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1.
Eur Radiol ; 34(2): 780-787, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37597029

RESUMO

OBJECTIVES: To identify the diagnostic performance of clinical and radiological signs (on voiding cystourethrography [VCUG]) to detect posterior urethral valves (PUV) in the post-neonatal period. MATERIALS AND METHODS: One hundred eighteen males (median age = 0.8 years, range = 1 month-14 years, 48 toilet-trained) undergoing VCUG in a 2-year period were prospectively enrolled. Direct (dilated posterior urethra) and indirect (hypertrophied bladder neck, musculus interuretericus hypertrophy, and trabeculated appearance of the bladder wall) PUV signs on VCUG were assessed. Uroflowmetry was defined pathological by patterns suggesting infravesical obstruction. RESULTS: Twenty-two patients with direct, 28 with indirect PUV signs on VCUG, and one with normal VCUG but persisting micturition symptoms with pathological uroflowmetry underwent urethrocystoscopy and in 43/51 a PUV diagnosis was made (n = 22, 51.2%, with direct PUV signs). In 8/28 patients with indirect signs, PUV were not confirmed. Among non-toilet-trained patients, none of the clinical signs/symptoms was associated with PUV while among toilet-trained patients only pathological uroflowmetry (odds ratio, OR = 4.0 [95% confidence interval:1.2-13.2; p = 0.02]) and pathological uroflowmetry with history of urinary tract infection (OR = infinity) were significantly associated with PUV. Significant associations with PUV of direct and indirect signs on VCUG were found both in toilet-trained and non-toilet trained patients. Direct PUV sign had 100% specificity and sensitivity while indirect PUV signs showed sensitivity = 58.1% and specificity = 89.3%. The absence of any radiological sign had a negative predictive value = 98.5%. CONCLUSION: Only half of patients with endoscopy-confirmed PUV presents with direct sign of PUV on VCUG. Accounting for indirect PUV signs on VCUG and pathological uroflowmetry (in toilet-trained children) could improve the PUV detection rate. CLINICAL RELEVANCE STATEMENT: Indirect radiological PUV signs should be valorized when interpreting VCUG to improve the PUV detection rate. The absence of any radiological PUV (direct and indirect) sign on VCUG excludes PUV with a very high negative predictive value. KEY POINTS: • Worldwide agreement is that a non-dilated urethra on voiding cystourethrography excludes obstruction. • Half of patients with posterior urethral valves have non-dilated urethra on voiding cystourethrography. • Accounting for indirect signs of posterior urethral valves on voiding cystourethrography improves the diagnostic performance.


Assuntos
Infecções Urinárias , Micção , Humanos , Criança , Masculino , Recém-Nascido , Lactente , Uretra/diagnóstico por imagem , Bexiga Urinária/diagnóstico por imagem , Cistoscopia
2.
Gynecol Endocrinol ; 38(7): 612-614, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35532169

RESUMO

BACKGROUND: The presence of a rare lesion for pediatric population, encountered in twins, as far as we know, is an exceptional event. The event made the management of a pathology, for which there are no guidelines in the pediatric field, even more delicate. CASE REPORT: Two twin sisters came to our attention, a short time after each other, due to the presence of a voluminous abdominal mass. After complete resection, the histological diagnosis was that of a paraovarian cystoadenofibroma. DISCUSSION: In pediatric surgery being minimally invasive is mandatory. However in selected cases the only objective, especially speaking of pediatric patients, ais to be radical and to protect the adnexal structures.


Assuntos
Irmãos , Gêmeos , Adolescente , Criança , Humanos
3.
Pediatr Med Chir ; 45(1)2023 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-37428106

RESUMO

Urothelial bladder neoplasms (UBN) are uncommon in children and are poorly understood. Their management is contentious, and there are currently no pediatric guidelines available, making it difficult to envision a surgical approach that can be defined as the gold standard for the treatment of these diseases. Pneumovesicoscopy, which has already been used to treat other urological diseases, could be a promising treatment option for selected cases of this group of pathologies. We present our experience with three pediatric UBN cases in which pneumovesicoscopy was used for complete excision of a perimeatal papilloma in two cases and biopsy of a botryoid rhabdomyosarcoma in one. The pneumovesicoscopic approach, in our experience, provided a viable alternative technique for the management of selected cases of UBN.


