Surgical Treatment on Subungual Osteochondromas in Paediatric Feet: A Case Series Study.

Subungual osteochondroma (SO) is an infrequent and non-malignant bone tumour of the distal phalanx, especially prominent in paediatric populations. The aim of this research was to describe a case series of paediatric feet with SO which received surgical treatments. The secondary purpose was to compare these descriptive data by sex distribution. METHODS: Twenty-three paediatric feet with SO confirmed by clinical or radiological features received surgical treatment. Socio-demographic (age, sex, height, weight and BMI) and clinical features (side, location, tumour or pain presence, and nail lift before surgery, as well as recurrence and adverse effects at one month after intervention) were reported. RESULTS: Regarding clinical features before intervention, the most frequent locations of SO were the first toe (86.8%) and the right lower limb (56.5%). In addition, the presence of the tumour, pain and nail lift showed a prevalence of 91.3%, 69.5% and 47% of the study sample, respectively. Considering clinical features at one month after intervention, the most frequent adverse effect was the pain presence (69.5%). In addition, one case (4.4%) presented ulceration. Only one patient (4.4%) suffered from recurrence with a new tumour. There were not statistically significant differences by sex distribution (p > 0.05). CONCLUSIONS: This novel study showed that surgery treatment for SO in paediatric populations presented a very low recurrence degree with minor adverse effects and without differences by sex distribution. Thus, further randomized clinical trials should be carried out in order to determine the effectiveness of this intervention in this special population.

Guía de práctica clínica Ibero-Latinoamericana sobre la esofagitis cáustica en Pediatría: aspectos terapéuticos (2a. Parte) / Ibero-Latinamerican Clinical Practical Guidelines on pediatric caustic esophagitis: therapeutical aspects (Part 2)

Resumen: La ingestión de cáusticos representa un grave problema médico-social por las consecuencias devastadoras e irreversibles que puede producir en el tracto digestivo superior. En Iberoamérica no se han publicado datos fidedignos sobre la incidencia o la prevalencia de lesiones inducidas por cáusticos. La información disponible sobre la presentación clínica, diagnóstico, tratamiento y pronóstico se basa en series retrospectivas de casos y, de hecho, su manejo clínico se sustenta en muchos casos fundamentalmente en la opinión de expertos. Recientemente como una iniciativa de la Sociedad Latinoamericana de Gastroenterología, Hepatología y Nutrición Pediátrica (SLAGHNP) y con la co laboración de colegas de la Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediá trica (SEGHNP), hemos diseñado una Guía de Práctica Clínica (GPC) la cual incluye una serie de enunciados y recomendaciones dirigidos a optimizar la atención a los pacientes y que se basan en la revisión sistemática de la evidencia. En dos (2) manuscritos sucesivos nos hemos enfocado primero, en los aspectos fisiopatológicos y de diagnóstico clínico-endoscópico de la esofagitis cáustica en niños (1a. Parte) y en segundo lugar, en los aspectos más relevantes del tratamiento (2a. Parte). Esperamos esta guía se convierta en una herramienta útil para el clínico en el difícil proceso de toma de decisio nes a la hora de evaluar un paciente posterior a la ingesta de una sustancia cáustica.

Abstract: Caustic ingestion represents a serious social-medical problem due to the devastating and irreversible consequences it can produce in the upper digestive tract. In Ibero-America, there are no published reliable data on the incidence or prevalence of caustic-induced injuries, and most of the available information on clinical presentation, diagnosis, treatment, and prognosis is based on retrospective clinical series and, indeed, its clinical management is often based primarily on expert opinion. Re cently as an initiative of the Latin American Society for Pediatric Gastroenterology, Hepatology and Nutrition (LASPGHAN) and with the cooperation of the Spanish Society for Pediatric Gastroente rology, Hepatology and Nutrition (SEGHNP), we have designed a Clinical Practice Guideline that include a series of statements and recommendations aimed at optimizing patient medical care which is based on the systematic review of evidence. Two (2) separate papers focused on the evaluation of physiopathological and clinical-endoscopic diagnostic features of caustic esophagitis in children (1st. Paper) and, on the other hand, the most relevant therapeutic considerations (2nd. Paper). We expect this guideline to become a useful tool for the physician in the difficult decision-making process when assessing patients after caustic ingestion.

