[Vascular anomalies in the neonatal period]. / Anomalías vasculares de presentación neonatal.

OBJECTIVE: Vascular anomalies in the neonatal period are a diagnostic challenge for the lack of evident signs, symptoms and follow-up, and the convenience of restricting aggressive diagnostic tests. The aim of this work is to review the characteristics of neonatal cases presented to our Vascular Anomalies Unit in the last 5 years. MATERIALS AND METHODS: All cases of suspected vascular anomaly presented to our unit before 1 month of age between 2010 and 2015 were reviewed, diagnostic tests and treatments carried out with chronology were analyzed. Presumptive diagnosis and final diagnosis (when available) were compared. RESULTS: Fifteen vascular tumors were found, 2 with visceral involvement: 6 infantile hemangiomas (IH), 3 NICH, 4 RICH, 1 tufted hemangioma, 1 unspecified liver vascular tumor, 3 venous malformations (2 equivocal MRI and a hyperkeratotic venous malformation), 4 lymphatic malformations, 3 of them macrocystic, and 2 vascular lesions that were diagnosed of fibrosarcoma and sclerema neonatorum and they were not vascular anomalies. Only 3 patients with macrocystic lymphatic malformations had prenatal diagnosis. CONCLUSION: Accurate diagnosis of vascular anomalies during the first month of life is difficult, even with MRI. Only in a few cases early treatment is needed, so it is worth taking time to follow-up. Different types of treatment (observation, propranolol, biopsy, laser, embolization, and resection) will depend on the condition to be treated. A continuous observation can avoid unnecessary procedures and risks.

OBJETIVOS: Las anomalías vasculares de presentación neonatal suponen un reto diagnóstico por la ausencia de semiología florida, de historia evolutiva y la conveniencia de restringir pruebas diagnósticas agresivas. El objetivo es revisar las características de los casos neonatales presentados a nuestra Unidad de Anomalías Vasculares en los últimos 5 años. MATERIAL Y METODOS: Se recogen todos los casos de sospecha de anomalía vascular presentados a nuestra Unidad antes de 1 mes de edad entre 2010 y 2015. Se revisa el momento del diagnóstico en relación con la anomalía, las pruebas diagnósticas y los tratamientos efectuados con su cronología. Se comparan el diagnóstico de presunción y el de certeza, cuando lo hay. RESULTADOS: Se incluyen 26 pacientes: 15 tumores vasculares, 2 de ellos con afectación visceral (6 hemangiomas infantiles (HI), 3 NICH, 4 RICH, 1 hemangioma en penacho, 1 tumor vascular hepático no especificado. 3 malformaciones venosas: 2 con RM equívoca y una malformación venosa hiperqueratótica. 4 malformaciones linfáticas: 3 macroquísticas y una microquística. 2 lesiones muy vasculares que se diagnosticaron posteriormente (fibrosarcoma y adiponecrosis) y no eran anomalías vasculares. Solo 3 pacientes tenían diagnóstico prenatal, las malformaciones linfáticas macroquísticas. CONCLUSION: El diagnóstico preciso de las anomalías vasculares durante el primer mes de vida es difícil, incluso con RM. En pocos casos se necesita un tratamiento precoz, por lo que conviene dar tiempo a la evolución, al menos durante unas semanas. Los diferentes tipos de tratamiento (observación, propranolol, biopsia, láser, embolización, exéresis) dependerán de la patología a tratar. Una observación continuada puede evitar procedimientos y riesgos innecesarios.

[Surgical treatment of giant congenital melanocytic nevi: a change of aim]. / Tratamiento quirúrgico del nevus melanocítico gigante: un cambio de objetivo.

OBJECTIVE: To review the surgical experience in Giant Congenital Melanocytic Nevi (GCMN). MATERIAL AND METHODS: Review of GCMN cases consulting at the Department of Pediatric Surgery since 1994. Data registered were: year and age at 1st consultation, type of treatment, number of surgical procedures and complications, histology, central nervous system MRI and follow-up. RESULTS: Eleven patients with GCMN > 10% of body surface consulted at ages ranging from newborn to 8 years. All of them had multiple surgical procedures (2-19), from nevus removal to only biopsies. Eight patients had tissue expansion, completed in 3 of them with skin grafts on dermal substitute. Six patients had complications: 4 expander extrusions, 5 infections, 3 flap necrosis and 1 dehiscence. In 6 children a total or subtotal resection of the nevus was achieved; in 2 the treatment was interrupted, remaining 20% and 50% of the initial nevus; three patients had not had nevus treatment. None of the patients presented cutaneous melanoma; one died from intracranial melanoma; another one has leptomeningeal melanosis. The first 4 patients underwent an average of 16 surgical procedures each, the last 7 patients only 5. CONCLUSIONS: The aim of GCNM management has changed: GCNM treatment is now surgically conservative. Complete excision is now not the aim when technically unfeasible in few procedures; multiple surgical procedures with poor cosmetical results are not acceptable. The gravity is determined by CNS involvement.

