Primary closure of a sternal cleft in the neonatal period.

Sternal cleft is a rare malformation of the midline fusion of the sternal bars; the most common form is the superior partial defect. Surgical correction with primary closure is the gold standard. It is recommended that the procedure be performed before 3 months of age because of the greater compliance and maximal flexibility of the thoracic wall.  These features ensure a safer repair with a low risk of complications and allow for a less extensive procedure that does not require the use of additional techniques. A midline incision is performed in the anterior thoracic wall, and the major pectoralis flaps are raised. The main surgical goal is to change the remaining sternum from a U to a V shape. Transfixing interrupted sutures are placed in the cartilaginous borders for midline closure. Hemodynamics and ventilation are monitored at this time. Closure is performed by layers.

Accessory Soleus: A Case Report of Exertional Compartment and Tarsal Tunnel Syndrome Associated With an Accessory Soleus Muscle.

An accessory soleus muscle is a rare anatomic variant that frequently presents as an asymptomatic soft tissue swelling in the posteromedial ankle. Less frequently, the anomalous muscle can cause pain and swelling with activity. We present the case of a 17-year-old male with exertional compartment syndrome and associated tarsal tunnel syndrome secondary to a very large accessory soleus muscle. After surgical excision, the patient was able to return to full activity with complete resolution of symptoms.

Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus / Clinical and psychosocial challenges in the treatment of a patient with Proteus syndrome

A Síndrome de Proteus é uma rara associação de malformações que podem afetar vários tecidos e órgãos. É caracterizada por macrodactilia bilateral, hipertrofia craniana, anomalias ósseas, escoliose, hamartomas de tecidos moles, nevo verrucoso pigmentar, anormalidades viscerais e outras hipertrofias. Há pouco mais de 200 casos notificados em todo o mundo. O presente artigo relata a evolução clínica de um paciente pediátrico com essa síndrome. A criança apresentou desnutrição grave associada ao extremo gigantismo de membros inferiores. Além disso, apresentou repercussões psicossociais relacionadas à exclusão social. A doença tornou-se mais grave e progrediu como síndrome consumptiva. Finalmente, os pais concordaram com a amputação dos membros inferiores hipertróficos. Um ano após as amputações, a criança estava totalmente reabilitada, protetizada dos membros amputados, com melhora nutricional, além de apresentar notória recuperação psicológica e reinserção social, o que representou melhora significativa da qualidade de vida para o paciente.

Proteus syndrome is a rare combination of malformations that can affect several tissues and organs. It is characterized by bilateral macrodactyly, cranial hypertrophy, bone anomalies, scoliosis, soft-tissue hamartomas, verrucous pigmented nevus, visceral abnormalities and other forms of hypertrophy. Just over 200 cases have been reported worldwide. This article reports on the clinical course of a pediatric patient with this syndrome. The child had severe malnutrition associated with extreme gigantism of the lower limbs and also psychosocial problems related to social exclusion. As the disease progressed it exacerbated and evolved into a wasting syndrome. After several years, the parents agreed to amputation of the hypertrophic lower limbs. One year after the amputations the child had been rehabilitated and had adapted to prostheses, with nutritional improvement and notable psychological recovery and social reintegration, which represented a significant improvement in his quality of life.

Transposition flap for correction of ectopic breast in Poland's syndrome using a pre-molded silicone prosthesis / Retalho de transposição para correção de mama ectópica em síndrome de Poland usando prótese de silicone pré-moldada

The authors report a variation of Poland's syndrome with ectopic right breast. Three surgeries were performed at different times: transposition flap to place the mammary gland in an anatomic position; reduction of the contralateral left breast; and implantation of a pre-molded silicone prosthesis to correct the malformation caused by the absence of the major and minor pectoral muscles. The surgeries were performed at six-month intervals, and the final outcome was new and acceptable positioning of the breasts.