Guía de práctica clínica Ibero-Latinoamericana sobre la esofagitis cáustica en Pediatría: fisiopatología y diagnóstico clínico-endoscópico (1a Parte) / Ibero-latinamerican clinical practical guidelines on pediatric caustic esophagitis: physiopathology and clinical-endoscopic diagnosis (1st Part)

Resumen: La ingestión de cáusticos representa un grave problema médico-social por las consecuencias devastadoras e irreversibles que puede producir en el tracto digestivo superior. En Iberoamérica no se han publicado datos fidedignos sobre la incidencia o la prevalencia de lesiones inducidas por cáusticos. La información disponible sobre la presentación clínica, diagnóstico, tratamiento y pronóstico se basa en series retrospectivas de casos y, de hecho, su manejo clínico se sustenta en muchos casos fundamentalmente en la opinión de expertos. Recientemente como una iniciativa de la Sociedad Latinoamericana de Gastroenterología, Hepatología y Nutrición Pediátrica (SLAGHNP) y con la co laboración de colegas de la Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediá trica (SEGHNP), hemos diseñado una Guía de Práctica Clínica (GPC) la cual incluye una serie de enunciados y recomendaciones dirigidos a optimizar la atención a los pacientes y que se basan en la revisión sistemática de la evidencia. En dos (2) manuscritos sucesivos nos hemos enfocado primero, en los aspectos fisiopatológicos y de diagnóstico clínico-endoscópico de la esofagitis cáustica en niños (1a. Parte) y en segundo lugar, en los aspectos más relevantes del tratamiento (2a. Parte). Esperamos esta guía se convierta en una herramienta útil para el clínico en el difícil proceso de toma de decisio nes a la hora de evaluar un paciente posterior a la ingesta de una sustancia cáustica.

Abstract: Caustic ingestion represents a serious social-medical problem due to the devastating and irreversible consequences it can produce in the upper digestive tract. In Ibero-America, there are no published reliable data on the incidence or prevalence of caustic-induced injuries, and most of the available information on clinical presentation, diagnosis, treatment, and prognosis is based on retrospective clinical series and, indeed, its clinical management is often based primarily on expert opinion. Re cently as an initiative of the Latin American Society for Pediatric Gastroenterology, Hepatology and Nutrition (LASPGHAN) and with the cooperation of the Spanish Society for Pediatric Gastroente rology, Hepatology and Nutrition (SEGHNP), we have designed a Clinical Practice Guideline that include a series of statements and recommendations aimed at optimizing patient medical care which is based on the systematic review of evidence. Two (2) successive papers focused on the evaluation of physiopathological and clinical-endoscopic diagnostic features of caustic esophagitis in children (1st. Paper) and, on the other hand, the most relevant therapeutic considerations (2nd. Paper). We expect this guideline to become a useful tool for the physician in the difficult decision-making process when assessing patients after caustic ingestion.

[Epigenetics as a diagnostic and prognostic tool for pyeloureteral stenosis.] / La epigenética como herramienta diagnóstica y pronóstica en la estenosis pieloureteral.

OBJECTIVE: In the last few years, numerous studies have focused on the genetics of the renal system. Betchel et al in 2010 demonstrated that methylation, as a epigenetic phenomenon, would be involved in the perpetuation of fibrosis. In our study, we want to demonstrate whether epigenetics is related to pyeloureteral stenosis and, if that is the case, if it could be used as prognostic and diagnostic biomarker. METHODS: This is a descriptive observational and cross-sectional study that analyzed the methylation in DNA extracted from pyeloureteral junction samples obtained from surgery in pediatric patients in the period from 1999 to 2015, resulting in a total of 20 patients. Clinical data were analyzed using correlation tests and they were grouped with a free access software statistical phylogenetic package called PHYLIP. The selected genes for methylation-specific PCR (MSP) were the following: p16, RASSF1A, MGMT, Cyclin D-2, HIN-1, E-Cadherin and RASAL-1. RESULTS: The clinical-radiological data analyzed phylogenetically by the PHYLIP program established 7 groups of patients. The results of methylation showed a considerable proportion of aberrant methylation in the promotor region of the genes p16 (25%), MGMT (15%), E-Cadherin (25%), HIN-1 (25%) and RASAL-1 (35%). The association of the clinical-radiological groups with methylation/non-methylation states of each gene was also analyzed. CONCLUSIONS: This study demonstrates that methylation does have a role in fibrosis developed in pyeloureteral stenosis. Two clinical patterns of poor prognosis associated with two epigenetic methylation cluster. RASAL- 1, E-Cadherin, HIN-1 and p16 would be candidates for future studies on their prognostic implications in pyeloureteral stenosis.

