Manejo quirúrgico de la aplasia cutis congénita / Surgical management of aplasia cutis congenita

Introducción: La aplasia cutis congénita (ACC) es una malformación congénita rara que afecta sobre todo al cuero cabelludo, aunque puede afectar al pericráneo, el cráneo y la meninges. Las complicaciones pueden llegar a ser fatales, por lo que es necesario un tratamiento oportuno. El tratamiento sigue siendo controvertido, sin encontrar un consenso entre el abordaje conservador y el quirúrgico. El objetivo de este estudio es describir nuestra experiencia en el manejo de la ACC. Material y métodos: Estudio descriptivo retrospectivo de las historias clínicas de los pacientes menores de 14 años con diagnóstico de ACC, atendidos entre el año 2000 y el 2013. Resultados: Veintidós casos de ACC con lesiones que variaban de 1cm (0,79 cm2) a 14cm (153,94 cm2). Dieciocho casos presentaron lesiones en el cuero cabelludo, 3 en extremidades y uno en tronco. Se realizó tratamiento conservador en 9 y quirúrgico en 13 (8 cierres primarios, 2 plastias, 2 injertos cutáneos y un colgajo). Dos pacientes fallecieron por complicaciones de otras patologías no asociadas a la ACC. Conclusiones: La ACC es infrecuente y puede tener un desenlace fatal. Para prevenirla es necesaria una evaluación inicial completa para establecer un tratamiento oportuno. La cirugía es una buena opción terapéutica, sobre todo en defectos con diámetro>4cm (12,6 cm2), ya que disminuye el riesgo de complicaciones mortales (AU)

Introduction: Aplasia cutis congenita (ACC) is a rare congenital malformation that commonly involves the scalp, but can affect pericranium, bone and dura mater. Complications are rare, but can be fatal, so early treatment must be achieved. The treatment remains controversial with no consensus between the conservative and surgical approach. The aim of this study is to describe our experience in the management of ACC. Material and methods: Retrospective review of the medical records of all children up to 14 years diagnosed with ACC and treated between 2000 and 2013. Results: There were a total of 22 cases of ACC with lesions ranging from 1cm (0.79 cm2) to 14cm (153.94 cm2). ACC of the scalp was found in 18 cases, with 3 in extremities and 1 in trunk. Conservative treatment was performed on 9 patients and 13 underwent surgical treatment (8 primary closures, 2 plasties, 2 skin grafts, and 1 skin flap). Two patients died due to complications of other diseases not related with the ACC. Conclusions: ACC is a rare disease that can be fatal. A complete initial assessment to establish early treatment is necessary to prevent this. Surgery should be considered as an initial therapeutic option in defects >4cm (>12.6 cm2) as it prevents the risk of fatal complications (AU)

[Surgical management of aplasia cutis congenita]. / Manejo quirúrgico de la aplasia cutis congénita.

INTRODUCTION: Aplasia cutis congenita (ACC) is a rare congenital malformation that commonly involves the scalp, but can affect pericranium, bone and dura mater. Complications are rare, but can be fatal, so early treatment must be achieved. The treatment remains controversial with no consensus between the conservative and surgical approach. The aim of this study is to describe our experience in the management of ACC. MATERIAL AND METHODS: Retrospective review of the medical records of all children up to 14 years diagnosed with ACC and treated between 2000 and 2013. RESULTS: There were a total of 22 cases of ACC with lesions ranging from 1cm (0.79 cm(2)) to 14cm (153.94 cm(2)). ACC of the scalp was found in 18 cases, with 3 in extremities and 1 in trunk. Conservative treatment was performed on 9 patients and 13 underwent surgical treatment (8 primary closures, 2 plasties, 2 skin grafts, and 1 skin flap). Two patients died due to complications of other diseases not related with the ACC. CONCLUSIONS: ACC is a rare disease that can be fatal. A complete initial assessment to establish early treatment is necessary to prevent this. Surgery should be considered as an initial therapeutic option in defects >4cm (>12.6 cm(2)) as it prevents the risk of fatal complications.

