The pelagic food web of the Western Adriatic Sea: a focus on the role of small pelagics.

The Adriatic Sea is one of the largest areas of occurrence of shared small pelagic stocks and the most fished area of the Mediterranean Sea, which is in turn one of the most exploited basins of the world. The variations in the stable isotope contents (δ15N and δ13C) were determined for three small pelagic fishes (i.e., Engraulis encrasicolus, Sardina pilchardus, and Sprattus sprattus, respectively known as anchovies, sardines and sprats) collected across the western side of the basin. Our data allowed to determine the width and features of their trophic niches, to assess potential overlap or resource partitioning among them, and likely anticipate species adaptation to future climate change scenarios. Moreover, variations in stable isotope contents were correlated to both resource availability (i.e., mesozooplankton) and environmental variables. The high productivity and in turn the high resource availability of the basin, especially in the northern part, resulted in favor of the resource partitioning that occurs in each sub-area of the Adriatic Sea among the three species. Medium-sized specimens of the three species mostly fed on small zooplankton, while adult sprats relied on large copepods and those of sardines and anchovies also consumed large portion of phytoplankton, confirming the high trophic plasticity of these two dominants small pelagic species. However, considering that anchovies have the greatest degree of trophic diversity compared with the other two species, they could be the most adapted to changing feeding conditions. The increase in sea temperatures that are reducing primary production and in turn zooplankton abundances, coupled with even more frequent extreme meteorologic events could exacerbate the competition for trophic resources among pelagic mesopredators, and could lead to more notable stocks' fluctuations and unpredictable wasp-waist effects.

Crowding in the middle of marine food webs: A focus on Raja asterias and other mediterranean batoids.

The Mediterranean Sea is among the three biodiversity hotspots of the world where elasmobranchs are severely threatened. Elasmobranchs act as apex or meso-predators within marine food webs and the loss/decline of apex predators determines the mesopredator release, leading in turn to increased predation on smaller prey. However, also several mesopredators (including rays, skates and small sharks) are intensively fished, being of commercial interest, or by-caught, and thus mesopredators increase could not be so evident. We analysed the trophic ecology of an endemic Mediterranean ray, the starry ray Raja asterias, at a seasonal scale from the Adriatic basin, one of the most intensively exploited area of the Mediterranean, by means of stomach contents and stable isotopes analyses. Our results evidenced that starry rays rely on benthic sources including species of local commercial values, such as swimming crabs, small cephalopods, and stomatopods and share the same trophic position with other elasmobranchs (rays, skates, and small sharks) and other mesopredators (e.g., common soles, Norway lobsters and mullets). As all mesopredators are overexploited, as well as their benthic prey are affected by intense trawl-fishing, the whole food webs are disrupted and neither the classical trophic cascade nor the mesopredator release hypothesis could be verified. Conservation measures for these species, such as the release after capture or the application of exclusion grids to the net, should be applied in areas where populations are strongly impacted by trawling.

Seasonal trophic ecology of the invasive crab Percnon gibbesi (Brachyura, Plagusiidae) in the southwestern mediterranean: Insights from stomach contents and stable isotope analyses.

The invasive sally lightfoot crab Percnon gibbesi (H. Milne Edwards, 1853) has spread among the coasts of the Mediterranean Sea, including the coasts of Annaba Gulf (Algeria). Investigating the trophic position of the species and looking for the seasonal variations in the diet of this alien decapod was the aim of this study. To do this, samples of P. gibbesi were collected along Annaba coasts within a year. The traditional stomach content analysis (SCA) was integrated to the stable isotope analysis (SIA) of nitrogen and carbon to provide a comprehensive evaluation of the feeding ecology of P. gibbesi, and its within-year variability. Results highlighted, for the first time, significant seasonal variation in P. gibbesi diet, improving our understanding of its trophic plasticity and potential dietary overlaps with other herbivore species. Its feeding plasticity is an asset in the successful expansion of its distribution. Relating its diet composition through the different seasons with the interactions with native herbivores will be essential to fully appreciate the impact of the spread of P. gibbesi in the Mediterranean Sea.

Impact of historical contamination on meiofaunal assemblages: The case study of the Bagnoli-Coroglio Bay (southern Tyrrhenian Sea).

The effects of contaminants on marine organisms have been documented since decades, but the long-term responses and recovery rates of benthic communities to mixtures of contaminants, several years after the cessation of industrial activities, need to be further investigated. Bagnoli-Coroglio Bay (Gulf of Naples, Tyrrhenian Sea) is a typical example of historically contaminated coastal area due to industrial activities stopped at the beginning of nineties. In the present study we carried out a fine spatial scale analysis of the distribution of meiofaunal (and nematodes) assemblages along five bathymetric transects located at increasing distance from the historical source of contamination in relation with the polycyclic aromatic hydrocarbon and heavy metal concentrations present in the sediment. Meiofaunal abundance and biomass changed widely along transects but independent from the distance from the source of contamination. Even when the contamination levels were expected to induce significant detrimental biological consequences, meiofaunal abundance and biomass were similar to those reported in unpolluted benthic coastal areas worldwide. Conversely, biodiversity in terms of meiofaunal taxa richness was generally low (range: 5-8 taxa in 12 of the overall 15 stations investigated). This was explained by the lack of sensitive groups such as ostracods, gastrotrichs and tardigrades commonly encountered in benthic coastal ecosystems, thus reflecting an overall poor/moderate environmental quality of the investigated area. Nematode (structural and functional) diversity was also low, particularly at stations characterized by higher contamination levels. At the same time, nematode species composition did not change significantly among stations suggesting a widespread effect of contaminants able to reduce the variability (i.e., turnover diversity) within the assemblages of the whole study area. Overall, our results indicate that even decades after the cessation of contaminant emissions, benthic biodiversity was affected in terms of both meiofaunal taxa and nematode species. These findings strongly reinforce the call for reducing sources of chronic pollution in marine ecosystems and provide new insights for a better understanding of the ecological recovery of historically contaminated marine environments.