Assuntos
Neoplasias Epiteliais e Glandulares , Neoplasias da Bexiga Urinária , Humanos , Criança , Neoplasias da Bexiga Urinária/diagnóstico , Neoplasias da Bexiga Urinária/cirurgia , Neoplasias da Bexiga Urinária/patologia , Bexiga Urinária/patologia , Bexiga Urinária/cirurgia , Biópsia
4.
J Clin Med ; 12(5)2023 Feb 28.
Artigo em Inglês | MEDLINE | ID: mdl-36902711

RESUMO

The aim of the study was to evaluate whether serum aldosterone levels or plasmatic renin activity (PRA) measured early in life (1-3 months) could predict a future surgical intervention for obstructive congenital anomalies of kidney and urinary tract (CAKUT). Twenty babies aged 1-3 months of life with suspected obstructive CAKUT were prospectively enrolled. The patients underwent a 2-year follow-up and were classified as patients needing or not needing surgery. In all of the enrolled patients, PRA and serum aldosterone levels were measured at 1-3 months of life and were evaluated as predictors of surgery by receiver-operating characteristic (ROC) curve analysis. Patients undergoing surgery during follow-up showed significantly higher levels of aldosterone at 1-3 months of life compared to those who did not require surgery (p = 0.006). The ROC curve analysis of the aldosterone for obstructive CAKUT needing surgery showed an area under the ROC curve of 0.88 (95%CI = 0.71-0.95; p = 0.001). The aldosterone cut-off of 100 ng/dL presented 100% sensitivity and 64.3% specificity and predicted surgery in 100% of cases. The PRA at 1-3 months of life was not a predictor of surgery. In conclusion, serum aldosterone levels at 1-3 months could predict the need for surgery during obstructive CAKUT follow-up.

5.
Children (Basel) ; 9(3)2022 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-35327790

RESUMO

We herein report a case of giant congenital hepatoblastoma in a 3-month-old male treated with neoadjuvant chemotherapy and hepatic resection. After considerable reduction of the tumor with chemotherapy, a right bloodless hemihepatectomy using saline-linked radiofrequency technology (SLRT) and without clamping of the hepatic pedicle was performed. Intraoperative blood loss was minimal, and consequently, no blood transfusions were required. The surgery lasted 140 min, and SLRT was used for a total of 60 min. No complications were observed during or after the surgery. In conclusion, congenital hepatoblastoma is a very rare cancer for which surgery is an essential therapeutic step, and in our presented case, we showed that SLRT allowed for a safe and effective bloodless liver resection.

6.
Children (Basel) ; 9(7)2022 Jul 08.
Artigo em Inglês | MEDLINE | ID: mdl-35884005

RESUMO

Introduction: Robotic surgery has shown explicit benefits and advantages in adults, but it is not yet strongly established in the pediatric population, even though its popularity is increasing, especially in the urologic field. Materials and methods: In this article we present our experience with the Da Vinci System (SI first and XI nowadays) at our pediatric institution in order to analyze our progress over the years. We considered all patients from the start of the robotic surgery program in 2016 until the end of 2021, dividing them into general abdominal surgery and genitourinary surgery. Analyzed data were the patient's demographic, details of surgery, and intra and post-operative complications. Results: The total number of patients (pts) included in this study was 76, of whom 40 (52%) were male and 36 (48%) were female. The mean age at surgery was 90.9 months (range 10-207 months), and the mean weight at surgery was 29.3 kg (range 9.5-68 kg). There were 18 general abdominal robotic surgeries and 58 genitourinary robotic surgeries performed. The most performed surgeries in these two categories were fundoplication for gastro-oesophageal reflux disease (11%) and Anderson-Hynes pyeloureteroplasty. The mean operative time was 224.2 min (range 72-530 min): the mean times in the two groups (general abdominal surgery and genitourinary surgery) were 165 min (range 84-204 min) and 194 min (range 95-360 min), respectively. A total of four (5%) minor complications were reported. The total conversions were two (2.6%) and the mortality rate was 0%. Conclusions: Pediatric robotic surgery is a field of considerable interest and it is rapidly expanding. In our experience, it is evident how the learning curve has increased gradually, but steadily, allowing us to advance from standardized surgery, such as fundoplication and pieloplasty, towards a more technically complex one, achieving the same good results. We believe that robotic surgery is very respectful of tissues and feasible, especially for reconstructive surgery. For these reasons, it could become of common use also in the pediatric population, overcoming impediments such as excessive cost and the lack of pediatric instruments, in order to be able to treat children with a progressively lower age and weight.