OBJETIVO: En los últimos años, numerosos estudios se han centrado en la genética del sistema renal. Betchel et al. en 2010, demostraron como la metilación, fenómeno epigenético, estaría implicado en la perpetuación de la fibrosis. En nuestro estudio queremos demostrar si la epigenética tiene relación con la estenosis pieloureteral y en caso de ser así, si podría ser utilizada como material pronóstico y diagnóstico. MATERIAL Y MÉTODOS: Se ha realizado un estudio descriptivo observacional o transversal en el que se analizó la metilación en el ADN extraído de las muestras de unión pieloureteral en pacientes pediátricos obtenidas durante la cirugía entre 1999 y 2015, resultando un total de 20 pacientes. Los datos clínicos-radiológicos se analizaron según correlación y agrupación de los mismos mediante un paquete software filogenético/estadístico denominado PHYLIP de acceso libre gratuito. Los genes seleccionados sobre los que se realizó la PCR específica de metilación (MSP) fueron: p16, RASSF1A, MGMT, Ciclina D-2, HIN-1, E-Cadherina y RASAL-1. RESULTADOS: Los datos clínico-radiológicos analizados filogenéticamente mediante el programa PHYLIP establecieron 7 grupos de pacientes. Los resultados con respecto a la metilación mostraron una proporción considerable de metilación aberrante en la región del promotor de los genes p16 (25%), MGMT (15%), E-Cadherina (25%),HIN-1 (25%) y RASAL-1 (35%). Se analizó la asociación de los grupos clínico-radiológicos con los estados de metilación/no metilación de cada gen. CONCLUSIONES: Se demuestra que la metilación sí tiene un papel en la fibrosis desarrollada en la estenosis pieloureteral destacando dos patrones clínicos de mal pronóstico asociados a dos clusters epigenéticos de metilación. RASAL-1, E-Cadherina, HIN-1 y p16 serían los candidatos para desarrollar estudios futuros sobre sus implicaciones pronósticas en la estenosis pieloureteral.

Perineal groove in female infants: A case series and literature review.

BACKGROUND: Perineal groove is a rare congenital malformation that is unknown to many clinicians and is often misdiagnosed. Although it may be self-resolving during childhood, this nonepithelized mucous membrane can pose the risk of local irritation and infection, particularly urinary tract infection. METHODS: A retrospective study of female infants diagnosed with a perineal groove was performed, demographic characteristics and clinical features were analyzed, and a photographic review was conducted. RESULTS: Five patients with perineal groove were observed in our clinic in 2015-16. The mean age was 14 months. None had symptoms, and no treatment was required. During follow-up of 1 year, all remained asymptomatic. CONCLUSIONS: Our retrospective review suggests that perineal groove may be an underdiagnosed condition. Most cases resolve spontaneously, but confusion in diagnosis may lead to misdiagnosis or misinterpretation of sexual abuse and unnecessary treatments.

A simple dressing for hypospadias surgery in children.

One of the most controversial aspects of hypospadias surgery is the election of an appropriate wound dressing. In fact, there may be as many different types of dressing as there are types of surgical repair. Here, we describe a new, simple method for hypospadias dressing in children that minimizes painful removal.

Umbilical cord and visceral hemangiomas diagnosed in the neonatal period: A case report and a review of the literature.