Abdomen agudo secundario a invaginación apendicular / Acute abdomen secondary to appendiceal intussusception

La invaginación apendicular es una entidad rara que puede ocurrir a cualquier edad. Actualmente, están descritos pocos casos, en su mayoría hallazgos intraoperatorios. La conducta terapéutica suele ser quirúrgica, variando desde una apendicectomía hasta una hemicolectomía, principalmente para la toma de biopsia y descartar neoplasias. Se presentan 3 casos de ni˜nos menores de 14 a˜nos intervenidos por cuadro de dolor abdominal agudo localizado en la fosa iliaca derecha, uno de ellos con diagnóstico preoperatorio mediante ecografía y los demás con hallazgo intraoperatorio de invaginación del apéndice cecal, con apendicitis aguda confirmada histológicamente

Appendiceal intussusception is a rare condition that can occur at any age. Only a few cases have been reported, and most are found during surgery. The therapeutic approach is usually surgical, ranging from an appendectomy to a hemicolectomy, primarily for biopsy and to rule out possible malignancy. Three cases of children under 14 years who underwent surgery for acute abdominal pain located in the right iliac fossa are presented; one with preoperative diagnosis by ultrasound, and the other two with positive intraoperative findings of intussusception of the cecal appendix, with acute appendicitis being histologically confirmed

[Acute abdomen secondary to appendiceal intussusception]. / Abdomen agudo secundario a invaginación apendicular.

Appendiceal intussusception is a rare condition that can occur at any age. Only a few cases have been reported, and most are found during surgery. The therapeutic approach is usually surgical, ranging from an appendectomy to a hemicolectomy, primarily for biopsy and to rule out possible malignancy. Three cases of children under 14 years who underwent surgery for acute abdominal pain located in the right iliac fossa are presented; one with preoperative diagnosis by ultrasound, and the other two with positive intraoperative findings of intussusception of the cecal appendix, with acute appendicitis being histologically confirmed.

Focal nodular hyperplasia on accessory lobe of the liver: preoperative diagnosis and management.

We report the case of a 13 year-old girl transferred to our department with a one month history of vomiting, weight loss, and abdominal mass that was diagnosed as Focal Nodular Hyperplasia in an accessory lobe of the liver. Accessory liver lobe is a rare congenital anomaly whose preoperative diagnosis is difficult and usually presents as an incidental finding intraoperatively. Depending on their location and size, they can present as acute abdomen or abdominopelvic mass. In the literature reviewed, we found no association of focal nodular hyperplasia and accessory liver lobe in children.

Miofibroma infantil solitario congé nito. Presentación de dos casos / Congenital solitary infantile myofibroma: Report of two cases

La miofibromatosis infantil es una patología rara que suele afectar a niños menores de 2 años. Más de la mitad son congénitos, localizándose en cabeza-cuello (..) (AU)

The infantile myofibromatosis is a rare pathology usually affecting children under 2 years. More than half are congenital, located in head and neck in a 30% of the (..) (AU)

[Congenital solitary infantile myofibroma: report of two cases]. / Miofibroma infantil solitario congénito. Presentación de dos casos.

UNLABELLED: The infantile myofibromatosis is a rare pathology usually affecting children under 2 years. More than half are congenital, located in head and neck in a 30% of the cases. Myofibroma is a benign mesenchymal tumor that appear an isolated lesion or multiple, with or without visceral involvement. Case 1: A newborn girl that presents lump in scalp suspected of encefalocele. Ultrasound and RM: solid and heterogeneous mass 4 x 2 cm with increased peripheral vascularisation. Case 2: A 7-month-old baby boy with lump in right occipital zone detected at 4 months. Ultrasound and RM: injury 1 x 1.5 cm in head esplenio muscle suggestive of hemangioma. RESULTS: After surgical total resection, the histological study and immunohistochemical identified the lesion as myofibroma. The evaluation of disease dissemination was normal and the recovery of the patients was favorable. The differential diagnosis of myofibroma must be carried out with other mesenchymal tumors and with non neoplastic injuries characteristics of the region affects. The visceral location and multiple forms worsen the prognosis, so is important do an exhaustive study. Treatment is conservative surgery, but if there is no risk to the child, the possibility of spontaneous regression makes the abstention therapeutic a correct alternative.

[First results with Integra artificial skin in the management of severe tissue defects in children]. / Primeros avances en el tratamiento del niño gran traumatizado con piel artificial Integra.