7.
J Pediatr Urol ; 17(3): 390.e1-390.e4, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33558173

RESUMO

BACKGROUND/INTRODUCTION: Ureteropelvic junction obstruction (UPJO) is the most frequent obstructive uropathy of the upper urinary tract in children. Video-sharing platforms have become a significant source of visual information for health care providers. Among these platforms, YouTube® (www.youtube.com), contains a high number of videos free of charge and represents one of the most important and known websites of video-sharing. YouTube® is a widely used open-access video sharing website that allows us to watch an unlimited number of video content, and to upload an infinitive number of videos. OBJECTIVE: This study aims to evaluate the educational quality of videos related to robotic pyeloplasty in pediatric age because an increasing number of videos addressing these procedures is now available on YouTube®. STUDY DESIGN: We performed a search on YouTube® by using the following keyword: "robotic pyeloplasty in children" on July 9, 2020. The first 50 videos were analyzed. The videos were classified according to the source in 1) academic (author/s was/were affiliated with a university), 2) physician (author/s who was/were not affiliated with a university), 3) patient, 4) commercial. All the videos were evaluated also according to the content in 1) surgical technique, 2) information about the surgery and disease 3) patient personal experience 4) advertisement. Duplicated videos and videos not in English were excluded. The search for videos was done based on the website's default settings in order of the proposed relevance. The reliability was evaluated using DISCREN and JAMA scores. The Global Quality Score (GWS) was used to assess the educational value. Time since upload, run time, like, dislike and number of views were recorded. RESULTS: The first 50 videos were analyzed. Seven videos (14%) did not meet our criteria and were excluded (three videos were duplicated while 4 out of seven were not in English). The mean DISCERN was 32.47 ± 12.24 (range 15-78). The mean JAMA Score was 2.1 ± 0.9 (range 0-4). Mean GQS was 2,12 ± 0.9. DISCERN and JAMAS and GQS scores of academic/physician sourced videos were significantly higher than the patient sourced videos (p = 0.037, p = 0.023, p = 0.017 respectively). Regarding content, the surgical technique had significantly higher DISCERN, JAMAS and GQS scores than videos based on patient experience (p = 0.012, p: 0.021, p = 0.023 respectively). CONCLUSIONS: Videos uploaded by Physicians and Academic Institutions show higher DISCERN and JAMAS and GQS compared to other sourced videos and should be considered more suitable for teaching respect to those originating from patients or non-physicians.


Assuntos
Procedimentos Cirúrgicos Robóticos , Mídias Sociais , Criança , Humanos , Disseminação de Informação , Reprodutibilidade dos Testes , Gravação em Vídeo
8.
Urol J ; 18(4): 466-468, 2021 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-33840088

RESUMO

The Disorders of Sex Differentiation (DSD) represent a wide range of congenital anomalies of the genitalia. Surgical treatment of these cases may become a challenge. We present a case of a 16-year-old boy with 46 XX DSD, SRY negative, presented with persistent dribbling incontinence, recurrent UTI and perineal pain. Past medical history included right orchiectomy, laparoscopic excision of uterus, fallopian tubes and left streak gonad at another institution at the age of 2 years. The native vagina was left in place. VCUG confirmed the presence of the residual vagina (8 cm in maximum length), connected with the bulbar urethra. Robotic-assisted laparoscopy of the vagina was performed with satisfying short and long-term results.


