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1.
J Pediatr Surg ; 59(3): 483-487, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-37858397

RESUMEN

BACKGROUND: Ganglioneuromas (GNs) are rare benign peripheral neuroblastic tumors (PNTs). We shared our institutional experience with childhood GNs. METHODS: Records of the children with PNTs between January 1995 and December 2021 were reviewed, and cases with histopathological diagnoses of GN were identified. Clinical, laboratory, radiological, and histopathological findings, image-defined risk factors (IDRFs), procedures, and overall outcomes were recorded. RESULTS: Of 668 cases with PNTs, 70 (10.4%) had GNs. The median age was 7.4 years (range, 2.6-15.7 years) (girls/boys, 41/29). Common presenting complaints were abdominal pain and cough; 33/70 cases (47.1%) were diagnosed incidentally. Primary tumors were in the abdomen in 41/70, the thorax in 25/70, the neck in 3 cases, and the pelvis in one. The median tumor size was 6.5 cm (range, 1.4-17). Fifty cases (71.4%) were staged as INRG-L1; 20 cases with IDRFs (15 single, five >1) were staged as INRG-L2. Complete and partial tumor resections were performed in 58/70 and 6/70 cases, while 6 had no resection. The overall complication rate was 17.1% (11/64). At a median follow-up of 9 years, five were lost to follow-up; 65 were alive. One patient with gross residue underwent total resection due to progression 13 years after the surgery, and one in the unresected group was lost to follow-up. Ten other cases without a complete resection experienced no tumor progression. CONCLUSIONS: Ganglioneuromas are benign PNTs, and most are free of IDRFs. Even without complete resection, long-term outcomes are excellent. Guidelines should be devised considering the high surgical complication rates and benign course of GNs. LEVEL OF EVIDENCE: Case series, IV.


Asunto(s)
Ganglioneuroma , Neuroblastoma , Niño , Masculino , Femenino , Humanos , Ganglioneuroma/epidemiología , Ganglioneuroma/cirugía , Ganglioneuroma/patología , Neuroblastoma/patología , Factores de Riesgo , Pelvis
2.
Turk J Pediatr ; 64(5): 940-945, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36305447

RESUMEN

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.


Asunto(s)
Neoplasias Óseas , Tumores de Células Gigantes , Pared Torácica , Femenino , Niño , Adolescente , Humanos , Neoplasias Óseas/diagnóstico por imagen , Neoplasias Óseas/cirugía , Costillas/diagnóstico por imagen , Costillas/cirugía , Tumores de Células Gigantes/patología , Pared Torácica/diagnóstico por imagen , Pared Torácica/cirugía , Tomografía Computarizada por Rayos X
3.
Pediatr Surg Int ; 38(9): 1303-1310, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-35821272

RESUMEN

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.


Asunto(s)
Neoplasias , Infección de la Herida Quirúrgica , Niño , Femenino , Humanos , Tiempo de Internación , Masculino , Neoplasias/complicaciones , Neoplasias/cirugía , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Periodo Posoperatorio , Estudios Retrospectivos , Infección de la Herida Quirúrgica/complicaciones
4.
ANZ J Surg ; 92(4): 742-746, 2022 04.
Artículo en Inglés | MEDLINE | ID: mdl-34664383