In extreme cases of tissue defects, wound coverage after excision may be problematic because of the limited existence of donor sites. An option for temporary wound coverage used in the management after early scar release is a dermal substitute: Integral artificial skin. The biosinthetic material consists of un upper silicone film and a lower layer of porous cross-linked bovine collagen and chondroitin-6-sulfate as a template for dermal regeneration after staged tangencial necrectomy. In the third and fourth weeks following application the silicone layer was easily removed and the newly formed neodermis covered with widely unmeshed thin split thickness autograft. We report 6 patients with tissue defects with open fractures in three cases, one hemorrhagic necrosis of the skin by meningococcemia who requiring amputations of all four extremities and two traumatic necrosis. Other patient has a retractil scar. All were treated with Integral and epidermical autograft. The good results with Integral regarding recovery may affect initial treatment and reconstruction planning after extensive wound injuries with tissue defects to obtain immediate wound closure.

Primeros avances en el tratamiento del niño gran traumatizado con piel artificial integral / First results with integra artificial skin in the management of severe tissue defects in children

En casos extremos, cuando existen grandes pérdidas de sustancia, pueden existir problemas para cubrir las lesiones debido a la limitación de zonas dadoras de autoinjertos. Una alternativa para la cobertura temporal de la lesión, usada tras la escarectomía precoz, es un sustituto dérmico o piel artificial Integra. Este material biosintético consta de una lámina superior de silicona y otra inferior formada por fibras de colágeno bovino entrelazadas y condroitín-6-sulfato, que proporciona una regeneración dérmica tras la escarectomía tangencial. Entre la 3° y la 4° semanas siguientes, se practica retirada de la lámina de silicona tras la formación de una neodermis, que se cubre con un autoinjerto epidérinico no mallado de espesor fino. Presentamos seis pacientes con pérdidas de sustancia graves y extensas, secundarias a fracturas abiertas en tres casos, una necrosis por sepsis meningocócica que requirió amputaciones de los cuatro miembros, dos necrosis por aplastamiento y una secuela retráctil. Todos fueron tratadas mediante implante de Integra y autoinjerto epidérmico. Los buenos resultados observados indican que Integra constituye un tratamiento útil e innovador para la cobertura de pérdidas críticas de sustancia, cuando debido a la extensión de las lesiones no es posible una cobertura inmediata de las mismas. (AU)

[Index finger pollicization for congenitally deficient first finger of the hand in children]. / Pulgarización en los déficits del primer rayo en la mano del niño.

Pollicization is a single-stage neurovascular pedicle transfer of the index digit to function as a thumb. The objective of this study is to investigate the results of index finger pollicization for correction of congenital deficiency of the first ray in pediatric hand. We have done 6 pollicizations of index fingers in 6 hands (there were 2 right hands, 2 left hands, and 1 bilaterally) in 5 patients (4 boys and 1 girl) who had absent or nonfunctioning thumbs (type III-V of Blauth's classification). Associated anomalies where numerous and included radial club hand, mirror hand and cardiovascular and urologic anomalies. The average time of Kirschner wire extraction was 32 days (30 to 36 days) and to beginning the hand rehabilitation at 5 degrees to 10 degrees day. The average age at pollicization was 5.5 years (range 2 to 8 years), and follow-up averaged 8 years (5 to 11 years). The cosmetic and functional results were excellent, with manual dexterity of prehension and opposition. Pollicization in children can be performed at least 2 years of age, to due of minor risk of neurovascular lesion but without delayed the cortical representation of the pollicized finger.

Síndrome de bandas de constricción congénitas / Congenital constriction band syndrome

Se presentan 7 pacientes pediátricos con bandas de constricción congénitas,tratadas en un solo tiempo quirúrgico mediante la escisión del surco de constricción anular,junto con plastias en Z múltiples.En todos los casos se obtuvieron excelentes resultados estéticos y funcionales,sin complicaciones ni lesiones vásculo-nerviosas

Síndrome de bandas de constricción congénitas / Congenital constriction band syndrome

Se presentan 7 pacientes pediátricos con bandas de constricción congénitas,tratadas en un solo tiempo quirúrgico mediante la escisión del surco de constricción anular,junto con plastias en Z múltiples.En todos los casos se obtuvieron excelentes resultados estéticos y funcionales,sin complicaciones ni lesiones vásculo-nerviosas

[Bone lengthening in children]. / Alargamientos óseos en niños.