Ecotoxicological potential of non-steroidal anti-inflammatory drugs (NSAIDs) in marine organisms: Bioavailability, biomarkers and natural occurrence in Mytilus galloprovincialis.

Pharmaceuticals represent a major environmental concern since the knowledge on their occurrence, distribution and ecotoxicological potential is still limited particularly in coastal areas. In this study, bioaccumulation and cellular effects of various non steroidal anti-inflammatory drugs (NSAIDs) were investigated in mussels Mytilus galloprovincialis to reveal whether common molecules belonging to the same therapeutic class might cause different effects on non target organisms. Organisms exposed to environmental concentrations of acetaminophen (AMP), diclofenac (DIC), ibuprofen (IBU), ketoprofen (KET) and nimesulide (NIM) revealed a significant accumulation of DIC, IBU and NIM, while AMP and KET were always below detection limit. Nonetheless, for all tested NSAIDs, measurement of a large panel of ecotoxicological biomarkers highlighted impairment of immunological parameters, onset of genotoxicity and modulation of lipid metabolism, oxidative and neurotoxic effects. Laboratory results were integrated with a field study which provided the first evidence on the occurrence of DIC, IBU and NIM in tissues of wild mussels sampled during summer months from an unpolluted, touristic area of Central Adriatic Sea. Overall results demonstrated M. galloprovincialis as a good sentinel species for monitoring presence and ecotoxicological hazard of pharmaceuticals in the Mediterranean.

Síndrome de Bean o Blue rubber bleb nevus syndrome. Presentación de 6 pacientes / Bean or blue rubber bleb nevus syndrome. Presentation of 6 patients

Introducción/objetivos. El síndrome de Bean o Blue Rubber BlebNevus Syndrome es una enfermedad poco frecuente caracterizada por malformaciones venosas multifocales que afectan predominantemente a la piel, tejidos blandos y tracto gastroinestinal. Nuestro objetivo es familiarizar al cirujano pediátrico con esta entidad, fomentar el diagnóstico precoz y el tratamiento quirúrgico seriado como opción terapeútica. Revisamos el diagnóstico y tratamiento de seis pacientes tratados desde 1993 a 2009.Material y mótodos. Todos los pacientes presentaron una malformación venosa (> 10 cm) que actuó como marcador. Posteriormente, aparecieron lesiones cutáneas características, así como anemia crónica por sangrado digestivo asociado a hipofibrinogenemia. La edad de aparición del cuadro anémico varía entre los 6 meses y los 7 años siendo más agresivo cuanto más temprano es. El diagnóstico de la enfermedad se realizó por medios endoscópicos. Todos precisaron trasfusión de hemoderivados entre 5 y 25 ocasiones al año. El tratamiento fue multidisciplinar; tasfusiones periódicas en todos los casos, esclerosis endoscópica en lesiones que afectan a colon o a estómago y tratamiento quirúrgico de las del yeyuno e ileon, en dos de estos pacientes extirpando (..) (AU)

Introduction/Aims. Bean or Blue Rubber Bleb Nevus Syndrome(BRBNS) is an uncommon disease characterized by multifocal venous malformations that predominantly affect the skin, soft tissues and gastrointestinal tract. Our objective is to familiarize the pediatric surgeon with this condition, promoting early diagnosis and seriated surgical treatment as therapeutic options. We have reviewed the diagnosis and treatment of 6 patients treated from 1993 to 2009. Material and methods. All the patients had a venous malformation (> 10 cm) that acted as a marker. After, characteristic skin lesions appeared as well as chronic anemia due to digestive bleeding associated to hypofibrinogenemia. Age of appearance of the anemic picturevaried from 6 months to 7 years, it being more aggressive the earlier its appearance. The diagnosis of the disease was made by endoscopically. All the patients required transfusion with blood derivatives 5 to 25 times (..) (AU)

Indicación para la extirpación precoz de los nevus congénitos gigantes / Indication for early excision of the giant congenital nevus

Introducción y objetivos. El conocimiento progresivo de la epidemiología del nevus congénito gigante (NCG) nos permite acceder a información que confirma una menor tasa de malignización respecto a datos precedentes. Esto ha hecho que la indicación quirúrgica de estas lesiones se cuestione o al menos se difiera. A pesar de ello, hemos encontrado en la práctica clínica durante los últimos 15 años pacientes con NCG que, por distintos motivos, se han beneficiado de su extirpación precoz. Material y métodos. Los diferentes grupos estudiados en el beneficio de la extirpación precoz son: lactantes con cambios preocupantes en el análisis dermatoscópico de la lesión pigmentaria, pacientes connevus gigantes nodulares, ulcerados, niños que presentando un nevusson intervenidos por otro motivo, niños con nevus que provoca distrés (..) (AU)

Introduction. Most of specialists involved in giant congenital nevi(GCN) care, agree that the risk of melanoma is probably not significant enough to warrant the prophylactic removal of all those lesions. However, many patients benefit from very early surgical procedures. Material and methods. In the last 15 years 41 children under went surgical removal of their large or giant melanocityc lesions in the first year of life at our institution. Indications for such an early excision were as follows: painful ulceration or bleeding, alarming dermatoscopic changes, location in the scalp being removable with primary closure, concomitant surgical procedures, multiple expected interventions and severe parental psychosocial distress. Results. All patients had a good cosmetic result. All nevus in the scalp between 3 and 5 cms were removed with a primary closure in order (..) (AU)

[Bean or blue rubber blue nevus syndrome. Presentation of 6 patients]. / Síndrome de bean o Blue rubber bleb nevus syndrome. presentación de 6 pacientes.