RESUMEN

BACKGROUND: Appendiceal neurendocrine tumours (NETs) are rare neoplasms and diagnosis is commonly incidental following appendectomy. We aimed to review our experience with appendiceal NETs. METHODS: Records of children with appendiceal NETs were reviewed and data concerning demographic characteristics, clinical findings, surgical procedures, histopathological findings, management and outcomes were recorded. RESULTS: Between 1985 and 2021, 33 cases with appendiceal NETs (median age 11.8 years, range 7.8-16; male/female = 10/23) were identified. All but one patients presented with abdominal pain, six had vomiting, four had fever and they underwent appendectomies with presumed diagnosis of acute appendicitis. Abscess drainage and appendectomy was performed in a 16-year-old girl for suspected right ovarian mass, and tumour was positive in the omentum. Histopathological diagnosis was classical carcinoid tumour (NET) in all cases. Median tumour size was 0.9 cm (n = 26, range, 0.1-3.5 cm); tumours were ≤1 cm in 19 cases, 2 cm in one, 3.5 cm in another case. Tumours were located in the tip (n = 11), body (n = 6) and base of appendix (n = 1) (in others data unavailable). In 28 patients with data, tumour extended to submucosa in five, to tunica muscularis in seven, to subserosa in six, to serosa in six, to mesoappendix in three, to periappendiceal fat in one. Three cases were lost to follow-up, 31 cases were alive (median follow-up 53 months). CONCLUSIONS: Paediatric appendiceal NETs do not behave aggressively and appendectomy alone is sufficient for tumours ≤2 cm regardless of local invasion. The need for further extensive surgery in tumours >2 cm also remains controversial.


Asunto(s)
Neoplasias del Apéndice , Apendicitis , Apéndice , Tumor Carcinoide , Tumores Neuroendocrinos , Adolescente , Apendicectomía/métodos , Neoplasias del Apéndice/diagnóstico , Neoplasias del Apéndice/patología , Neoplasias del Apéndice/cirugía , Apendicitis/diagnóstico , Apendicitis/patología , Apendicitis/cirugía , Apéndice/patología , Tumor Carcinoide/cirugía , Niño , Femenino , Humanos , Masculino , Tumores Neuroendocrinos/diagnóstico , Tumores Neuroendocrinos/patología , Tumores Neuroendocrinos/cirugía , Estudios Retrospectivos
5.
Surg Today ; 52(2): 330-336, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-34223990

RESUMEN

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.


Asunto(s)
Fuga Anastomótica/terapia , Quilo , Neoplasias/cirugía , Nutrición Parenteral Total , Complicaciones Posoperatorias/terapia , Somatostatina/administración & dosificación , Factores de Edad , Fuga Anastomótica/epidemiología , Fuga Anastomótica/etiología , Niño , Preescolar , Drenaje , Femenino , Humanos , Incidencia , Infusiones Intravenosas , Laparotomía/efectos adversos , Masculino , Complicaciones Posoperatorias/etiología , Estudios Retrospectivos , Toracotomía/efectos adversos , Factores de Tiempo
6.
Am J Med Genet A ; 185(11): 3427-3432, 2021 11.
Artículo en Inglés | MEDLINE | ID: mdl-34403180

RESUMEN

Pyloric atresia (PA) is a rare gastrointestinal anomaly that occurs either as an isolated lesion or in association with other congenital or hereditary anomalies. Familial occurrence of PA with epidermolysis bullosa (EB) has been well documented and variants in ITGA6, ITGB4, and PLEC are known to cause EB with PA. However, no gene variants have been defined in familial isolated PA. Five siblings with familial isolated PA are presented that suggest biallelic ITGB4 variants may underlie the development of PA without EB. Five siblings from two unrelated families with isolated PA were studied with exome sequencing (ES) to identify the genetic etiology in isolated familial cases. Exome sequencing was performed in one affected patient from each family. Validation and segregation studies were done by Sanger sequencing. Parents were first cousins in one family but there was no consanguinity in the other family. Type-2 PA was detected in both families and none of the probands had associated anomalies. All patients underwent successful gastroduodenostomy and have been under follow-up uneventfully. All patients had biallelic ITGB4 variants, c.2032G > T p.(Asp678Tyr) being a novel one. Biallelic ITGB4 variants may underlie the development of PA without associated EB. Further detection of variants in this gene may establish any possible genotype-phenotype correlations.