Assuntos
Transtornos do Desenvolvimento Sexual , Laparoscopia , Procedimentos Cirúrgicos Robóticos , Adolescente , Criança , Pré-Escolar , Transtornos do Desenvolvimento Sexual/cirurgia , Feminino , Humanos , Masculino , Pelve , Vagina/cirurgia
9.
Pediatr Med Chir ; 43(2)2021 Jun 16.
Artigo em Inglês | MEDLINE | ID: mdl-34148340

RESUMO

Bronchogenic Cysts (BCs) are benign congenital malformations commonly located in the mediastinum. In recent years the development of antenatal diagnosis has changed the BCs management allowing an earlier minimally invasive approach. We report a case of an asymptomatic 8-months-old girl with antenatal diagnosis of subcarinal posterior mediastinal BC. Thoracoscopic excision of the cyst was successfully performed. The management of antenatally diagnosed BCs is discussed. Thoracoscopic treatment of BCs is safe and effective with the advantage of a reduced morbidity compared to thoracotomy.


Assuntos
Cisto Broncogênico , Cisto Mediastínico , Cisto Broncogênico/diagnóstico por imagem , Cisto Broncogênico/cirurgia , Feminino , Humanos , Lactente , Cisto Mediastínico/diagnóstico por imagem , Cisto Mediastínico/cirurgia , Mediastino/diagnóstico por imagem , Mediastino/cirurgia , Gravidez , Tomografia Computadorizada por Raios X , Resultado do Tratamento
10.
J Pediatr Adolesc Gynecol ; 34(6): 872-875, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34157379

RESUMO

BACKGROUND: Yolk sac tumor (YST) is a malignant entity that often occurs in girls less than 3 years of age and is the most frequent type of primary extragonadal germ cell tumor. CASE: We describe the case of an 11-month-old girl who was referred to our center for vaginal bleeding with evidence of a uterine mass on ultrasonography. Preoperative investigations confirmed YST of the uterine cervix without metastasis. After 4 cycles of systemic chemotherapy, the patient was treated with laparoscopic trachelectomy (fertility-sparing surgery) without perioperative complications. SUMMARY AND CONCLUSION: After 12 months of follow-up, no residual mass was seen. The laparoscopic technique for trachelectomy for uterine cervix YST seems to be feasible and safe in children under 1 year of age.


Assuntos
Tumor do Seio Endodérmico , Laparoscopia , Traquelectomia , Neoplasias do Colo do Útero , Tumor do Seio Endodérmico/diagnóstico por imagem , Tumor do Seio Endodérmico/tratamento farmacológico , Tumor do Seio Endodérmico/cirurgia , Feminino , Humanos , Lactente , Neoplasias do Colo do Útero/cirurgia
11.
Swiss Med Wkly ; 151: w20513, 2021 06 07.
Artigo em Inglês | MEDLINE | ID: mdl-34161596

RESUMO

AIMS OF THE STUDY: To evaluate the efficacy of high-pressure balloon dilatation (HPBD) as treatment of primary obstructive megaureter (POM) in paediatric patients, we analysed the data of our institute from June 2018 to September 2019. METHODS: 14 patients, aged 5 months to 5 years, with POM were treated with HPBD. All patients had a distal ureter dilatation greater than 7 mm associated with obstructive features on a mercaptoacetyl triglycine-3 diuretic renogram scan, and a voiding cystourethrogram without vesicoureteral reflux. HPBD was performed in 12 patients, whereas 2 patients (14%), aged 5 and 6 months, required open surgical treatment because of failure to pass the balloon catheter through the vesicoureteral junction. The procedure was performed with a 5 Fr balloon catheter for two cycles of 5 minutes each at 17 atm. A double-J stent and a urinary catheter were inserted at the end of procedure in all patients. RESULTS: No operative complications or symptoms or recurrence were recorded in our series. The patients were generally discharged 24 hours after surgery. All the patients showed an improvement on ultrasonography at the postoperative follow-up, with no evidence of obstruction. During the procedure a clear stenotic ring was identified in 10 of the 12 patients, which disappeared in all 10 cases after the HPBD technique. CONCLUSIONS: Based on our experience, HPBD may be considered the first-line surgical approach in the treatment of POM in children, avoiding bladder surgery in most cases.