BACKGROUND: Umbilical cord hemangioma is very rare and may not be detected prenatally. However, it should be considered in differential diagnosis with other umbilical masses because it can cause significant morbidity. METHODS: We report the case of a newborn referred with suspected omphalitis and umbilical hernia. RESULTS: Physical examination showed an irreducible umbilical tumor, the size of olive, with dubious secretion. The initial suspected diagnosis was urachal or omphalomesenteric duct remnants. Abdominal ultrasound and magnetic resonance imaging showed an umbilical and a mesenteric mass. Tumor markers were negative. A definitive diagnosis of umbilical cord and intestinal hemangioma was established after surgical excision and histologic examination of the umbilical mass. Propranolol was prescribed due to the extent of the intestinal lesion. CONCLUSION: This report highlights the diagnostic challenges of hemangiomas in unusual locations. Apart from the rarity of these tumors, few tests are available to guide diagnosis, and surgery and histologic examination are generally required for a definitive diagnosis. Finally, it is essential to rule out associated malformations and hemangiomas in other locations.

Periungual and subungual congenital melanocytic nevus on the foot: A rare case report.

A 5-year-old caucasian child is described with a congenital melanocytic periungual and subungual nevus on the right second toe. Histopathologic examination revealed a junctional nevus. Congenital subungual nevi in this location are rare. To the best of our knowledge, this represents the first case of lesion on the right second toe to be reported in the literature.

Congenital absence of preputial foreskin: an extremely uncommon anomaly.

The part of the penile skin that covers the glans penis is named prepuce or foreskin. The embryologic development of the prepuce and urethra is related. Several congenital anomalies of the prepuce have been previously reported, but the absence of the prepuce with a normal development of the urethra is a very rare association. We report a sporadic case with absence of the prepuce and normal urethral development.

Midline congenital cervical cleft mimicking linear scleroderma.

Midline congenital cervical cleft is an extremely uncommon anomaly of the neck. Fewer than 100 cases have been reported. It is usually described as a cervical scar-like skin defect. We present a case of midline cervical cleft mimicking linear morphea and treated with topical steroids for 2 years. This is an unusual presentation of this entity that must be treated with surgical excision to confirm the diagnosis histopathologically.

Giant presacral tailgut cyst mimicking rectal duplication in a girl: report of a pediatric case.

Tailgut cyst, or retrorectal cystic hamartoma, is a rare congenital lesion found in the presacral space. The lession has been infrequently reported in medical literature. It is most common in middle-aged women and is rare in children. We report a case of a tailgut cyst mimicking a rectal duplication in an 8-year-old child. Excision and histological examination of the mass confirmed the tailgut cyst.

Incomplete unilateral polyorchidism (bilobed testicle) mimicking testicular tumour.

Incomplete polyorchidism (also called bilobed testicle) is an extremely uncommon congenital anomaly. Only 3 cases of bilobed testicle were previously reported in the available literature. We describe a case of a 4-year-old boy who presented with a 6-month history of an asymptomatic scrotal mass located in the upper pole of the left testicle mimicking testicular tumour. After partial orchiectomy, macroscopic and pathological examination of the lesion confirmed the diagnosis of normal testicular tissue.

Bipolar electrothermal vessel sealing system and 5-mm 2 expandable trocar approach in pediatric laparoscopic varicocelectomy: a successful time-effective technical refinement.

BACKGROUND: To present our initial experience with electrothermal bipolar vessel device and only 2 expandable ports for laparoscopic Palomo varicocele procedure in pediatric and adolescent population. METHODS: In a 3-year period between 2006 and 2009, sixty-three boys and adolescents diagnosed in our institution as having varicocele underwent Palomo laparoscopic surgery with a two 5-mm expandable-trocar laparoscopic approach using the LigaSure technology (Valleylab Inc., Covidien, Boulder, CO) for spermatic vessels sealing. The outcome variables recorded for analysis were age at presentation, symptoms, varicocele grade based on Dubin and Amelar Classification, testicular atrophy, operative time, complications, recurrence, and reactive hydrocele formation. Mean follow-up was 1.8 years (range, 6 mo to 3 y). RESULTS: Children's age at diagnosis ranged between 9 and 19 years. Mean age at operation was 14.8 ± 1.2 years. All cases were left side varicoceles and 70% had grade III varicocele. Testicular atrophy was noticed in 39.8% of cases. All boys underwent Palomo laparoscopic sealing of the spermatic vessels using bipolar vascular electrothermal device Ligasure with a 2-trocar approach. Mean operative surgery time was 21 minutes. Median hospital stay was 21 ± 8 hours. No conversion cases were registered. Nine patients developed hydrocele after laparoscopic procedure (14.2%). Three patients of these cases underwent Winkelman-Lord's hydrocelectomy (4.7% of total). CONCLUSIONS: Laparoscopic Palomo varicocele surgery for pediatric patients using Ligasure as spermatic vessels sealant and only 2 radially expandable 5-mm trocars is a safe, feasible, and time-effective technical improvement.