Os autores apresentam uma variante da síndrome de Poland com mama direita ectópica. Foram realizados três tempos cirúrgicos: retalho de transposição para colocação da glândula mamária em posição anatômica; redução da mama contralateral esquerda; e implantação de prótese de silicone pré-moldada para correção do defeito causado pela agenesia dos músculos peitoral maior e peitoral menor. Com intervalo de seis meses entre as cirurgias, o resultado final apresenta novo posicionamento aceitável das mamas.

Thoracoscopic cartilage resection with partial perichondrium preservation in unilateral pectus carinatum: preliminary results.

BACKGROUND/PURPOSE: Although minimally invasive repair of pectus excavatum has gained worldwide acceptance, treatment of pectus carinatum is mostly performed with open procedures. Different minimally invasive alternatives have been proposed in the last few years, including subpectoral CO(2) dissection and intrathoracic compression (Abramson technique), or conservative procedures, as dynamic compression system. Recently, another surgical technique has been proposed for the treatment of unilateral pectus carinatum, consisting of a thoracoscopic approach and multiple cartilage incisions. The aim of this work is to present our modification to this approach. METHODS: We have modified this technique by introducing complete cartilage resection of all anomalous costal cartilages, performed thoracoscopically. Three thoracoscopic ports were used. Cartilage is removed progressively using a rongeur and preserving the anterior perichondrium. RESULTS: We have performed this technique in 4 patients during the last year. Follow-up ranged from 6 to 14 months. No intraoperative or postoperative complications were observed. The results, assessed by the patients themselves, were good in 2 cases, quite good in one, and fair in the first patient of our series, who was reoperated using a classical open approach. Pain was well controlled without the need of an epidural catheter. CONCLUSION: Thoracoscopic cartilage resection with perichondrium preservation can be considered as feasible alternative for the treatment of unilateral pectus carinatum.

La minifijación ósea externa en las malformaciones congénitas / The external bony minifixation in congenital malformations

Se realizó un estudio de los distintos procederes quirúrgicos con el sistema de minifijación ósea externa RALCA a 74 pacientes portadores de alguna malformación congénita operados en el CCOI "Frank País" desde 1990 hasta 1999. Se presentan los resultados obtenidos con dicho procedimiento en el difícil tratamiento de estas entidades

Reconstrucción de la pared torácica mediante prótesis sintética moldeada durante el acto operatorio / Abdominal wall of the reconstruction intervening synthetical prothesis during surgery acto

La reparación de la pared torácica consecutiva a resecciones extensas de esqueleto debidas por lo general a neoplasias, debe ser cuidadosamente efectuada con la finalidad de restaurar la rigidez y la estabilidad del torax, previniendo trastornos ventilatorios que de otro modo inevitablemente ocurrirían. A 4 pacientes que habían sido sometidos a la exéresis parcial de varias costillas, 3 de ellos con parte de esternón, se les colocó una prótesis fabricada con malla de marlex y metilmetacrilato. Para confeccionar esa prótesis fue utilizado un molde o matriz de aluminio con la forma y el tamaño de una sección de la pieza operatoria; por consiguiente aquélla se adaptó con bastante precisión a la brecha parietal. Se describe el procedimiento. El resultado funcional fue muy satisfactorio.

Reconstrucción de la pared torácica mediante prótesis sintética moldeada durante el acto operatorio / Abdominal wall of the reconstruction intervening synthetical prothesis during surgery acto

La reparación de la pared torácica consecutiva a resecciones extensas de esqueleto debidas por lo general a neoplasias, debe ser cuidadosamente efectuada con la finalidad de restaurar la rigidez y la estabilidad del torax, previniendo trastornos ventilatorios que de otro modo inevitablemente ocurrirían. A 4 pacientes que habían sido sometidos a la exéresis parcial de varias costillas, 3 de ellos con parte de esternón, se les colocó una prótesis fabricada con malla de marlex y metilmetacrilato. Para confeccionar esa prótesis fue utilizado un molde o matriz de aluminio con la forma y el tamaño de una sección de la pieza operatoria; por consiguiente aquélla se adaptó con bastante precisión a la brecha parietal. Se describe el procedimiento. El resultado funcional fue muy satisfactorio. (AU)