Assuntos
Ureter , Obstrução Ureteral , Criança , Dilatação , Endoscopia , Humanos , Lactente , Estudos Retrospectivos , Ureter/diagnóstico por imagem , Ureter/cirurgia , Obstrução Ureteral/cirurgia
12.
Children (Basel) ; 8(4)2021 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-33916416

RESUMO

Introduction: To report our experience in continent urinary diversions, we describe two cases we treated performing detubularized ureterosigmoidostomy. In children, in the case of malformations or neoplastic diseases affecting the bladder, the need for a cystectomy is not so frequent. When cystectomy becomes mandatory, there is a need to create a continent bladder diversion. Mainz pouch II and Cologne pouch are procedures that utilize a detubularized sigma as a reservoir in order to build up a continent neo-bladder. Materials and methods: This is a retrospective study performed at the Pediatric Surgical Unit of the Salesi Children's Hospital. In this work, we reviewed data about two patients who underwent surgery for the creation of a sigmoid neo-bladder by the Mainz pouch II and Cologne pouch techniques. Results: In our experience, we treated a girl who was affected by a bladder's rabdomiosarcoma and a girl born with a bladder exstrophy and treated at birth abroad. In both patients, a complete cystectomy was performed and a continent neo-bladder was created by a detubularized ureterosigmoidostomy. In the first case, we performed the Mainz pouch II technique and in the second, the Cologne pouch technique. Discussion: Different techniques have been developed with the main goal of the creation of an orthotopic neo-bladder, which has to be a low pressure reservoir with a continent sphincteric mechanism. Detubularized ureterosigmoidostomy is a good choice in pediatric patients. Our study, according to other works, shows that these procedure are safe with good long-term outcomes.

13.
Int J Med Robot ; 17(3): e2246, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33626232

RESUMO

OBJECTIVE(S): The incidence of urinary tract stone disease is steadily increasing in both adult and paediatric populations. This condition develops due to different factors: dietary or metabolic alterations, infection, and congenital anatomic malformations. Standard indications and treatments for children are analogous to the ones indicated for adults. Extracorporeal shock wave lithotripsy, ureterorenoscopy and percutaneous nephrolithotomy (PCNL) should be preferred to more invasive techniques. Moreover, the introduction of laparoscopic and robot-assisted laparoscopic approaches have improved surgical outcomes, lowering the bleeding risk with higher stone-free rates, even in complicated cases. Despite these well-known improvements, there are few reports regarding laparoscopic robot-assisted management for urinary tract stone disease in paediatric patients under the age of 10, especially with concomitant treatment of ureteropelvic junction obstruction and multiple calyceal stones. PATIENT AND METHOD(S): A 4-year-old child was referred for recurrent right abdominal flank pain, macroscopic haematuria and a previous history of urinary tract infections. A computed tomography of the abdomen showed right ureteropelvic junction obstruction associated with multiple unilateral stones located in the renal pelvis and in the interpolar renal calyces. Due to its complexity, we held a multidisciplinary meeting with paediatric surgeons and nephrologists to determine optimal treatment. As a result, a combined robot-assisted laparoscopic pyeloplasty (LP) and renal calculi holmium laser lithotripsy using a digital flexible ureteroscope through an abdominal robotic trocar was performed. No post-surgical complications were recorded, and the patient was discharged within 48 h following surgery. At subsequent regular follow-up examinations over a period of 24 months, no signs of recurrence were detected for both ureteropelvic junction obstruction and stone disease. RESULT(S): Robot-assisted LP with concomitant laser lithotripsy is a reasonable treatment option for designated young paediatric patients with challenging ureteropelvic junction obstruction complicated by urolithiasis, especially in cases where stones are not amenable with standard procedures.