INTRODUCTION/AIMS: Bean or Blue Rubber Bleb Nevus Syndrome (BRBNS) is an uncommon disease characterized by multifocal venous malformations that predominantly affect the skin, soft tissues and gastrointestinal tract. Our objective is to familiarize the pediatric surgeon with this condition, promoting early diagnosis and seriated surgical treatment as therapeutic options. We have reviewed the diagnosis and treatment of 6 patients treated from 1993 to 2009. MATERIAL AND METHODS: All the patients had a venous malformation (> 10 cm) that acted as a marker. After, characteristic skin lesions appeared as well as chronic anemia due to digestive bleeding associated to hypofibrinogenemia. Age of appearance of the anemic picture varied from 6 months to 7 years, it being more aggressive the earlier its appearance. The diagnosis of the disease was made by endoscopically. All the patients required transfusion with blood derivatives 5 to 25 times a year. Treatment was multidisciplinary: periodic transfusions in every case, endoscopic sclerosis in lesions that affect the colon or stomach and surgical treatment for lesions of the jejunum and ileum. In two of these patients, 23 and 46 lesions were excised, respectively. In the last year, the tendency has been to use enterotomy and fulguration with Argon. RESULTS: The patients who have undergone excision of the digestive malformations have experienced a significant decrease in their transfusion needs. One female patient with miliary dissemination died. CONCLUSIONS: BRBNS is a difficult to diagnose due to its low frequency. Treatment of gastrointestinal tract lesions is multidisciplinary, combining endoscopic and surgical techniques that decrease transfusions and improve the quality of life of the patients.

[Indication for early excision of the giant congenital nevus]. / Indicación para la extirpación precoz de los nevus congénitos gigantes.

INTRODUCTION: Most of specialists involved in giant congenital nevi (GCN) care, agree that the risk of melanoma is probably not significant enough to warrant the prophylactic removal of all those lesions. However, many patients benefit from very early surgical procedures. MATERIAL AND METHODS: In the last 15 years 41 children undergwent surgical removal of their large or giant melanocityc lesions in the first year of life at our institution. Indications for such an early excision were as follows: painful ulceration or bleeding, alarming dermatoscopic changes, location in the scalp being removable with primary closure, concomitant surgical procedures, multiple expected interventions and severe parental psychosocial distress. RESULTS: All patients had a good cosmetic result. All nevus in the scalp between 3 and 5 cms were removed with a primary closure in order to avoid future need of scalp expansion. Familial psychological distress improved significantly and disturbances related to scholar activities were not noticed. CONCLUSIONS: Despite the lack of international consensus regarding the appropriate timing for large or giant congenital nevi excision, a selection of patients and their families can benefit from early surgical removal.

Anomalías vasculares congénitas óseas / Congenital vascular anomalies of the bone

El hueso no es un tejido habitualmente afectado por las anomalías vasculares; sin embargo, han sido descritas malformaciones venosas, arteriovenosas y linfáticas intraóseas aunque frecuentemente bajo denominaciones erróneas. El objetivo de la presente revisión es profundizar en las claves para la caracterización, diagnóstico precoz y tratamiento de las anomalía vasculares óseas en la infancia. Entre el año 2000 y 2008, han sido evaluados en el Departamento de Cirugía Pediátrica del Hospital Infantil La Paz 37 pacientes con anomalías vasculares óseas. El grupo más frecuente (24 pacientes) es el de las malformaciones linfáticas. Diez presentaban malformaciones venosas (incluyendo uno con simus pericráneo); 1) malformación linfático-venosa; y 2) malformaciones arteriovenosas. Las localizaciones y formas de presentación han sido heterogéneas ya fueran como trastornos de sobrecrecimiento o hipoplasia ósea. El diagnostico fue tardía en la mayoría de los casos. El tratamiento ha sido individualizado según el tipo de malformación y la localización. En los niños con infiltración linfática polióstica (síndrome de Gorham) el protocolo farmacológico establecido ha sido interferón y bifosfonatos. En resumen, el niño con anomalías vasculares óseas presenta unas peculiaridades clínicas que hacen que el diagnóstico sea difícil y el tratamiento complejo, por lo que debe ser evaluado por un equipo multidisciplinar pediátrico, quirúrgico, ortopédico, radiológico e histopatológico. El objetivo final debe ser evitar tratamientos innecesarios o erróneos y minimizar secuelas (AU)

Vascular anomalies involving the bone are uncommon. In addition, intraoseus venous, arteriovenous and lymphatic malformations have been widely described under confusing and wrong terminology. Our purpose is to review the epidemiology and management of vascular abnormalities in skeletal tissue. Between 2000 and 2008, 37 patients with vascular bone anomalies were seen in the Vascular Anomalies Center at La Paz Children Hospital. 24 lymphatic malformations, 10 venous malformations, (including one patient with sinus pericranii), one linfovenous and tow arteriovenous malformations have been reviewed. The location and presentation were heterogeneous including both, bone overgrowth or hypotrophy. Treatment has been individualized for each patient by a multidisciplinary team. Only patients with poliostotic lymphatic infiltration in Gorham syndrome received pharmacological therapy with Interferon and bisphosphonates. In our experience, diagnosis has been significantly delayed and treatment difficulted. As in the more common skin vascular anomalies the distinction between tumor and vascular malformation is essential for a proper therapeutical approach. Early diagnosis, appropriate therapy and close follow up are necessary in order to improve their management, avoid mistreatment and minimize secuelae (AU)

[A satisfaction survey of the evolution of 238 otoplasty]. / Estudio de la evolución de 238 otoplastias mediante encuesta de satisfacción.