[Palatal necrosis in children. Case report]. / Necrosis palatina en el lactante. A propósito de un caso.

Palate necrosis as a consequence of palate infection it's an exceptional condition about there's not too much references at literature. We present a case of a 6 months old child who present a palatal necrosis after a supurative medial otitis that involved hard and soft palate, with positive culture for Pseudomona aeruginosa causing a almost complete absence of the palate that simulate a bilateral palatal cleft.

Gastroschisis in the partially extraamniotic fetus: experimental study.

The short, thick, matted bowel, often found in gastroschisis, has been attributed to a variety of factors. We designed an original animal model in order to isolate the effect of amniotic fluid on the fetal bowel. We created experimental gastroschisis in fetal rabbits. Extruded bowel was kept out of the amniotic sac in half of the operated fetuses. At term, 7 days after surgery, 21 surviving fetuses (12 intraamniotic gastroschisis [IAG] and 9 extraamniotic gastroschisis [EAG]) and 16 control littermates were collected for study. Five parameters were measured in each animal: body weight, intestinal weight, intestinal length, bowel diameter, and bowel wall thickness. Fetal mortality due to the extraamniotic fixation technique was 25%. We found no statistically significant difference in body weight, intestinal weight, or bowel wall thickness. An enormous difference was found in intestinal length (P < .0001 among the three groups). Bowel diameter was significantly different (P = .02) in the extreme groups (IAG and controls), with the intestine being more dilated in IAG than in EAG and controls. Gastroschisis in the absence of amniotic fluid was not as damaging to the fetal intestine as "natural" intraamniotic gastroschisis.

Segmental dilatation of the duodenum.

In this article two cases of idiopathic duodenal dilation are described. These cases meet the present criteria for segmental dilations of the intestine. The onset of the clinical features was acute in one case and chronic in the other. However, the radiological images were similar in both cases and provided the diagnosis. Surgical treatment consisted of duodenal resection and tapering and was curative in one case. The other patient died shortly after surgery. The duodenal specimens showed normal three-layer duodenal architecture in the pathology study. The intramural nervous plexi were unimpaired. Angiodysplasia was found in one case. Vascular abnormalities were also found in 10 other segmentary intestinal dilatations reported in the literature. The surgical implications of this new localization of segmentary intestinal dilatation are analyzed.

Antireflux role of free muscle transplantation. Experimental study in a reflux esophagitis rat model.

PURPOSE: Experimental work on free muscle transplantation has resulted in some successful clinical applications. After a previous experimental study that established the viability of this procedure on the distal esophagus of the rat, we investigated the antireflux efficacy of free muscle transplantation on the distal esophagus in a reflux esophagitis rat model. METHODS: Two groups of Sprague-Dawley rats were submitted to a reflux-inducing operation. Thirteen animals underwent esophagojejunostomy only (EJ) and fourteen were submitted to esophagojejunostomy plus free muscle transplantation of plantaris on the distal esophagus (ETx). Eight weeks later, the esophagi of survivors were removed after sacrifice (EJ, n = 10; ETx, n = 10) and the macroscopic longitudinal extension (mm) and the histological grade (1-4) of esophagitis evaluated. RESULTS: All esophageal specimens of both groups had esophagitis. However, the extent of esophagitis (median: ETx: 23 vs EJ: 32) and the frequency of high grades of esophagitis (ETx: 30 % vs. EJ: 100 %) were significantly lower in the ETx group (p = 0.005 and p = 0.001, respectively). All except one ETx subject presented with an extent and/or a grade of esophagitis which was less than the EJ subjects' lowest values. CONCLUSIONS: Experimental free muscle transplantation on the distal esophagus seems to have antireflux function, thus its therapeutic implications deserve further investigation. The incomplete efficacy of the procedure may suggest that a putative anatomic sphincter per se is not enough to promote an effective antireflux mechanism.

Treatment of an enteric fistula with somatostatin in a premature.