Assuntos
Cálculos Renais , Laparoscopia , Litotripsia a Laser , Obstrução Ureteral , Adulto , Criança , Pré-Escolar , Humanos , Cálculos Renais/cirurgia , Procedimentos Cirúrgicos Robóticos , Resultado do Tratamento , Obstrução Ureteral/cirurgia
14.
Int Surg ; 95(3): 265-9, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-21067008

RESUMO

We present preliminary data from a prospective observational study on an initial group of 40 patients, selected from our Department, affected by grade III-IV hemorrhoids and treated with a new less invasive technique called Doppler-guided recto-anal repair [DG-RAR; Agency for Medical Innovations GmbH (AMI), Feldkirch, Osterreich, Austria]. This study was performed by analyzing bleeding, pain, and prolapse in the preoperative period and after surgery. Follow-up ranged from 5 to 37 months. We used this technique to treat the "vascular factor" with a Doppler-guided suture of the terminal branches of the hemorrhoidal arteries (HAL Doppler), and then we reduced hemorrhoidal prolapse [recto-anal repair (RAR)]. Recto-anal repair was performed with a special proctoscope with an oblique slot that when rotating shows a progressively wider portion of anorectal mucosa and submucosa in a longitudinal direction. Furthermore, this rotation enables the performance of a longitudinal pexy where the prolapse is located. The result is an immediate reduction of hemorrhoidal prolapse. Postoperative follow-up showed disappearance of pain and no bleeding. Relapse of prolapse occurred in 2 (5%) patients. Complications included 2 rectal impactions and 2 cases of thrombosis. The data appear encouraging for grade III-IV hemorrhoids treated with DG-RAR because of reduced trauma and a lower rate of complications with respect to other techniques used for prolapse reduction.


Assuntos
Hemorroidas/cirurgia , Proctoscopia/métodos , Cirurgia Assistida por Computador/métodos , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prolapso , Técnicas de Sutura , Ultrassonografia Doppler
15.
Urology ; 143: 238-240, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32422158

RESUMO

Fibroepithelial urethral polyp is a rare benign tumor more common in children than adult that can cause bladder outlet obstruction. We describe a 3-year-old boy who presented with intermittent acute urinary retention affected by a fibroepithelial urethral polyp managed with endoscopic transurethral resection by HOLMIUM laser; no complications neither recurrence were observed. To our knowledge this is the second pediatric case of fibroepithelial urethral polyp treated by HOLMIUM laser.


Assuntos
Lasers de Estado Sólido/uso terapêutico , Neoplasias Fibroepiteliais/cirurgia , Pólipos/cirurgia , Neoplasias Uretrais/cirurgia , Pré-Escolar , Endoscopia/métodos , Humanos , Masculino
16.
Int J Med Robot ; 16(5): 1-4, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32721101

RESUMO

BACKGROUND: Perivascular epithelioid cell tumours (PEComas) represent a rare group of mesenchymal tumours with an unpredictable outcome. They are usually observed in middle-aged women whilst they are very rare in children, with limited cases described. Due to the rare anatomical location in kidneys, a correct diagnosis and treatment is really challenging. METHODS: We observed and treated a case of kidney's PEComa in a 14-year-old boy. RESULTS: An individualized approach based on anatomical (3D CT-reconstructions) and histopathological (US-guided preliminary biopsy) features lead us to perform a successful robotic-sparing surgery enabling the preservation of two-thirds of the kidney involved. CONCLUSIONS: A meticulous preoperative planning in selected patients can lead to a minimally invasive approach even in some paediatric kidney's neoplasms.