PURPOSE: The prominent ear is a frequent problem that can be a source of important psychosocial troubles. The aim of this paper has been to evaluate the psychosocial repercussion, cosmetic result and degree of satisfaction in patients treated at our Institution for prominent ears in the last 4 years. MATERIAL AND METHODS: A questionnaire has been sent to 127 patients. All of them underwent Mustarde's otoplasty. RESULTS: 238 otoplasty were performed in 127 patients (87% bilateral procedures). The mean age was 8.15 +/- 3.25 years (range: 1-15 years), with a follow-up of 27.15 +/- 13.62 months (range: 4-52 months). Sixty-two % of the patients underwent otoplasty without having self-image affected while 38% of the group were patients older enough to see surgery to correct a long-standing defect. Patients did not require a period of postoperative psychosocial adjustment to their new appearance. Two patients described their results of surgery as little improvement. Three undergo partial recurrence and 5 suffered from total recurrence. All of the patients resume their lives with an improved level of self confidence. Conclusions. Otoplasty is a safe procedure for prominent ears to be considered in children with psycosocial, emotional or behavioural effects.

Estudio de la evolución de 238 otoplastias mediante encuesta de satisfacción / A satisfaction survey of the evolution of 238 otoplasty

Objetivo. La procidencia auricular es un problema frecuente que puede ser fuente de importantes alteraciones psicosociales. El objetivo de este trabajo ha sido valorar la repercusión psicosocial, resultado y grado de satisfacción de los pacientes tratados en nuestro Servicio por orejas procedentes en los últimos 4 años. Material y Método. Se han evaluado mediante encuesta telefónica 127 pacientes intervenidos en nuestro Servicio siguiendo el protocolo para otoplastia definido en nuestro centro mediante técnica de Mustardé. Resultados. Se realizaron un total de 238 otoplastias en 127 pacientes(87% procedimientos bilaterales). La edad media fue de 8,15±3,25años (rango = 1-15 años). El tiempo medio de seguimiento fue de27,15±13,62 meses, (rango = 4-52 meses).En 78 pacientes (62%), la objetivación del problema provenía del entorno (padres o pediatra), y en los 49 restantes (38%), del propio paciente. Cuando el niño demandó la consulta existía un trastorno social previo, y en 16 afectaba a su rendimiento escolar. Todos los pacientes con alteraciones sociales y/o escolares recuperaron su autoestima tras el procedimiento. La complicación local más frecuente fue el rechazo de algún punto (9,4% de los casos). Dos de los pacientes se encuentran “algo mejor”, el resto están “mejor” o “mucho mejor”. Se ha producido recidiva en 8pacientes (6,29%), 3 recidivas parciales y 5 recidivas completas. Conclusiones. La otoplastia de Mustardé es una técnica simple indicada en casos evidentes de procidencia auricular, pero también debe considerarse en niños donde la percepción del problema es importante con transcendencia social y escolar (AU)

Purpose. The prominent ear is a frequent problem that can be a source of important psychosocial troubles. The aim of this paper has been to evaluate the psychosocial repercussion, cosmetic result and degree of satisfaction in patients treated at our Institution for prominent ears in the last 4 years. Material and Methods. A questionary has been sent to 127 patients. All of them underwent Mustarde`s otoplasty. Results. 238 otoplasty were performed in 127 patients (87% bilateral procedures). The mean age was 8,15 ± 3,25 years (range:1-15 years),with a follow-up of 27,15 ± 13,62 months (range: 4-52 months). Sixty-two% of the patients underwent otoplasty without having self-image affected while 38% of the group were patients older enough to see surgery to correct a long-standing defect. Patients did not require a period of postoperative psychosocial adjustment to their new appearance. Two patients described their results of surgery as little improvement. Three undergo partial recurrence and 5 suffered from total recurrence. All of the patients resume their lives with an improved level of self-confidence. Conclusions. Otoplasty is a safe procedure for prominent ears to be considered in children with psychosocial, emotional or behavioural effects (AU)

[Surgical treatment in severe dog bites injures in pediatric children]. / Reconstrucción quirúrgica de las lesiones graves por mordedura de perro en niños.

INTRODUCTION: We report a retrospective study of dog-bitten-children who underwent surgical treatment in our department between 1995 and 2005. We checked the different surgical techniques used to cover the defect as well as short and long-term functional and aesthetic results, considering also infection and hospital stay. MATERIALS AND METHODS: During this period of time, 21 patients received surgical treatment because of dogs bites. Surgical approach was decided considering location and severity of lessions. RESULTS: Our serie consists of 12 males and 9 females. Head (71%) and extremities (23%) were the most frequent affected areas. Primary closure was carried out in 18 cases and in the remaining 3 artificial skin or tissue flaps were needed; in these 3 cases the resulting scar was later removed. Surgical infection occurred in less than 10% of the children and in all cases, long-term aesthetic results were considered excellent. CONCLUSIONS: Primary closure of dog-bites-injures improves functional and aesthetic results and reduces the surgical procedures in those patients.

[Aplasia cutis congenita: surgical treatment and results in 36 cases]. / Revisión de 36 casos de aplasia cutis congénita. Protocolo quirúrgico y resultados.