Asunto(s)
Epidermólisis Ampollosa/genética , Obstrucción de la Salida Gástrica/genética , Predisposición Genética a la Enfermedad , Integrina beta4/genética , Píloro/anomalías , Adulto , Alelos , Niño , Preescolar , Epidermólisis Ampollosa/patología , Femenino , Obstrucción de la Salida Gástrica/patología , Humanos , Lactante , Recién Nacido , Masculino , Píloro/patología , Hermanos , Secuenciación del Exoma
7.
Turk J Pediatr ; 63(3): 500-505, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34254496

RESUMEN

BACKGROUND: DICER1 syndrome is a hereditary cancer predisposition syndrome which is related DICER1 gene and may present a variety of manifestations. CASE: A prepubertal girl with ovarian Sertoli-Leydig cell tumor, thyroid follicular carcinoma, embryonal rhabdomyosarcoma of the cervix and lung cyst is presented. Genetic analysis demonstrated mutation (c.3377delC, c.71delC) in 14q32.13 loci and confirmed the diagnosis of DICER1 syndrome. CONCLUSION: The case is presented to emphasize the importance of early diagnosis of alterations in DICER1 gene and close follow-up for the development of DICER1 syndrome related pathologies, and necessity for genetic evaluation of the family.


Asunto(s)
Carcinoma , Rabdomiosarcoma Embrionario , Tumor de Células de Sertoli-Leydig , Neoplasias del Cuello Uterino , ARN Helicasas DEAD-box/genética , Femenino , Células Germinativas , Humanos , Masculino , Mutación , Rabdomiosarcoma Embrionario/genética , Ribonucleasa III/genética , Tumor de Células de Sertoli-Leydig/diagnóstico , Tumor de Células de Sertoli-Leydig/genética , Glándula Tiroides
8.
J Pediatr Urol ; 17(3): 400.e1-400.e7, 2021 06.
Artículo en Inglés | MEDLINE | ID: mdl-33593626

RESUMEN

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.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Paraganglioma , Feocromocitoma , Adolescente , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/cirugía , Adulto , Niño , Femenino , Humanos , Masculino , Paraganglioma/diagnóstico , Paraganglioma/epidemiología , Paraganglioma/cirugía , Feocromocitoma/diagnóstico , Feocromocitoma/cirugía , Estudios Retrospectivos , Centros de Atención Terciaria
9.
ANZ J Surg ; 91(5): 992-999, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33438328

RESUMEN

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.


Asunto(s)
Neoplasias de la Corteza Suprarrenal , Carcinoma Corticosuprarrenal , Neoplasias de la Corteza Suprarrenal/cirugía , Carcinoma Corticosuprarrenal/cirugía , Niño , Preescolar , Humanos , Estudios Prospectivos , Estudios Retrospectivos , Centros de Atención Terciaria
10.
Turk J Pediatr ; 62(5): 836-842, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33108088

RESUMEN

BACKGROUND: High-grade neuroepithelial tumor with areas resembling medulloepithelioma was diagnosed in an infant with coccygeal and inguinal masses. Hemihypertrophy is associated with Wilms tumor, hepatoblastoma and pancreatic tumors in children. CASE: The authors report on the first case of peripheral HNET associated with hemihypertrophy in an infant, with special discussion on histopathological differential diagnosis and management of this rare and highly malignant tumor. CONCLUSIONS: HNET should be included into the list of hemihypertrophy associated tumors. Complete surgical excision with free margins is essential for the successful treatment of such cases and should be tried in suitable cases at the time of diagnosis. Continued treatment should be decided individually on a case to case basis.


