Surgical correction of ectopic penis and scrotum associated with bilateral orchidopexy / Correção cirúrgica de pênis e escroto ectópicos associada à orquidopexia bilateral

ABSTRACT Ectopic penis is usually associated with penoscrotal transposition, and it is rarely observed in isolation. We report a surgical approach for an extremely rare case. A 10-year-old male patient with bilateral cryptorchidism and ectopic penis and scrotum in perineal area, with no penoscrotal transposition, representing an association not yet described in literature. A previous orchiopexy failed due to ectopic scrotum. By means of an inverted Y incision, the penis was mobilized and a perineal skin flap in form of a testicular sac was prepared. Finally orchiopexy was performed. The surgery was essential to treat cryptorchidism and to improve the self-image of the patient.

RESUMO O pênis ectópico geralmente ocorre associado à transposição peno-escrotal, sendo raro isoladamente. Relatamos uma abordagem cirúrgica para um caso extremamente raro. Tratava-se de paciente do sexo masculino, 10 anos, com criptorquidia bilateral e pênis e escroto ectópicos, na região perineal, sem transposição peno-escrotal, representando uma associação ainda não descrita na literatura. Orquidopexia prévia sem sucesso, devido à ectopia do escroto. Por meio de uma incisão em Y invertido, mobilizou-se o pênis e preparou-se um retalho da pele perineal em forma de bolsa testicular. Por fim, realizou-se a orquidopexia. A cirurgia foi fundamental para tratar a criptorquidia e promover ganho na autoimagem do paciente.

Transposición penoescrotal completa: presentación de dos casos y revisión de la literatura / Complete penoscrotal transposition: A two case report and literature review

La transposición penoescrotal es una anomalía congénita donde el escroto se localiza en posición superior y anterior al pene. Se puede clasificar según su compromiso en transposición completa o incompleta, siendo más frecuente esta última. Se han reportado alrededor de 27 casos en la literatura de transposición penoescrotal completa y la gran mayoría se ha asociado con otras malformaciones congénitas. Presentamos dos casos de óbitos fetales con transposición penoescrotal completa con malformaciones congénitas, y además realizamos una revisión de la literatura de los casos publicados.

Penoscrotal transposition is a congenital anomaly in which the scrotum is positioned superiorly and anteriorly to the penis. It may be classified as complete or incomplete transposition, according to severity. Incomplete type is more common. Around 27 cases of complete penoscrotal transposition have been reported in the literature usually associated with other congenital malformations. We report on two cases on fetal obitus with complete penoscrotal transposition associated with congenital malformations. We also reviewed the literature on published cases.

Manejo quirúrgico del linfedema escrotal severo / Surgical management of severe scrotal lymphedema

Resumen:El linfedema consiste en la retención de líquido linfático en los tejidos, causada por una obstrucción del sistema linfático. El sistema linfático devuelve el líquido intersticial al conducto torácico y este luego al torrente sanguíneo, y de allí se recircula a los tejidos. Puede ser un fenómeno aislado o no, tal como manifestación de una displasia linfática congénita (primaria), desarrollada durante la etapa tardía de linfangiogénesis; o puede ser secundario, es decir, causado por una lesión u obstrucción en los vasos linfáticos, ya sea por disección quirúrgica radical, irradiación, malignidad, linfangitis con linfagioesclerosis, trauma o causa idiopática. Los síntomas pueden incluir fatiga grave, edema focal o generalizado en algunas regiones del cuerpo, así como descoloración de la piel que recubre el linfedema y, finalmente, la deformidad, que en el caso de las extremidades inferiores se llama elefantiasis. El linfedema no solo puede causar desfiguración, sino también limitación del estilo de vida, e incluso trastornos psicológicos. Se presenta el caso de un paciente de 45 años, portador de linfedema severo (elefantiasis) de miembros inferiores crónico y del saco escrotal, valorado y manejado quirúrgicamente en el Servicio de Cirugía Plástica y Reconstructiva del Hospital México.

Abstract:Lymphedema is a localized retention of lymphatic fluid in the tissues, usually caused by a blockage of the vessels that carry lymph. The lymphatic system returns the interstitial fluid to the thoracic duct and this in turn, brings it to the bloodstream, where it is recirculated to the tissues. It may be an isolated phenomenon or not, as a manifestation of a congenital lymphatic dysplasia (primary) developed during the late stage of lymphangiogenesis; or it may be secondary, caused by an injury or blockage to the lymphatic vessels, either by radical surgical dissection, irradiation, malignancy, lymphangitis with lymphangiosclerosis, trauma or of an idiopathic cause. Symptoms may include severe fatigue, focal or generalized edema of some regions of the body, as well as discoloration of the skin overlying the affected area, and eventually deformity, which is called elephantiasis when it refers to the lower limbs. Lymphedema not only may cause disfiguration, but also can limit lifestyle, and even cause psychological disorders. We present the case of a 45-year old patient with severe chronic lower limb (elephantiasis), and scrotal sac lymphedema, who was evaluated and successfully treated by the Department of Plastic and Reconstructive Surgery at the Hospital Mexico.