INTRODUCTION: Aplasia cutis congenita is a rare congenital absence of skin most commonly affecting the scalp. Although most defects are small and superficial, approximately 20% of cases involve absence of the skull. Such defects expose the brain and sagital sinus, with concomitant risk of fatal hemorrage, infection, or both. This anomaly most commonly presents as a solitary defect, but sometimes it may occur as multiple lesions. The lesions are noninflammatory and well demarcated, and range is variable from 0.5 cm to 10 cm or more. Although the majority of these scalp defects occurs sporadically, many family cases have been reported. Multiple causes have been suggested for aplasia cutis: genetic causes, syndromes and teratogens, intrauterine infection -varicella zoster virus, herpes simplex virus-, fetal exposure to cocaine, heroin, alcohol or antithyroid drugs. MATERIALS AND METHODS: A retrospective study of children with Aplasia Cutis Congenita who received treatment in Hospital La Paz, in Madrid between 1995 and 2005 was undertaken. We checked location, moment of the surgery, type of surgery and aesthetic results. RESULTS: In the 20 year period between 1985-2005, we treated 36 patients with Aplasia Cutis. 33 of them have the scalp affected and only in 3 cases the trunk was involved. In 4 cases there was an absence of the skull, two slight and two severe. 3 patients had Adams-Oliver and one Cutis Marmorata Telangiectasica syndrome. Fifteen patients were operated in neonatal period with direct closure or advancement or rotational flaps, and in 17 cases the late treatment included use of tissue expanders to cover definitely the defect. One of the patients died for bleeding of the sagital sinus while was waiting for the secondary closure of the wound, and other patient required complex skull reconstruction to achieve a complete coverage. CONCLUSIONS: In view of our experience and results, we believe that early surgery prevents vital risks, reduces local complications and makes easier the final reconstruction.

[Early surgical treatment in nasal tip hemangiomas: 36 cases review]. / Tratamiento quirúrgico precoz de los hemangiomas nasales: revisión de 36 casos.

BACKGROUND: Hemangiomas are the most common benign tumours in infancy. Its trophoblastic origin is now confirmed and it would explain their increasing incidence that runs parallel to the more frequent use of fertilization in vitro techniques and the high rates of prematurity and twinning. It is not a coincidence that they appear more often in the face and its location is related to the merging lines of the facial embryonal buds. Nose tip, upper-eyelid and orbital regions are special areas that need a different approach in each case. Over the last years we changed our therapy protocol for these tumours, in an attempt to decrease the psychological, social and scholar impact in children. PATIENTS AND METHODS: During the last 20 years, 36 patients with nasal tip hemangiomas underwent surgical treatment in our institution. We divided them into two groups, those treated between 1985 and 1992 (n=l1) and those treated between 1992 and 2005 (n=25). Traditionally, conservative management including close observation, corticosteroid therapy and finally surgical treatment of the sequelae, was proposed. Late surgical treatment was reserved for incompletely regressed or unsuccessfully treated facial tumours. Our more recent approach involves early surgical excision, in order to avoid psychological distress and cosmetics defects. Age at diagnosis, appearance at that moment, sort of treatment and time of surgical procedure were recorded to evaluate long-term results. RESULTS: In this paper we report our series of 36 patients, consisting of 26 females and 10 males, with a mean age of 5 +/- 2 months at the first visit. In 11 patients from the first period (1985-1992), corticosteroid therapy was applied waiting the spontaneous regression and they finally underwent delayed surgical treatment at a mean age of 8 years. In the remaining 25 patients treated in the second period, early surgical excision was carried out with a mean age of 3 years and before school age. Indications for corticosteroid therapy include uncontrolled growth and complications (ulceration). CONCLUSIONS: The approach to management of nasal tip hemangiomas should be individualized and must take into account their depth, location, rate of involution and functional disturbance. Children age as well as psychological problems arising from the presence of proliferative hemangioma must be considered when deciding a surgical approach. Upon the high rates of scholar failure in children with deforming hemangiomas, the lack of response to medical therapy and the need of surgical treatment in all cases, our policy now it is to attempt surgical excision and reconstruction before school age. Based on data reported and on our own experience, we consider that very early surgical treatment improves quality of life of our patients and their families.

[Complex lymphatic malformations: diagnostic and therapeutical implications]. / Malformaciones linfáiticas complejas: implicaciones diagnósticas y terapéuticas.

BACKGROUND: Complex lymphatic malformations (CLM) consist of disturbances of lymphatic system development, most often with a genetic origin and with mixed vascular system involvement: lymphatic, venous and capillary. They affect a large corporal area or are associated to other syndromes or systemic diseases. METHODS: We reviewed 21 patients with CLM treated in our hospital during the last 15 years. We used D2-40 monoclonal antibody (by immunohistochemistry) as lymphatic marker to evaluate the level of lymphatic involvement. Furthermore we analysed surgical implications in this group of patients. RESULTS: Twelve children had only lymphatic involvement and nine mixed lymphatic-capillary or lymphatic-venous one. Two died of: respiratory insufficiency (in the neonatal period) and refractory hypoproteinemia (at 8 years of age). The skin was affected between 10 and 35% of total body surface. Three patients suffered from visceral involvement (lungs and mediastinum) and eighteen musculoskeletal. Severe deformity (20), lymphorhagia (15), repeated lymphangitis and chronic pain (5) were the most common symptoms reported. The immunoreaction intensity with monoclonal antibody D2-40 was related to the severity of the local and systemic involvement as well as to the presence of associated malformations. Fifteen cases underwent sequential surgical treatment, seven were treated with sclerotherapy (OK-432) and four with CO2 laser vaporization. Residual lymphorhagia in patients with total extirpation of the lymphatic malformation stopped after repeated evacuator punctures and healing took place. CONCLUSIONS: (1) D2-40 monoclonal antibody is a marker of bad prognosis in CLM. (2) The complete excision of the lymphatic malformation lead to healing and the associated lymphorragia should not be considered as a recurrence, which will stop with evacuator punctures in all cases. (3) A multidisciplinary team approach is essential for the proper care of CLM in order to minimize postoperative sequelae and late complications.