Asunto(s)
Neoplasias Renales , Tumores Neuroectodérmicos Primitivos , Tumor de Wilms , Sistema Nervioso Central , Niño , Humanos , Hipertrofia , Lactante , Neoplasias Renales/complicaciones , Neoplasias Renales/diagnóstico , Neoplasias Renales/cirugía
11.
Turk J Gastroenterol ; 31(8): 596-602, 2020 08.
Artículo en Inglés | MEDLINE | ID: mdl-32915148

RESUMEN

BACKGROUND/AIMS: Pediatric intestinal pseudo-obstruction (PIPO) is the most severe form of intestinal dysmotility in children. This study aims to present the cases of PIPO to discuss its diagnosis, management, and prognosis. MATERIALS AND METHODS: We retrospectively analyzed the medical records of the patients with PIPO between 2010 and 2018. RESULTS: A total of 7 patients were included. The admission age was 3 days-10 years. The complaints were abdominal distention and constipation in all the patients. All the patients had passed meconium in the first 48 hours of their life. An upper gastrointestinal (GI) series revealed slow transit in 6 patients and malrotation in 2 patients. Full-thickness rectum biopsies revealed normal ganglion cells. Neurological examination revealed postinfectious pandysautonomy in 1 patient. Furthermore, 2 patients are under follow-up with ileostomy and TPN, 1 patient is with enteral feeding and ileostomy, and 3 patients are stable with pyridostigmine, enemas. Moreover, 1 patient died because of sepsis. The prognosis was not significantly correlated with initial presentation time, lag time, and presence of extraintestinal manifestations (p>0.05). The prognosis was significantly better when fewer number of operations were performed (p=0.029) Conclusion: PIPO is a broad-spectrum disease group that is difficult to diagnose and treat. It is mandatory to rule out the secondary causes of diagnosis. Medical and surgical treatments are used to support the nutritional status, prevent sepsis, and restore the intestinal motility. The prognosis was better when the secondary causes were identified and fewer operations were performed.


Asunto(s)
Manejo de la Enfermedad , Seudoobstrucción Intestinal/diagnóstico , Seudoobstrucción Intestinal/terapia , Niño , Preescolar , Diagnóstico Diferencial , Femenino , Motilidad Gastrointestinal , Humanos , Lactante , Recién Nacido , Seudoobstrucción Intestinal/etiología , Masculino , Pronóstico , Estudios Retrospectivos
12.
Pediatr Hematol Oncol ; 37(8): 656-664, 2020 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-32705927

RESUMEN

Gastrointestinal tract is the most common extranodal site for childhood non-Hodgkin lymphomas (NHLs). However, primary gastric lymphoma (PGL) is very rare. We report our experience with PGL. Between 1972 and 2019, patients with PGL among 1696 NHL cases were evaluated retrospectively. Patient characteristics, treatments, and survival rates were recorded. We also reviewed the cases reported in literature. There were 16 PGL (11 males, five females) cases with a median age of 10 years. Most frequent complaints, similarly to the literature, were pain and vomiting. Hematemesis/melena and anemia were present in 20% of patients. Most common tumor location was antrum. Histopathological subtypes were Burkitt and non-Burkitt B-cell lymphoma in 43.75% and marginal zone lymphoma (MZL) in 6.25% of cases while mucosa-associated lymphoid tissue (MALT) and low-grade lymphomas constitute 15.3% of cases reported in the literature. In our series, Helicobacter pylori (H. pylori) was analyzed in only the case with MZL and found to be positive. However, H. pylori positivity was reported in 75% of the cases in the literature. H. pylori eradication, chemotherapy, and radiotherapy were applied in one, 14, and five patients. Subtotal gastrectomy with gastroduodenostomy/jejunostomy was performed in three patients. Gastrojejunostomy was done without tumor resection in two patients. Nine patients lived without disease for a median of 59 (12-252) months. Five-year EFS and OS were 69.6% and 64.3%, respectively. PGL constitutes 0.94% of our NHL cases. Interestingly, most of the cases in the literature were from Turkey. While adult PGL is mostly MALT lymphoma, most pediatric cases had high-grade histopathology. Although surgery and radiotherapy were applied earlier, chemotherapy alone is sufficient.