Baraitser-winter syndrome: an additional Egyptian patient with skeletal anomalies, bilateral iris and choroid colobomas, retinal hypoplasia and hypoplastic scrotum

We report a 3.5 year old male child, second in order of birth of non consanguineous Egyptian parents with Baraitser-Winter syndrome [BRWS]. The patient had bilateral colobomas of the iris and choroid. Our patient had also retinal hypoplasia, which was not reported previously in this syndrome, bilateral congenital ptosis, hypertelorism, moderate mental retardation, short stature, short neck, hyperextensibility of the joints of the hands, talipes equinovarus, kyphoscoliosis and unilateral hypoplastic scrotum and testis

Duplicación rectal extrofiada asociada a malformación anorrectal y transposición pene-escrotal con hipospadias perineal: Reporte de un caso clínico / Exstrophy of rectal duplication associated with anorectal malformation and penoscrotal transposition with perineal hypospadias: A case report

Se presenta el caso de un paciente masculino quien requirió tratamiento por agenesia anorrectal con fístula rectouretral y transposición pene-escrotal con hipospadias perineal, acompañados de una masa perineal. La tumoración perineal se encontró íntimamente adherida y en continuidad al recto, lo que la hace compatible con una duplicación rectal extrofiada. La reconstrucción quirúrgica de la anomalía se realizó en etapas hasta lograr resultados funcionales y estéticos aceptables.

We present the case of a male patient who required treaatment due to anorectal agenesis with recto urethral fistula and penoscrotal transposition with perineal hypospadias, associated with a perineal tumor. The perineal tumor was found strongly adhered and contiguous to the rectum which makes it compatible with an exstrophy of rectal duplication. Surgical reconstruction of the birth defect was performed in stages until acceptable biological function and esthetic results were obtained.

Understanding the processus vaginalis: the abdomino scrotal hydrocoele / Entendiendo el proceso vaginal: el hidrocele abdomino-escrotal

A knowledge of the embryology and descent of the testes is essential for all clinicians. Congenital hydrocoele, though an embryologic anomaly can present in adulthood. A abdomino scrotal hydrocole must be considered when a mass in the lower abdomen co exists with a scrotal hydrocoele.

El conocimiento de la embriología y el descenso de los testículos es esencial para todos los clínicos. El hidrocele congénito, aunque es una anomalía embriológica se puede presentar en la edad adulta. Un hidrocele abdomino-escrotal debe ser considerado cuando una masa en la parte inferior del abdomen co existe con un hidrocele testicular.

Transposição penescrotal / Penoscrotal transposition

Transposição de pênis e escroto no ser humano é uma rara anomalia da genitália externa, caracterizada pela má posição do pênis em relação ao escroto, podendo ser na forma incompleta (parcial) ou completa. Frequentemente a transposição de pênis está associada a anomalias do genital (hipospadia, uretra curta), sistema renal, esquelético, gastrointestinal, cardiovascular e, ocasionalmente, a síndrome de regressão caudal. A reconstrução da transposição penescrotal, com prioridade a correção do ?chordee?, deverá produzir resultados satisfatórios, em termos estético e funcional, desde que o escroto e o pênis sejam colocados em suas posições anatômicas verdadeiras, evitando assim efeitos psicogênicos adversos.

Ectopia testicular perineal: presentación de un caso / Perineal testicular ectopia: a case report

La ectopia testicular perineal es una anomalía congénita infrecuente y se incluye dentro del síndrome de escroto vacío. Se reporta el caso de un paciente con escroto derecho vacío y masa perineal ipsolateral palpable, asociada a hernia inguinal derecha. El paciente fue operado en el Hospital Pediátrico Universitario de Centro Habana. Durante la exploración quirúrgica el testículo derecho y su gubernaculum testis se encontraban fijados en el perineo derecho. Las características del testículo y sus elementos eran normales. El testículo se fijó en el escroto derecho sin dificultad. La detección precoz de esta patología y su corrección quirúrgica en el tiempo establecido favoreció la funcionalidad del órgano.