Reconstrucción quirúrgica de las lesiones graves por mordedura de perro en niños / Surgical treatment in severe dog bites injures in pediatric children

Objetivo. En este trabajo presentamos la serie de pacientes intervenidos en nuestro hospital por mordeduras de perro y que precisaron tratamiento quirúrgico inmediato bajo anestesia general. Se presentan las diferentes opciones quirúrgicas que hemos utilizado, cierre primario, injerto de piel, cobertura con dermis artificial y expansores titulares. Los resultados se valoran a corto y largo plazo desde el punto de vista funcional y estético, asimismo se valoran la infección, la estancia hospitalaria y la morbilidad de estos pacientes. Material y métodos. Veintiún pacientes ingresados en nuestro hospital entre 1995-2005 precisaron tratamiento quirúrgico tras una agresión por perro. En función de la localización y la gravedad de las lesiones se decidió la técnica quirúrgica a emplear. Resultados. De los 21 pacientes, 12 eran hombres (57%) y 9 mujeres (43%). Las zonas afectadas fueron la cabeza en el 71% de los casos, las extremidades en el 23% de los casos y el resto en otras localizaciones. En 18 pacientes se consiguió la reconstrucción total mediante cierre primario. En 3 casos se utilizaron substitutos dérmicos biosintéticos o injertos de piel y posteriormente expansores cutáneos para extirpación de la cicatriz. Las complicaciones infecciosas fueron inferiores al 10% y los resultados estéticos a largo plazo han sido muy buenos. Conclusiones. El cierre quirúrgico primario de las lesiones graves producidas por mordeduras de perro disminuye las secuelas funcionales y estéticas y el número de intervenciones quirúrgicas en estos pacientes (AU)

Introduction. We report a retrospective study of dog-bitten- children who underwent surgical treatment in our department between 1995 and 2005. We checked the different surgical techniques used to cover the defect as well as short and long-term functional and aesthetic results, considering also infection and hospital stay. Materials and methods. During this period of time, 21 patients received surgical treatment because of dogs bites. Surgical approach was decided considering location and severity of lessions. Results. Our serie consists of 12 males and 9 females. Head (71%) and extremities (23%) were the most frequent affected areas. Primary closure was carried out in 18 cases and in the remaining 3 artificial skin or tissue flaps were needed; in these 3 cases the resulting scar was later removed. Surgical infection occurred in less than 10% of the children and in all cases, long term aesthetic results were considered excellent. Conclusions. Primary closure of dog-bites-injures improves functional and aesthetic results and reduces the surgical procedures in those patients (AU)

Revisión de 36 casos de aplasia cutis congénita. Protocolo quirúrgico y resultados / Aplasia cutis congenita: surgical treatment and results in 36 cases

Introducción/Objetivo. La aplasia cutis es una rara enfermedad congénita caracterizada por ausencia de una porción de piel, sobre todo a nivel del cuero cabelludo. Aunque la mayoría de los defectos son pequeños y superficiales, aproximadamente un 20% de los casos incluye ausencia de hueso. Este tipo de lesión expone el cerebro y el seno sagital superior con el riesgo consiguiente de infecciones, hemorragias o ambas. Esta anomalía se presenta generalmente de forma aislada, pero en ocasiones pueden aparecer múltiples lesiones. El defecto es una lesión de tipo no inflamatorio y bien delimitada, que tiene una extensión variable desde 0,5 cm a 10 o más centímetros. Aunque la mayoría de las veces, esta lesión aparece de forma aislada, se han descrito muchos casos familiares. Se postulan múltiples etiologías para explicar la aplasia cutis incluyendo factores genéticos, sindrómicos y teratógenos (infecciones intrauterinas como VVZ, VHS; cocaína, heroína, alcohol y fármacos antitiroideos). El objetivo de este trabajo es presentar nuestro protocolo quirúrgico y experiencia en el tratamiento de esta anomalía. Pacientes y métodos. Hemos revisado 36 pacientes con Aplasia Cutis Congénita tratados en nuestro hospital en los últimos 20 años (1985- 2005). Recogimos datos sobre la localización de esta malformación cutánea, momento de intervención, técnica quirúrgica utilizada y resultado estético. Resultados. De todos los pacientes revisados, 33 tenían afectación del cuero cabelludo y 3 presentaban lesión a nivel del tronco. En 4 pacientes, el defecto incluía al diploe craneal y existía un encefalocele asociado, de carácter leve en dos casos y severo en otros dos. Tres pacientes eran portadores del Síndrome de Adams Oliver y uno de Cutis Marmorata Telangiectásica Congénita. En 15, el tratamiento quirúrgico se llevó a cabo en el periodo neonatal (cierre primario del defecto, colgajo de rotación, injerto…) y en 17 el tratamiento diferido incluyó la aplicación de expansores tisulares para la cobertura definitiva de la zona alopécica. Uno de los pacientes falleció por rotura del seno longitudinal a la espera de epitelización espontánea y otro precisó derivación ventrículo- peritoneal, extirpación del encefalocele y cobertura con colgajo libre para cierre del defecto. Conclusiones. A la vista de nuestra experiencia parece evidente que la cobertura precoz del defecto no solo disminuye el riesgo vital, sino que además reduce significativamente las complicaciones locales y facilita la reconstrucción definitiva (AU)