Asunto(s)
Infecciones por Helicobacter/complicaciones , Linfoma no Hodgkin/tratamiento farmacológico , Neoplasias Gástricas/tratamiento farmacológico , Adolescente , Niño , Preescolar , Femenino , Gastrectomía , Derivación Gástrica , Infecciones por Helicobacter/patología , Helicobacter pylori/aislamiento & purificación , Humanos , Linfoma de Células B de la Zona Marginal/tratamiento farmacológico , Linfoma de Células B de la Zona Marginal/microbiología , Linfoma de Células B de la Zona Marginal/patología , Linfoma no Hodgkin/mortalidad , Linfoma no Hodgkin/patología , Linfoma no Hodgkin/cirugía , Masculino , Estudios Retrospectivos , Neoplasias Gástricas/congénito , Neoplasias Gástricas/mortalidad , Neoplasias Gástricas/patología , Neoplasias Gástricas/cirugía , Tasa de Supervivencia , Turquía
13.
Int J Adolesc Med Health ; 33(3): 127-131, 2020 Jun 08.
Artículo en Inglés | MEDLINE | ID: mdl-32549138

RESUMEN

AIM: Primary spontaneous pneumothorax (PSP) is a rare pulmonary pathology that occurs in the absence of known lung disease. A retrospective study was performed to evaluate the results and outcome of PSP treatment in adolescents. METHODS: The cases with PSP from January 2004 to December 2017 were evaluated for age, sex, family and smoking history, clinical and radiological findings and results of treatment. RESULTS: Ten cases with PSP were included. The mean age of the patients was 15 years (10-17 years) and the male to female ratio was 9:1. Two of the patients (20%) had family history of PSP and four cases (40%) had smoking history. The initial complaints were chest pain (n=8), acute onset of cough (n=1) and breathing difficulty (n=1). Tube thoracostomy was performed in nine cases in which three of them were bilateral. Chest computed tomography (CT) demonstrated bullae (n=4; 40%) and subpleural blebs (n=2; 20%). Pleurodesis with talc was performed in four patients with pneumothorax for longer than a week in follow-up (n=4; 40%). Five cases had recurrent PSP within one year (n=5; 50%) and underwent pleurodesis with talc (n=4), autologous-blood (n=1) and bleomycin (n=1). Bleb excision was performed in two cases with persistent pneumothorax despite pleurodesis. CONCLUSION: Tube thoracostomy and oxygen supplementation, are considered as initial and adequate treatment of PSP in most of the adolescents. Prolonged air leaks require pleurosdesis as the first line treatment and surgical excision of blebs should be reversed for the patients who are unresponsive to other treatment options.


Asunto(s)
Evaluación de Resultado en la Atención de Salud , Neumotórax/cirugía , Adolescente , Niño , Femenino , Humanos , Masculino , Neumotórax/fisiopatología , Estudios Retrospectivos
14.
Pediatr Allergy Immunol Pulmonol ; 32(2): 52-55, 2019 Jun 01.
Artículo en Inglés | MEDLINE | ID: mdl-31508256

RESUMEN

Background: Foreign body aspiration (FBA) is a serious life-threatening condition in childhood. "Baby-led weaning (BLW)" is a popular method in which the babies are encouraged to self-feed to gain oral motor abilities. The role of BLW in FBA is controversial. A retrospective study was performed to evaluate the results of FBA in infants (<1 year of age) and its relation to the feeding method. Materials and Methods: Children who underwent bronchoscopy for FBA for the past 10 years were included. Infants (<1 year of age) were evaluated for age, gender, clinical findings, and the results of bronchoscopy. The type of feeding, including self-feeding or caregiver-assisted feeding, was noted. Results: The medical records of 826 patients who underwent bronchoscopy were evaluated. FBA was noted in 50.2% (n = 417) of cases. Only 9.07% (n = 75) of patients were <1 year of age and 67% (n = 50) of them had a foreign body according to the bronchoscopy. The mean age was 9 months (5-12 months) and 36% of them were male. When the feeding characteristics of patients were surveyed, 80% of cases aspirated when self-feeding and 14% aspirated during caregiver-assisted feeding. Conclusions: Self-feeding to promote oral motor function may cause FBA in infants. Emergent bronchoscopy is more common in infants and reveals the aspiration of foods that cannot be consumed safely in this age group.