The authors present the case of an extremely premature baby affected by severe Necrotizing Enterocolitis (NEC) needing intestinal resection and ileostomy. An enteric fistula developed 3 days after ostomy closure. The baby was started on Total Parenteral Nutrition (TPN), and 7 days later the fistula output remained constant. Somatostatin (SM) was then given intravenously (3.0 micrograms/Kg/hr) and the fistula closed on the 3rd day of treatment. Since SM was introduced in 1986 as an adjunct treatment to TPN in enteric fistulas, the authors believe that theirs is the first report of a successful SM treatment in a premature.

[Fractures of the orbit floor]. / Fracturas del suelo de la órbita.

Fractures of the floor are not common during childhood, their main cause being trauma. The mechanism is an increased pressure in the orbital cavity, which breaks at is weakest point, the floor, where soft tissue may be trapped- periorbitary fat, inferior rectus muscle, and inferior oblique muscle. Symptoms are diplopia, enoftalmos, eyelid ptosis and soft tissue haematoma. The diagnosis is made on the clinical and imaging findings, CT-Scan being the most reliable technique. Surgical treatment is necessary when symptoms do not subside and when the muscles or the infraorbitary nerve are compromised. We present four cases of orbit floor fracture which were completely resolved with conservative management (2 cases) or with surgical release of the muscular structures and orbit floor reconstruction (2 cases).

[Mammaplasty in adolescent girls]. / Mastoplastia en la adolescente.

The aim of this work is to analyze the experience in our pediatric center on the surgical management of mammary malformation in teenagers. We have reviewed or mammaplasty cases until 1999 (n = 24). We have divided them in two groups: augmentation (A) and reduction (R) mammaplasty. The analyzed parameters were: ethiology, associated pathology, surgical approach, aesthetic results and complications. Group A (n = 14; 17 implants). The surgical indication was moderate-severe mammary hypoplasia with psychological repercussion. Associated pathology: thoracic malformation (n = 13) and psychiatric pathology (n = 2). The surgical approach was submammary in 6 cases, videoassisted transaxillary in 4 and iterative on thoracic scar in 3. All the implants were located at the subglandular space. No major complications were found and the cosmetic results were good, with only one reoperation because of asymmetry. Group R (n = 10). Surgery was indicated because of bilateral puberal mammary hipertrophy in all cases. Associated pathology: obesity (n = 3), psychiatric and behaviour disorders (n = 3), scoliosis (n = 2), and one case of isosexual precocious puberty. In all cases but one the Strömbeck mammaplasty was performed. We used the Lejour technique in this single case. The cosmetic results were good, except for 2 cases of hypertrophic scar. The only complication was a wound infection that healed well.

[Surgical management of congenital tracheal stenosis]. / Manejo quirúrgico de la estenosis traqueal congénita.

A six month old infant with dextrocardia, butterfly vertebrae and oesophageal atresia type I is reported. After surgical repair, she developed respiratory distress, needing ventilatory support. The bronchoscopy and bronchography demonstrated segmental tracheal stenosis. A resection of the stenotic segment was performed, and tracheal reconstruction with terminoterminal anastomosis. After a period of clinical improvement, respiratory distress came back and reestenosis was ascertained locating its level on the anastomosis. Three sessions of balloon dilation were ineffective and because that it's decided the introduction of a metallic self-expandable stent. After a transient relief, the patient had a bilateral pneumonia ending with the dead of the patient. In spite of the treatment used, it was not possible to accomplish solution in a middle or a large place. This constitutes a clear exponent of the complexity in the management of the congenital tracheal stenosis.

[Experimental alkaline reflux esophagitis. Role of the refluxed material]. / Esofagitis por reflujo alcalino experimental. Papel del material refluyente.

Alkaline reflux is new recognised to play an important place in gastroesophageal reflux disease. In order to investigate the role of refluxate in the development of esophagitis we have performed an experimental study in laboratory animals submitted to alkaline reflux inducing operations. We produced esophagitis in two experimental groups of Sprague-Dawley rats: group I, end-to-side esophagojejunostomy (EY) and group II, esophagojejunostomy with partial derivation of afferent loop by an omega-like conduit (EO) designed to attain lesser reflux. Group III consisted of sham laparotomized animals (L). RESULTS. At day 14, all the animals of experimental groups have developed esophagitis, but the macroscopic extent and histological grade in group EO (med: 27 mm; grade 2) were significantly lower than in group EY (med: 35 mm; grade 3). Biochemical measurements (med) in esophageal washout revealed: a) pH were alkaline in all groups with a decrease in experimental groups that was only significant in group EY; b) Amylase, lipase and bile acids in groups EY (8090; 498; 32) and EO (12840; 449; 50) were similar, but significantly more higher than in group L (920; 24; 0). The analysis of correlation between esophagitis parameters and biochemical measurements revealed no significant ones except for pH (r = -.437 and -.417). CONCLUSIONS. 1- The alternative model (EO) presents lesser grade and extent of esophagitis. 2) The refluxate was rich in bile acids and pancreatic enzymes what is proof of the actual occurrence of reflux and supports its determinant contribute to the development of esophagitis. 3) Esophagitis has developed at alkaline pH but with no alkalinity increase in the experimental groups what indicate that "alkaline reflux esophagitis" is not an appropriate denomination. 4) The discrepancy between the biochemical analysis in esophageal washout and the extent and grade of esophagitis suggests that others than the nature and amount of refluxate (e.g. mucosal resistance) could play a role in reflux esophagitis.

