previously reported cases. Herein, the authors present an adolescent premenarcheal girl with idiopathic IFTT, which was treated by laparoscopic detorsion, to emphasize that tubal torsion should be included in the list of differential diagnoses of abdominal pain in adolescent girls. Minimally invasive, organ-saving surgery should be done, and the child should be followed up for recurrent torsion.

Ureterovesical junction obstruction causes increment in smooth muscle contractility, and cholinergic and adrenergic activity in distal ureter of rabbits.

BACKGROUND/PURPOSE: The controversy in management of primary obstructed megaureter necessitates further elucidation of the underlying pathophysiology. We evaluated smooth muscle contractility, and cholinergic, adrenergic and serotonergic activity of rabbit distal ureters after ureterovesical junction (UVJ) obstruction. METHODS: Sham (SH) operation, partial obstruction (PO) and complete obstruction (CO) of the right UVJ were performed in rabbits. Three weeks later, distal ureters were isolated; spontaneous contractions (SC), contractile responses to electrical field stimulation (EFS), high KCl, carbachol, phenylephrine and serotonin were recorded. RESULTS: SC amplitudes increased in CO compared to PO and SH (p<0.001). SC frequency was higher in CO (p<0.05). EFS-induced contraction amplitudes were greater in CO than other groups (p<0.05). High KCl-induced contractions were greater in CO (p<0.001) and PO (p<0.01). Carbachol-induced contractility was enhanced in CO and PO (p<0.05). Contractile response to phenylephrine was greater in CO than other groups (p<0.05). Serotonin induced contractile responses in CO and PO, greater in CO (p<0.05). UVJ obstruction also increased spontaneous contractility in contralateral PO and CO ureters. CONCLUSIONS: UVJ obstruction increased spontaneous and neurotransmitter-induced contractions in an obstruction grade-dependent manner. Obstruction also altered contractility of the contralateral ureters. Our findings may serve to provide further understanding of the pathophysiology of megaureter.

Papillary thyroid carcinoma does not have standard course in children.

BACKGROUND AND PURPOSE: Papillary thyroid carcinoma has a favorable outcome in children. Recent experience with young children cases with early recurrences, after the total thyroidectomy and excision of palpable lymph nodes, in contrast to usual course of disease in adolescent cases, prompted us to review our experience on papillary carcinoma surgery. MATERIALS AND METHODS: Sixteen children who underwent surgery for papillary carcinoma between 1997 and 2010 were included. The charts and surgery notes were evaluated retrospectively and age, sex, complaints and physical findings at presentation, past medical history, results of laboratory tests, imaging findings, aspiration biopsy, extent of disease, details of surgery, complications and postoperative course were noted. RESULTS: Male to female ratio was 3:1. The mean age was 10.9 years (range, 5-16). The presenting symptom was palpable swelling in the neck (n = 12) or asymptomatic nodule was detected incidentally (n = 4). Physical examination findings were palpable nodule (n = 7), lymphadenopathy (n = 6) or both (n = 4). Thyroid ultrasound (US) revealed nodule (n = 9), heterogenisity of the parenchyma and enlargement of thyroid (n = 9), and microcalcifications (n = 5). Fine-needle aspiration biopsy was performed in 12 patients and was suggestive for malignancy in most cases (91%). Pulmonary metastasis was detected at presentation in two patients and appeared after thyroidectomy in one patient. Complementary thyroidectomy was performed in nine patients because the total thyroidectomy with or without cervical lymph node dissection was the treatment of choice (n = 15). Iodine scan was performed 4 weeks later following thyroidectomy. Radioactive iodine ablation (RIA) therapy was given to 11 patients who had residual or recurrent disease. Postoperative complications were permanent hypoparathyroidism (n = 4), recurrent laryngeal nerve injury (n = 3), lymphorea (n = 1) and Horner's syndrome (n = 1). CONCLUSION: Total thyroidectomy and excision of affected lymph nodes is the current mode of surgical management for thyroid papillary carcinoma in children. However, especially in young children (<10 years), modified lymph node dissection should be added to total thyroidectomy in order to avoid leaving the residual tumor foci which can impair the efficacy of the ablation therapy.