15.
Turk J Gastroenterol ; 30(5): 467-474, 2019 May.
Artículo en Inglés | MEDLINE | ID: mdl-31061002

RESUMEN

BACKGROUND/AIMS: The present study aimed at investigating the long-term outcomes and prognostic factors of patients with biliary atresia (BA) diagnosed and followed at a single center. MATERIALS AND METHODS: Patients with BA treated during 1994-2014 at a large-volume pediatric tertiary referral center were reviewed retrospectively with regard to demographic, clinical, laboratory, and diagnostic characteristics for identifying the prognostic factors and long-term clinical outcomes. RESULTS: Overall, 81 patients (49 males, 32 females) were included. Mean age at diagnosis was 73.1±4.7 (median: 64) days. Of the patients included, 78 patients (96%) underwent a portoenterostomy procedure. Mean age at operation was 76.8±4.7 (median: 72) days. The surgical success rate was 64.8%. A younger age (either at diagnosis or surgery) was the only determinant of surgical success. The 2-, 5-, and 10-year overall survival (OS) rates, including all patients with or without liver transplantation, were 75%, 73%, and 71% respectively, whereas the 2-, 5-, and 10-year survival rates with native liver (SNL) were 69%, 61%, and 57%, respectively. Mean follow-up duration was 9.4±7.5 years. Successful surgery, presence of fibrosis and/or cirrhosis on the liver pathology, and prothrombin time [international normalized ratio (INR)] at presentation were independent prognostic factors for both OS and SNL. CONCLUSION: A younger age at diagnosis is strongly associated with surgical success in BA. Surgical success, the prothrombin time (INR) at presentation, and liver pathology are independent prognostic factors affecting the long-term outcomes in patients with BA. Therefore, timely diagnosis and early referral to experienced surgical centers are crucial for optimal management and favorable long-term results in BA.


Asunto(s)
Atresia Biliar/mortalidad , Portoenterostomía Hepática/mortalidad , Atresia Biliar/cirugía , Femenino , Humanos , Lactante , Trasplante de Hígado/mortalidad , Masculino , Portoenterostomía Hepática/métodos , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del Tratamiento
16.
Turk J Pediatr ; 59(2): 177-183, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-29276871

RESUMEN

Dogan G, Soyer T, Ekinci S, Karnak I, Çiftçi AÖ, Tanyel FC. Evaluation of surgically treated breast masses in children. Turk J Pediatr 2017; 59: 177-183. We aimed to define the diagnostic and treatment characteristics of breast masses among female children who had undergone surgical treatment in order to figure out which children should be followed conservatively or treated surgically. We reviewed retrospectively 64 female patients operated for breast masses under the age of 18 years between 1977 and 2013. Patient demographics, symptoms, physical examination findings, size of the mass and its relation with menstrual cycle, follow-up period before and after surgery, diagnostic characteristics, laboratory analysis, diagnosis before and after surgery, indications for surgery, histopathological characteristics of the mass, postoperative complications, and recurrence rate were recorded. Histopathologic investigations among 41 girls with complete hospital records showed that 26 had fibroadenoma (FA; 63.4%). Eleven girls among the other 23 cases with incomplete hospital records but with histopathological results have also had FA (47%). The majority of breast masses in childhood are FA, the frequency and duration of follow up is important. If there is a mass that will not regress, that continues to grow or does not reduce in size during follow up, and for those with family history of breast cancer to definitely exclude the possibility of malignancy, surgical treatment may be appropriate.