[Otological assessment in patients operated on for cleft palate]. / Valoración otológica en pacientes intervenidos de fisura palatina.

Tympanometry and otoscopy has been carried out to determine the presence of middle ear disease on patients with cleft palate and the influence of corrective surgery. This explorations have been evaluated preoperatively and at one and six months postoperative follow-up. There is a high incidence of otitis media with effusion in patients with cleft palate, almost 60 per 100 in our study. The influence of cleft palate surgery repair has not revealed as a mayor factor on its resolution, although a visible benefit has been observed in the long term controls. We recommend a otorhinolaryngological follow-up in these patients.

[Bilateral cleft lip and palate: experience from a center with a multidisciplinary approach]. / Fisura labiopalatina bilateral: experiencia de un centro con abordaje multidisciplinar.

The aim of this work is to analyse the global experience of a center where a pluridisciplinar approach of the child affected with a cleft lip and palate is regularly done. Since january 1980 until january 2000, a total of 36 children (8 F, 28 M) with bilateral cleft lip and palate were treated. Only 5 children were born at this hospital. The others (n = 31) were referred soon after birth (24/31) or later for treat sequels. A multidisciplinary team evaluated every case. The parameters analysed were: surgical protocol, aesthetic and speech outcome, hearing disturbance, complications and the number of secondary lip surgeries. Surgical approach consisted on a soft palate closure before 3 months follow by a bilateral cheiloplasty (6 months) and a hard palate closure before 4 years of age, in the majority of cases (24/36). The esthetical result was evaluated in 25 children and was acceptable in the great majority (22/25). 16 children were submitted to tympanic draining in order to treat their secretory otitis. Speech outcome was analysed in 27 children and was good in 23. With a follow-up of 8.4 years, 15 children (8 treated soon on this center and 7 that came for their sequels) were treated for complications. There was a media of 4.5 surgeries per children.

[Skeletal muscle graft on the terminal ileum as a substitute for ileocecal valve: experimental study]. / Injerto de músculo estriado en íleon terminal como sustituto de la válvula ileocecal: estudio experimental.

Behavior of a free skeletal muscle graft in contact with intestinal wall is analyzed in rats. On first phase, survival of the graft muscle in relation with previous denervation is studied. Nondenervated muscles suffer necrosis and fibrosis, whereas when denervated 4 weeks before grafting, the muscles are viable. On a second phase, the grafted muscle was studied on the long term. From 8th post-transplant week on, the structural and metabolic features of the graft were similar to normal. On the third phase the efficacy of the muscle implant as a substitute of the ileocecal valve is checked. Germs on either side of the valve (ileum and cecum) are counted in normal rats, rats after valve excision and rats with substitution of the valve by a free skeletal muscle implant. There haven been significant differences between normal and no-valve rats (P < 0.001) and between no-valve and artificial valve rats (P < 0.005). There has not been significant difference between normal rats and artificial valve rats. We conclude that free skeletal muscle survives transplant to intestine, and it prevents bacterial reflux from colon to ileum.

[The profile surgery. Mandibular osteotomies]. / Cirugía del perfil. Osteotomía mandibular.

During the years 1987-1994, 31 mandibular osteotomies have been performed in 25 patients, 15 had mandibular alteration alone, 10 of them with prognatism, 2 with microretrognatia and 3 with chin hipoplasia. The other 10 had a combined maxillary-mandibular alteration with hipoplasia and maxillary retrussion. The preoperative work-up included cephalometric and dental study, and a cast model was done to asses the theoretical benefic of the osteotomy. All these patients underwent orthodontic treatment before and after surgery. The results have been good or very good in 96% of the cases. The ortognatic surgery offers significant aesthetic and functional improvement to these patients.