Assuntos
Neoplasias Renais , Neoplasias de Células Epitelioides Perivasculares , Procedimentos Cirúrgicos Robóticos , Adolescente , Biópsia , Criança , Feminino , Humanos , Rim/diagnóstico por imagem , Rim/cirurgia , Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/cirurgia , Masculino , Pessoa de Meia-Idade , Neoplasias de Células Epitelioides Perivasculares/diagnóstico por imagem , Neoplasias de Células Epitelioides Perivasculares/cirurgia
17.
J Vasc Surg Venous Lymphat Disord ; 6(2): 230-236, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29233587

RESUMO

OBJECTIVE: Klippel-Trénaunay syndrome (KTS) is described in the literature as a complex syndrome characterized by various combinations of capillary, venous, and lymphatic malformations associated with limb overgrowth. In the first description by Maurice Klippel and Paul Trénaunay, tridimensional bone hypertrophy was believed to be the cause of limb enlargement. The purpose of this study was primarily to assess the presence of real bone hypertrophy as a cause of enlargement of the limb and to underline the rare presence of undergrowth of the affected limb in patients with KTS. METHODS: A two-center retrospective review including 17 KTS patients with various combinations of capillary, venous, and lymphatic malformation affecting the lower limb was performed. Differences in limb dimension were evaluated clinically. Width and length discrepancy of the affected limb was measured with radiologic imaging. RESULTS: We found an increase of length in the affected limb in 80% of the patients. The leg length discrepancy varied from 0.2 to 2.6 cm. The median leg length discrepancy was found to be 1.4 cm. Three patients had a reduced length of the affected limb. Girth enlargement of the affected extremity was noticed in 60% of the patients, and 2 of 17 patients had hypotrophy of the involved limb. Hypertrophy (an increase in both length and width) of the bone was found in none of our cases, and the circumferential enlargement of the affected extremity was related only to soft tissue enlargement. CONCLUSIONS: In the literature, KTS is considered the prototype of overgrowth syndromes associated with complex vascular malformations. The majority of our patients showed limb length increase associated with soft tissue enlargement without an increase of bone width; there were also two patients with limb undergrowth. A real bone overgrowth (an increase in both length and width) was not present in our patients. Therefore, we could consider the absence of real bone hypertrophy as probably a new aspect of such confusing and controversial definitions of KTS. In addition, it would be more accurate to classify KTS patients on the basis of their phenotypic features (type of vascular malformation, types of overgrown tissue) rather than by use of an outdated eponym.


Assuntos
Capilares/anormalidades , Síndrome de Klippel-Trenaunay-Weber/complicações , Desigualdade de Membros Inferiores/etiologia , Extremidade Inferior/irrigação sanguínea , Anormalidades Linfáticas/complicações , Vasos Linfáticos/anormalidades , Malformações Vasculares/complicações , Veias/anormalidades , Adolescente , Antropometria , Capilares/diagnóstico por imagem , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Itália , Síndrome de Klippel-Trenaunay-Weber/diagnóstico por imagem , Anormalidades Linfáticas/diagnóstico por imagem , Vasos Linfáticos/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Fenótipo , Estudos Retrospectivos , Espanha , Terminologia como Assunto , Malformações Vasculares/diagnóstico por imagem , Veias/diagnóstico por imagem , Adulto Jovem
18.
Swiss Med Wkly ; 137(23-24): 353-4, 2007 Jun 16.
Artigo em Inglês | MEDLINE | ID: mdl-17629806

RESUMO

A malposition of the median arcuate ligament (MAL) is a rare entity causing the celiac axis compression syndrome (CACS), first described by Harjola in 1963. The presence of anomalous fibrous diaphragmatic bands of the diaphragm compresses the celiac axis, especially during the expiration. In this report we present the fourth case in literature that was ever successfully treated by laparoscopy. A 38-year-old male presented with a history of intermittent epigastric pain, 15 kg weight loss caused by inappetence and frequent diarrhoea, over a 5-year period. The clinical examination revealed only a loud systolic bruit in the epigastrium, with loss of intensity during deep inspiration. Suspecting CACS, a spiral CT angiography was requested. The CT demonstrated the MAL crossing anteriorly to the celiac artery (CA) and the sagittal and the tridimensional reconstructions demonstrated the CA narrowing due to compression, while the superior mesenteric artery (SMA) was normal. The MAL was laparoscopically divided, releasing the celiac axis. The surgical time was 130 minutes, without significant blood losses. At 3-months follow-up, the CT-scan demonstrated no evidence of CACS with complete recovery.