Perineal testicular ectopia is a rare congenital anomaly and it is included in the empty scrotum syndrome. The case of a patient with empty right scrotum and a palpable ipsilateral perineal mass associated with a right inguinal hernia is reported. The patient was operated on in Centro Habana University Children Hospital. During the surgical exploration, the right testicle and its gubernaculum testis were fixed to the right perineum. The characteristics of the testicle and its elements were normal. The testicle was fixed to the right scrotum without difficulty. The early detection of this pathology and its surgical repair on time, favored the functionality of the organ.

Necrosis de escroto en adolescente / Necrosis of the scrotum in an adolescent

Paciente de 19 años, sin antecedentes mórbidos de importancia, que presentó necrosis y úlceras genitales sensibles durante un período de cuatro meses y medio, en relación a cuadros infecciosos amigdalianos y respiratorios.

We present the case of a 19-year-old patient, lacking significant morbid antecedents, who presented necrosis and sensitive genital ulcers during a period of four and a half moths, related with tonsillitis and respiratory infections.

Incisión pre-escrotal: una alternativa quirúrgica para la corrección de los trastornos inguino-escrotales / Pre scrotal access: a surgical alternative for inguino-scrotal disorders correction

La incisión inguinal ha sido la vía de acceso para la mayoría de los trastornos inguino-escrotales. Presentamos nuestra experiencia con el uso de la incisión pre-escrotal para el abordaje quirúrgico de los trastornos que atañen el canal inguinal y los testículos. La incisión inguinal para el tratamiento quirúrgico de los trastornos inguino-escrotales ha sido el acceso clásico; los elementos del cordón inguinal pueden ser de fácil identificación y rápida corrección. Con el uso de esta técnica, en la corrección de los TNDPs hay necesidad de una segunda incisión para garantizar que los testículos alcancen el escroto.

Congenital adrenal hyperplasia and pseudodiphallia, new association

We are presenting and to the best of our knowledge a new neonatal presentation of congenital adrenal hyperplasia, where a male newborn was born with pseudodiphallia and proved to have congenital adrenal hyperplasia. The metabolic derangement end product of congenital adrenal hyperplasia may have affected the fetus external genitalia leading to development of this anomaly. We therefore, presented this case because of this rare association

Yunis-Varon syndrome.

Yunis-Varon syndrome is a rare, autosomal recessive syndrome characterized by growth retardation, defective growth of the cranial bones along with complete or partial absence of the clavicles (cleidocranial dysplasia), characteristic facial features, and/or abnormalities of the fingers and/or toes.

Umbilical cord allantoic cysts in a newborn with vacterl association.

Allantoic cysts of umbilical cord are very rare. A preterm, low birth weight, male newborn with a new constellation of anomalies, allantoic duct cysts in the umbilical cord and associated perinatally lethal malformations of VACTERL sequence, is reported. In addition, the neonate also had duodenal atresia, patent urachus, obstructive uropathy and bifid scrotum. Association of Allantoic cysts with VACTERL sequence has not been described earlier.

Perineal accessory scrotum.

A boy with perineal accessory scrotum but without any other congenital anomaly is being described. He presented with a rugose skin tag on a midline perineal mound and the diagnosis could be confirmed by the histological findings of subcutaneous smooth muscles. Other reports of perineal scrota have been reviewed and a simple classification has been proposed for such cases, based on the appearance of the accessory scrotum and the associated anomalies.

Sacrococcygeal teratoma with anorectal malformation.

A 7-month-old child presented with imperforate anus, penoscrotal hypospadias and transposition, and a midline mucosa-lined perineal mass. At surgery the mass was found to be supplied by the median sacral artery. It was excised and the anorectal malformation was repaired by posterior sagittal anorectoplasty. Histologically the mass revealed well-differentiated colonic tissue. The final diagnosis was well-differentiated sacrococcygeal teratoma in association with anorectal malformation.

Versatility of pedicled anterolateral thigh flap for the coverage of the inguinoscrotoperineal defect

The main nutrient vessel of the anterolateral thigh flap is the perforator originating from the descending branch of the lateral circumflex femoral artery [LCFA].it supplies a large area of skin on the anterolateral aspect of the thigh. We report here a case of wide inguinoscrotoperineal defect in a young male patient following a post necrotizing fascitis defect which was covered by a pedicled anterolateral thigh flap. The result was satisfactory. The anterolateral thigh flap has the advantage of a long vascular pedicle, large caliber vessels, availability of large skin flap area, and is also suitable as a pedicle, free or flow through flap