[Experimental free muscle transplantation. Is autologous graft on the distal esophagus viable?]. / Trasplante muscular libre experimental. Es viable el implante autólogo en esófago distal?

UNLABELLED: Experimental free muscle transplantation has resulted in some successful clinical applications. AIM: The possibility that this type of transplantation could act as a sphincteric mechanism motivated us to start by assessing the viability of autologous skeletal grafts on the distal esophagus of laboratory animals. MATERIAL AND METHODS: Twenty transplants of previously denervated free plantaris muscle grafted on the distal esophagus of Sprague-Dawley rats were evaluated at the 1st, 2nd, 4th, 8th and 16th posttransplant week. Histological and histochemical studies were performed to evaluate general features of the grafts and the muscle fibers condition. RESULTS: One and two weeks after transplant the grafts show large areas of necrosis with inflammatory infiltrate. Between the 2nd and the 4th week, as revascularization and motor endplates become significant, the areas of necrosis begin to regress and they almost disappear by the 8th week. Since the 4th week after transplant, regenerated muscle fibers demonstrate morphological and biochemical features similar to normal. CONCLUSIONS: Experimental free plantaris muscle transplantation on the distal esophagus is viable and shows revascularized and reinnervated muscle fibers from the 8th week after transplant on, and at least until the 16th. These fibers have the structural and metabolic properties enabling contractile function. This original model may allow further investigation of some features related to pathophysiology and therapy of gastroesophageal reflux.

[Post-traumatic facial deformity. Globulomaxillary cyst]. / Deformidad facial postraumática. Quiste glóbulo-maxilar.

The case of a child treated for globulomaxillary cyst is presented. He first consulted because of progressive deformity of the face and upper gum, starting several months before. He had a history of facial contusion at the time the deformity began.

[Chronic granulomatous disease: the surgical aspects]. / Enfermedad granulomatosa crónica: aspectos quirúrgicos.

Chronic granulomatous disease is characterized by recurrent infections that have an aggressive course in spite of medical treatment. Surgery is necessary, starting early in life, to treat the infections episodes. We present four patients affected by recurrent infections episodes needing a variety of treatments, who were finally diagnosed with chronic granulomatous disease. Our aim is to emphasize the importance of clinical suspicion in order to make an early accurate diagnosis, and to underline the role of surgery in the resolution of the infection spells that occur along the file of the pediatric patients affected by this disease.

[Usefulness and indications for Port-a-Cath in children with acute leukemia]. / Utilidad e indicaciones del Port-a-Cath en niños afectos de leucemia aguda.

Duration of treatment for acute leukemia is as long as 2 years. During this time, venous punctures are repeated every few days. The Port-a-Cath system allows a long-lasting but intermittent venous access. Since March 1987, 11 Port-a-Cath systems have been implanted in 11 children (age 3 to 14) with the diagnosis of high risk acute leukemia (9 ALL and 2 AML). The facial vein has been used in five instances, the external jugular vein in five, and the jugular vein in one case. Ten systems have had a good function. Seven are still in use, and the duration ranges from 450 to 47 days. Two children died because of their disease, their catheters still functioning. Two systems have been withdrawn, one for incorrected location and the other for cutaneous necrosis of the puncture site. Two more children developed reversible thrombosis. The Port-a-Cath system is useful in children affected by high risk acute leukemia, ameliorating their life quality and avoiding the need for repeated vein punctures.

[Reflux esophagitis in an experimental model. Are the systemic consequences attributable to the esophagitis?]. / Esofagitis por reflujo en un modelo experimental. Son las repercusiones sistémicas atribuibles a las esofagitis?

The esophagojejunostomy model of reflux esophagitis has been shown to cause nutritional deterioration and alterations of hematologic parameters. However, these disturbances have been assumed rather than proved to be the consequence of esophagitis. Little attention has been paid to the anatomical and physiological alterations related to the reflux-inducing operation. We produced reflux esophagitis by an end-to-side esophagojejunostomy (EJ) in Sprague-Dawley rats. These rats were compared to a second group with a similar operation not leading to reflux esophagitis (Roux-en-Y esophagojejunostomy, R-Y). Sham operated animals were used as controls. Erosive esophagitis was produced in all but one of the EJ subjects at day 14, and in none of the R-Y animals. Significant weight loss (-21.7%), anemia (HCT, -17.3%) and hypoproteinemia (-14.2%) were seen in EJ model with respect to sham group. However, a similar variation of weight (-28.7%) and hematocrit (-26.2%) were also found in Y-R group; therefore, these parameters seemed to correlate more with the surgical challenge itself than with the esophagitis.