Asunto(s)
Neoplasias de la Mama/cirugía , Fibroadenoma/cirugía , Mastectomía/métodos , Adolescente , Biopsia con Aguja Fina , Neoplasias de la Mama/diagnóstico , Niño , Diagnóstico Diferencial , Femenino , Fibroadenoma/diagnóstico , Humanos , Recurrencia Local de Neoplasia , Estudios Retrospectivos , Resultado del Tratamiento , Ultrasonografía Mamaria
17.
J Pediatr Hematol Oncol ; 39(3): e140-e142, 2017 04.
Artículo en Inglés | MEDLINE | ID: mdl-28333838

RESUMEN

Ureteral extension of Wilms tumor (WT) is a rare occasion. The association of duplex collecting system and WT is extremely rare. Ureteral extension of WT in a duplex collecting system is an unreported entity to date. A 10-year-old girl presenting with WT extending into upper pole ureter of duplex system is reported to emphasize the importance of preoperative diagnosis to plan step by step surgery in this rare coincidental situation.


Asunto(s)
Obstrucción Ureteral/patología , Tumor de Wilms/patología , Tumor de Wilms/cirugía , Niño , Femenino , Humanos , Nefrectomía , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Ultrasonografía , Procedimientos Quirúrgicos Urológicos , Tumor de Wilms/diagnóstico por imagen
18.
Eur J Pediatr Surg ; 27(6): 526-532, 2017 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-28315283

RESUMEN

Aim The aim of this study was to evaluate the results of Swallowing Rehabilitation Protocol (SRP) on swallowing function (SF) of esophageal atresia and tracheoesophageal fistula (EA-TEF) patients with pharyngeal swallowing disorder. Materials and Methods In this study, 24 children with EA-TEF who had deglutitive and respiratory problems were grouped into either study (n = 12) or control group (n = 12) by basic randomization. Study group received the SRP including neuromuscular electrical stimulation, thermal tactile stimulation, and hyolaryngeal mobilization. The control group received nonnutritive stimulations. SF was evaluated with 3 mL liquid and pudding barium by videofluoroscopic swallowing study (VFSS) before and after 20 sessions of interventions. Results No statistical differences were found between groups in terms of descriptive characteristics including age, sex, weight, height, type of atresia, repair type, repair time, and start time of oral intake (p > 0.05). There were no statistical differences between groups in term of swallowing parameters (p > 0.05), except reflux (p = 0.004) according to VFSS findings. After 20 sessions of interventions, the study group showed improvement in penetration-aspiration scale scores, oral phase dysfunction, delay in swallowing reflex, and residue in valleculae and pyriform sinuses after pudding swallow. The control group showed improvement only in oral phase dysfunction. Conclusion SRP can be recommended to improve SF in patients with EA/TEF who have pharyngeal swallowing disorders.


Asunto(s)
Trastornos de Deglución/rehabilitación , Atresia Esofágica/rehabilitación , Fístula Traqueoesofágica/rehabilitación , Preescolar , Protocolos Clínicos , Terapia Combinada , Trastornos de Deglución/etiología , Trastornos de Deglución/fisiopatología , Atresia Esofágica/complicaciones , Atresia Esofágica/fisiopatología , Femenino , Humanos , Lactante , Masculino , Estudios Prospectivos , Fístula Traqueoesofágica/complicaciones , Fístula Traqueoesofágica/fisiopatología , Resultado del Tratamiento
19.
Eur J Pediatr Surg ; 27(4): 319-323, 2017 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-27699733