Introduction. Aplasia cutis congenita is a rare congenital absence of skin most commonly affecting the scalp. Although most defects are small and superficial, approximately 20% of cases involve absence of the skull. Such defects expose the brain and sagital sinus, with concomitant risk of fatal hemorrage, infection, or both. This anomaly most commonly presents as a solitary defect, but sometimes it may occur as multiple lesions. The lesions are noninflamatory and well demarcated, and range is variable from 0,5 cm to 10 cm or more. Although the majority of these scalp defects occurs sporadically, many family cases have been reported. Multiple causes have been suggested for aplasia cutis: genetic causes, syndromes and teratogens, intrauterine infection -varicella zoster virus, herpes simplex virus-, fetal exposure to cocaine, heroin, alcohol or antithyroid drugs. Materials and methods. A retrospective study of children with Aplasia Cutis Congenita who received treatment in Hospital La Paz, in Madrid between 1995 and 2005 was undertaken. We checked location, moment of the surgery, type of surgery and aesthetic results. Results. In the 20 year period between 1985-2005, we treated 36 patients with Aplasia Cutis. 33 of them have the scalp affected and only in 3 cases the trunk was involved. In 4 cases there was an absence of the skull, two slight and two severe. 3 patients had Adams-Oliver and one Cutis Marmorata Telangiectasica syndrome. Fifteen patients were operated in neonatal period with direct closure or advancement or rotational flaps, and in 17 cases the late treatment included use of tissue expanders to cover definitely the defect. One of the patients died for bleeding of the sagital sinus while was waiting for the secondary closure of the wound, and other patient required complex skull reconstruction to achieve a complete coverage. Conclusions. In view of our experience and results, we believe that early surgery prevents vital risks, reduces local complications and makes easier the final reconstruction (AU)

Tratamiento quirúrgico precoz de los hemangiomas nasales: revisión de 36 casos / Early surgical treatment in nasal tip hemangiomas: 36 cases review

Introducción. Los hemangiomas son los tumores benignos más frecuentes en la infancia. Recientemente se ha confirmado su origen placentario, que podría explicar el incremento en su incidencia paralelo al mayor uso de las técnicas de fecundación in vitro y las elevadas tasas de prematuridad y gemelaridad. Su aparición en la cara no es casual y sigue un patrón que se corresponde con las líneas de fusión de los mamelones faciales embrionarios. Nariz, órbita y párpado son áreas que requieren en cada caso una estrategia diferente. Durante los últimos años, nuestro protocolo de actuación frente a estos tumores se ha ido modificando y redefiniendo para minimizar la repercusión social, psicológica y escolar del niño. Pacientes y métodos. Revisamos retrospectivamente los 36 pacientes con hemangiomas de la pirámide nasal tratados en nuestro servicio durante los últimos 20 años. Dichos pacientes fueron divididos en 2 grupos según fueran intervenidos entre 1985-1992 (n=11) o entre 1992- 2005 (n=25). El protocolo tradicional, seguido hasta 1992, incluía observación y tratamiento esteroideo para corrección posterior de las secuelas. Con las nuevas actitudes terapéuticas, la edad de corrección quirúrgica se ha reducido notablemente y con ello las secuelas psicológicas. Analizamos edad de aparición, hallazgos al diagnóstico, modalidad y edad de tratamiento, para evaluar los resultados estéticos a largo plazo. Resultados. De los 36 pacientes de nuestra serie, 26 eran mujeres y 10 hombres, con edad media en la primera consulta de 5±2 meses. Los 11 pacientes diagnosticados en el primer período (hasta 1992) recibieron tratamiento con corticoides durante una media de 3 meses, para luego esperar la involución de la lesión y ser intervenidos con una edad media de 8 años. Por el contrario, los 25 pacientes intervenidos en el segundo período, se operaron precozmente, con una media de edad de 3 años y antes de la escolarización. Actualmente las únicas indicaciones del tratamiento con corticoides son el crecimiento desmesurado, la afectación generalizada o las complicaciones (ulceración). Conclusión. El tratamiento de los hemangiomas nasales debe individualizarse, teniendo en cuenta su localización, profundidad, velocidad de involución y repercusión funcional. La edad del niño y la repercusión psicológica sobre el paciente y su familia, influyen en la decisión quirúrgica. La alta tasa de fracaso escolar en niños con hemangiomas desfigurantes, la escasa respuesta al tratamiento farmacológico y la necesidad de tratamiento quirúrgico en todos los casos, han llevado a indicar la extirpación del hemangioma y la rinoplastia asociada antes de la escolarización. En base a los conocimientos actuales y a nuestra propia experiencia, consideramos que el abordaje quirúrgico precoz representa una mejoría significativa en la calidad de vida de los pacientes y sus familias (AU)