Assuntos
Arteriopatias Oclusivas/diagnóstico , Arteriopatias Oclusivas/cirurgia , Artéria Celíaca , Laparoscopia , Ligamentos/anormalidades , Dor Abdominal , Adulto , Angiografia/métodos , Descompressão Cirúrgica/métodos , Humanos , Laparoscopia/métodos , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos , Síndrome , Tomografia Computadorizada Espiral , Redução de Peso
19.
J Laparoendosc Adv Surg Tech A ; 17(3): 302-6, 2007 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-17570774

RESUMO

BACKGROUND: The traumatic diaphragmatic hernia (TDH) may appear acutely after a blunt or penetrating trauma, or it can remain missed also for many years. The discussion about the utility of a laparoscopic repair in acute and chronic TDH is controversial. METHODS: In this paper, we present two cases of chronic TDH that were successfully treated with laparoscopy. The first patient was treated 1 year after a stab wound and the second one 10 years after a firearm injury. RESULTS: In both cases, the diaphragmatic defects were easily laparoscopically detected and treated. The defects were repaired with a direct running suture owing to the acceptable dimensions of the tears. The mean operative time was 135 minutes (range, 75-195). The blood loss during the operations was unremarkable. No intraoperative complications occurred, and the conversion rate was null. The postoperative course was uneventful in the first patient, whereas the second patient, owing to the intraoperative respiratory problems, needed an accurate respiratory monitoring in the Intensive Care Unit. The mean length of stay after the operation was 7 days (range, 6-8). At follow-up, the clinical examination and the chest X-rays documented no recurrence. CONCLUSIONS: We recommend the use of laparoscopy in left chronic TDH repair, performing a direct suture of the diaphragm when possible.


Assuntos
Hérnia Diafragmática/diagnóstico , Laparoscopia/métodos , Traumatismos Torácicos/complicações , Ferimentos Penetrantes/complicações , Adulto , Doença Crônica , Seguimentos , Hérnia Diafragmática/etiologia , Hérnia Diafragmática/cirurgia , Humanos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Atelectasia Pulmonar/terapia , Técnicas de Sutura , Tomografia Computadorizada por Raios X , Ferimentos por Arma de Fogo/complicações , Ferimentos Perfurantes/complicações
20.
Am Surg ; 72(2): 137-8, 2006 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-16536243

RESUMO

The positioning of mesh and plug is routinely performed in hernioplasty, but knowledge about the long-term side effects of plug-induced fibrosis on the vas is incomplete. This is the first case of a documented fibrotic vas deferens obstruction resulting from compression of the plug that was in direct contact with the vas. During subsequent repair for a recurrent hernia, the cord and the plug appeared enveloped in dense fibrous tissue. Microscopy confirmed gross surgical findings. To reduce this morbidity, we suggest a cremaster sparing surgery, avoiding the positioning of bioprosthesis in direct contact with the vas deferens. We also found that positioning of a flat mesh over the transversalis fascia will avoid the unnecessary contact between mesh and bare vas below the transversalis fascia.


Assuntos
Procedimentos Cirúrgicos do Sistema Digestório/efeitos adversos , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Doenças dos Genitais Masculinos/etiologia , Hérnia Inguinal/cirurgia , Implantação de Prótese , Ducto Deferente/patologia , Constrição Patológica/etiologia , Fibrose/complicações , Humanos , Masculino , Pessoa de Meia-Idade
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