Since June 1987, were realized 45 limb lengthening (40 lower and 5 upper) in 34 children with between 3-15 years old. A dynamic axial fixator (DAF) system with telescoping capabilities, permits a slow distraction through a osteotomy and callotasis in 36 bones, and chondrodiatasis in 5. Twenty-six unilateral lengthening were realized and 5 alineation-lengthening, 20 congenital and 11 acquired causes. 14 bilateral lengthening were done in 5 patients (4 achondroplastic and one Turner's syndrome). The average increase in length was 5.5 cm. for limb and ranged from 4.3 to 8.0 cm. The healing index was 32.1 days for each centimeter of length gained in the femurs and 5.2 cm. with 28.2 days/cm. in the tibias. The complications were superficial pin tract infection (73%), that were not generally treated with antibiotic, 2 knee valve, 2 knee subluxation and one patella subluxation with knee flexion contracture.

[Treatment of radial club hand with double articulated external fixators]. / Tratamiento de la mano zamba radial mediante doble montaje articulado con fijadores externos.

Congenital absence of the radius is a rare malformation, characterized by radial deviation of the hand to more than 90 degrees. Early treatment with plaster cast use to be enough to correct radial deviation, making easier surgical correction on several years later. We report a case of a newborn with radial club hand absence of the thumb of the right hand, bilateral hydronephrosis and bilateral cryptorchidism. Was treated with a new procedure, permitting wrist centralization, coupled with osteotomy and callostasis of the ulnar diaphysis using external fixation. We obtained an important success with this serial lengthening of the ulna.

[Protocol for the treatment of hypertrophic cicatrix with silastic gel. First results]. / Protocolo para el tratamiento de cicatrices hipertróficas con gel de silastic. Primeros resultados.

Functional limitation and scarring use to be common sequelaes of a burns injury in children. We report our initial experience with silicone gel (silastic gel shetting) in the treatment of 15 children with hypertrophic scars, without success to other alternatives therapies. Improvements in texture, colour and height of the lesion were noted in all cases.

[Congenital sternal cleft]. / Hendidura esternal congenita.

We report two congenital sternal cleft cases, Ravitch type 1, and treated in our hospital during a period of five teen years. This is a rare malformation and the experience is poor. Surgical primary close was realized into the first month of life with satisfactory evolution.

[Limb lengthening in children]. / Alargamiento de miembros en niños.

Limb lengthening is a common used callotasis technique. A dynamic axial fixator system with telescoping capabilities permits a slow distraction and becomes a proximal submetaphyseal corticotomy callus. In the last two years we realized seven limb lengthening in children under fourteen years old, five of them were unilateral dismetry and one of them an achondroplasic girl bilateral. Of them all we had six easy resoluting complications.

[Digito-lateral flaps in the treatment of retraction after hand burns in children]. / Colgajos digito-laterales para el tratamiento de las secuelas retráctiles post-quemadura en la mano del niño.

The hand's severe burns are frequent in the children. The skin of the hand is bordering skin which develop towards a retractable consequence with scar. In a sample of 10 children with this consequence same type of digital side flap in 26 fingers is carried. A great uniformity of the results is obtained, the functional normality has reached to 96% and a low index of complications has arisen which can't be attributed to the technique.

[Experimental study on the process of repair-healing of the flexor system in the growing chick]. / Estudio experimental del proceso reparación-cicatrización del aparato flexor del pollo en crecimiento.

In the present work, in view of the concern for tendinous injuries in children hand flexor apparatus, tendinous healing and its functional and morphological recuperation were studied after section and surgical restoration of the leg flexor apparatus of chicken at different growth stages. We conclude that best results are obtained when the rehabilitation begins ten days after surgery.

[Prospective study of equinovarus foot in 20,000 live newborn infants]. / Estudio prospectivo del pie equinovaro en 20.000 recién nacidos vivos.

We report a prospective study about congenital clubfoot in 20,000 alive newborns and also a critical analysis about the treatment results. The frequency was 1.65 per thousand and sex ratio almost 2:1 for males. In springtime the incidence was higher. Surgical treatment was performed in less than 50% of the affected feet. There were excellent and good results in 90% of the feet.