RESUMEN

Aim A retrospective study was performed to evaluate the clinical features, diagnostic methods, and treatment alternatives of childhood inflammatory myofibroblastic tumors (IMTs). Patients and Methods Patients who underwent surgical treatment for IMT between 2000 and 2015 were evaluated for age, sex, presenting symptoms, physical examination findings, diagnostic methods, treatment modalities, histopathologic findings, and results of surgical treatment during long-term follow-up. Results Eleven patients who underwent surgical treatment were included in the study. Male:female ratio was 7:4 and the mean age of the patients was 6.09 years (1-10 years). Presenting symptoms were respiratory difficulty, cough (n = 7, 63.3%), abdominal pain, vomiting (n = 2, 18.8%), loss of body weight (n = 1, 9.09%), palpable mass (n = 1, 9.09%), and rectal bleeding (n = 1, 9.09%). Ultrasonography (n = 4, 36.3%) and computed tomography (n = 9, 81.1%) were used for diagnosis. Localizations of tumors were lungs (n = 5, 45.4%), mediastinum (n = 2, 18.1%), spleen (n = 1, 9.09%), neck (n = 1, 9.09%), colon (n = 1, 9.09%), and rectum (n = 1, 9.09%). The mean size of mass was 6.6 cm (2-12 cm) and six patients were diagnosed with preoperative biopsy. Lung lobectomy (right lower lobe; n = 3, right middle and lower lobe; n = 2), total resection of mass with adjacent bowel (n = 2), partial splenectomy (n = 1), total resection of neck mass (n = 1), and incomplete resection (n = 2) were the choice of surgical treatment. Incomplete resection was performed in masses closely adjacent to atrium and mediastinal structures. In histopathologic evaluation, surgical margins were free of tumor in four cases, positive in six cases, and were not reported in one case. Anaplastic lymphoma kinase (ALK) positivity was detected in six cases, negative in two cases, and was not evaluated in three cases. Two cases who had residual mass with positive ALK received chemotherapy. Mean follow-up time was 68.2 months (5 months to 12 years). During follow-up, there was no recurrence or distant metastasis. Ten patients survived and one patient was lost to follow-up. Conclusion IMT is a rare tumor of childhood with a spectrum of clinical findings because of variable localization. Surgical treatment is the first choice of treatment. Patients with residual mass and ALK positivity may require medical treatment. In our series, long-term survival of patients was favorable in patients with total resection.


Asunto(s)
Granuloma de Células Plasmáticas/cirugía , Niño , Preescolar , Femenino , Estudios de Seguimiento , Granuloma de Células Plasmáticas/diagnóstico , Humanos , Lactante , Masculino , Estudios Retrospectivos , Resultado del Tratamiento
20.
Turk J Pediatr ; 58(2): 168-171, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27976557

RESUMEN

Medical records of all prepubertal patients who underwent vaginoscopy to rule out vaginal foreign body between 2004 and 2013 were reviewed retrospectively. All patients were evaluated by pediatricians prior to surgical consultation. Vaginoscopy is performed in the operating room under general anesthesia. During the study period, 20 girls with persistent vaginal discharge with a mean age of 6.8 years (1-13 years) underwent vaginoscopy to rule out vaginal foreign body. Six patients had bloody vaginal discharge and 4 had recurrent vaginal bleeding lasting for more than one month. Ten patients had purulent vaginal discharge lasting for 1-7 months. None of vaginal cultures revealed pathological bacteria or candida species. Preoperative imaging techniques revealed vaginal foreign body in one patient only. Vaginoscopy demonstrated vaginal foreign bodies in four patients. Foreign bodies were grass inflorescence, safety pin and undefined brownish particles (n=2), which may be pieces of toilet paper or feces. There was no complication related to vaginoscopy and removal of foreign body. Hymen integrity was preserved in all patients. Persistent or recurrent vaginal discharge in prepubertal girls should raise the suspect of vaginal foreign body. Continuous flow vaginoscopy is mandatory to detect and remove any vaginal foreign body. Early diagnosis would prevent complications secondary to long-standing foreign bodies.


Asunto(s)
Cuerpos Extraños/diagnóstico , Laparoscopía/métodos , Excreción Vaginal/diagnóstico , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Estudios Retrospectivos , Excreción Vaginal/etiología
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