BACKGROUND: Hemangiomas are the most common benign tumours in infancy. Its trophoblastic origin is now confirmed and it would explain their increasing incidence that runs parallel to the more frequent use of fertilization in vitro techniques and the high rates of prematurity and twinning. It is not a coincidence that they appear more often in the face and its location is related to the merging lines of the facial embryonal buds. Nose tip, upper-eyelid and orbital regions are special areas that need a different approach in each case. Over the last years we changed our therapy protocol for these tumours, in an attempt to decrease the psychological, social and scholar impact in children. PATIENTS AND METHODS: During the last 20 years, 36 patients with nasal tip hemangiomas underwent surgical treatment in our institution. We divided them into two groups, those treated between 1985 and 1992 (n=l1) and those treated between 1992 and 2005 (n=25). Traditionally, conservative management including close observation, corticosteroid therapy and finally surgical treatment of the sequelae, was proposed. Late surgical treatment was reserved for incompletely regressed or unsuccessfully treated facial tumours. Our more recent approach involves early surgical excision, in order to avoid psychological distress and cosmetics defects. Age at diagnosis, appearance at that moment, sort of treatment and time of surgical procedure were recorded to evaluate long-term results. RESULTS: In this paper we report our series of 36 patients, consisting of 26 females and 10 males, with a mean age of 5 +/- 2 months at the first visit. In 11 patients from the first period (1985-1992), corticosteroid therapy was applied waiting the spontaneous regression and they finally underwent delayed surgical treatment at a mean age of 8 years. In the remaining 25 patients treated in the second period, early surgical excision was carried out with a mean age of 3 years and before school age. Indications for corticosteroid therapy include uncontrolled growth and complications (ulceration). CONCLUSIONS: The approach to management of nasal tip hemangiomas should be individualized and must take into account their depth, location, rate of involution and functional disturbance. Children age as well as psychological problems arising from the presence of proliferative hemangioma must be considered when deciding a surgical approach. Upon the high rates of scholar failure in children with deforming hemangiomas, the lack of response to medical therapy and the need of surgical treatment in all cases, our policy now it is to attempt surgical excision and reconstruction before school age. Based on data reported and on our own experience, we consider that very early surgical treatment improves quality of life of our patients and their families (AU)

Malformaciones linfáticas complejas: implicaciones diagnósticas y terapéuticas / Complex lymphatic malformations: diagnostic and therapeutical implications

Introducción. Las malformaciones linfáticas complejas (MLC) son alteraciones del desarrollo del sistema linfático, en la mayoría de los casos de origen genético, con afectación mixta del sistema vascular: linfático, venoso y capilar. Se caracterizan por afectar de forma extensa la superficie corporal del niño o por asociarse a otros síndromes o enfermedades sistémicas. Material y métodos. Revisamos los 21 pacientes con MLC tratados en nuestro centro en los últimos 15 años. Utilizamos el anticuerpo monoclonal D2-40 (inmunohistoquímica) para evaluar el grado de afectación linfática (como marcador de la afectación linfática). Así mismo analizamos las implicaciones quirúrgicas en este tipo de pacientes. Resultados. Doce niños tenían afectación linfática exclusivamente y nueve linfático-capilar o linfático-venosa. Dos fallecieron por insuficiencia respiratoria (período neonatal) e hipoproteinemia refractaria (a los 8 años de edad). La piel estaba afectada entre el 10 y 35% de la superficie corporal. En tres casos había afectación visceral (pulmonar y mediastínica) y en 18 musculoesquelética. La sintomatología más frecuente fue la deformidad severa (20), seguida de linforragia (15), linfangitis de repetición (7) y dolor crónico (5). La intensidad de la inmunorreacción con el anticuerpo monoclonal D2- 40 se correlacionó con la severidad de la afectación local y sistémica así como con la existencia de malformaciones asociadas. Quince de los pacientes fueron sometidos a tratamiento quirúrgico secuencial, siete recibieron esclerosis seriadas con OK-432 y cuatro vaporización con láser de CO2. La linforragia residual de los pacientes en los que se pudo extirpar completamente la malformación cedió tras punciones evacuadoras repetidas considerándose la curación de la enfermedad. Conclusiones: 1. El anticuerpo monoclonal D2-40 es un marcador de mal pronóstico en las MLC. 2. La extirpación radical de la malformación consigue la curación y la linforragia asociada no debe considerarse una recidiva, cediendo en todos los casos tras punciones evacuadoras. 3. Las MLC requieren protocolos de tratamiento por un equipo multidisciplinario para paliar las secuelas postoperatorias y a largo plazo (AU)

BACKGROUND: Complex lymphatic malformations (CLM) consist of disturbances of lymphatic system development, most often with a genetic origin and with mixed vascular system involvement: lymphatic, venous and capillary. They affect a large corporal area or are associated to other syndromes or systemic diseases. METHODS: We reviewed 21 patients with CLM treated in our hospital during the last 15 years. We used D2-40 monoclonal antibody (by immunohistochemistry) as lymphatic marker to evaluate the level of lymphatic involvement. Furthermore we analysed surgical implications in this group of patients. RESULTS: Twelve children had only lymphatic involvement and nine mixed lymphatic-capillary or lymphatic-venous one. Two died of: respiratory insufficiency (in the neonatal period) and refractory hypoproteinemia (at 8 years of age). The skin was affected between 10 and 35% of total body surface. Three patients suffered from visceral involvement (lungs and mediastinum) and eighteen musculoskeletal. Severe deformity (20), lymphorhagia (15), repeated lymphangitis and chronic pain (5) were the most common symptoms reported. The immunoreaction intensity with monoclonal antibody D2-40 was related to the severity of the local and systemic involvement as well as to the presence of associated malformations. Fifteen cases underwent sequential surgical treatment, seven were treated with sclerotherapy (OK-432) and four with CO2 laser vaporization. Residual lymphorhagia in patients with total extirpation of the lymphatic malformation stopped after repeated evacuator punctures and healing took place. CONCLUSIONS: (1) D2-40 monoclonal antibody is a marker of bad prognosis in CLM. (2) The complete excision of the lymphatic malformation lead to healing and the associated lymphorragia should not be considered as a recurrence, which will stop with evacuator punctures in all cases. (3) A multidisciplinary team approach is essential for the proper care of CLM in order to minimize postoperative sequelae and late complications (AU)