Reactive hydrocele after laparoscopic Palomo varicocele ligation in pediatrics.

OBJECTIVES: To evaluate the incidence of hydrocele following laparoscopic Palomo varicocele ligation in pediatric and adolescents in our institution. METHODS: Between 1997 and 2009, 180 boys diagnosed as having varicocele who underwent Palomo laparoscopic ligation were evaluated retrospectively. Outcome variables recorded for analysis were age at presentation, symptoms, varicocele grade (Dubin-Amelar classification), testicular atrophy, length of hospital stay, perioperative complications, recurrence and hydrocele formation after surgery. Mean follow up was 5.8 years (6 months- 9 years). RESULTS: Age at diagnosis ranged between 9 and 19 years. Mean age at operation was 14.1 ± 1.8 years. There were 177 left-side varicoceles (98%) and four cases were bilateral. Testicular atrophy was noted in 45. Mean operative time was 38 min. The last 63 surgeries were performed on a two-trocar basis with Ligasure vascular sealing device and operative time decreased significantly to 22 min. Median hospital stay was 31h. Twenty-three patients developed hydrocele (13%); 11 of these underwent Winkelman-Lord's hydrocelectomy at least 1 year after Palomo (9% of total). Of the remaining 12, two resolved spontaneously and 10 were stable at mean 4-year follow up. CONCLUSIONS: Laparoscopic Palomo procedure is a safe successful method to correct varicoceles in pediatric and adolescent males. Reactive hydrocele formation following laparoscopic varicocelectomy is a worrisome problem particularly in cases with longer follow-up.

Laparoscopic Palomo varicocele surgery: lessons learned after 10 years' follow up of 156 consecutive pediatric patients.

OBJECTIVES: To evaluate our experience using laparoscopic Palomo varicocele ligation in a population under 18years, and confirm the factors involved in postoperative hydrocele formation. PATIENTS AND METHODS: Between 1997 and 2007, 156 boys diagnosed as having varicocele were evaluated retrospectively. Outcome variables recorded for analysis were age at presentation, symptoms, varicocele grade (Dubin-Amelar classification), testicular atrophy, length of hospital stay, perioperative complications, recurrence and hydrocele formation after surgery. Mean follow up was 5.6 years (6 months- 9 years). RESULTS: Age at diagnosis ranged between 9 and 18 years. Mean age at operation was 14.1+/-1.8 years. There were 153 left-side varicoceles (98%) and three cases were bilateral. All patients had grade II or III varicocele (38%/62%) and testicular atrophy was noted in 43.8%; 8.1% mentioned testicular pain at diagnosis. All boys underwent Palomo laparoscopic ligation of the spermatic vessels. Mean operative time was 38 min (25-82 min). The last 51 surgeries were performed on a two-trocar basis with Ligasure vascular sealing device and operative time decreased significantly to 22 min (16-32 min) (P<0.05). Median hospital stay was 31+/-8 h. Conversion rate was 1.28%. Twenty-one patients developed hydrocele (13.5%); 11 of these underwent Winkelman-Lord's hydrocelectomy at least 1 year after Palomo (9% of total). Of the remaining 10, two resolved spontaneously and eight were stable at mean 4-year follow up. CONCLUSIONS: Laparoscopic Palomo varicocele surgery for pediatric patients is a safe and effective procedure. Recurrence and complication rates are similar to those reported with open surgery.

Giant omental lipoblastoma in a 10-month-old infant composing 20% of total body weight.

Lipoblastoma of the greater omentum is an extremely rare benign tumor with less than 10 cases reported in the available literature. We report an unusual case of a 10-month-old girl with a giant omental lipoblastoma that composed 20% of her total body weight (1480 g, 17 x 12 x 16 cm). Complete excision of the tumor was performed. Histology confirmed the diagnosis of omental lipoblastoma.