Congenital infantile fibromatosis of the cheek: report of a rare case and differential diagnosis.

Infantile fibromatosis is a benign tumour that consists of dense masses of fibroblasts and myofibroblasts with marked collagen production. Although it is a nonmetastasising tumour, it has significant potential for local invasion and recurrence and may be fatal because of its size and location. The authors report an unusual case of infantile fibromatosis of the cheek in an 8-month-old boy, present since birth. The patient underwent surgical treatment with tumour-free margins and the 6-month follow-up revealed no signs of recurrence. Infantile fibromatosis of the cheek is a very rare condition at this location, especially congenital cases. It should be considered in the differential diagnosis of congenital lesions in childhood.

Total forehead flap: hemicraniofacial giant congenital melanocytic nevus.

Being non-hair-bearing and relatively thin and having similar color and texture of the skin of the forehead provide an excellent characteristic not only for nasal reconstruction but also in other facial areas. A 28-year-old man presented in the Department of Plastic Surgery, Argerich Hospital, Buenos Aires, Argentina, with a giant congenital melanocytic nevus extended on complete left cheek, total nose, both upper and lower lids, ocular globe, and temporoparietal region. Tumor resection and reconstructive aspect were planned in 3 stages. (1) Excision of the tumor located in the cheek, nose, and both upper and lower left lids. The reconstruction of the cheek was made with a total forehead flap. The nose was resurfaced by means of a total skin graft. (2) resection of the temporoparietal nevus. (3) Treatment of the tumor involving the ocular structures. Total forehead flap was outlined, permitting carryout of skin of the total forehead area, and the blood supply was originated from the frontal and parietal branches of the temporal superficial artery and secondarily by anastomosis with the branches arising from both homolateral auricular and occipital vessels. Postoperative control 1 year after surgery showed an excellent aesthetic and functional result not only in the recipient area but also in the donor area.

Successful treatment with vincristine in PHACES syndrome.

We report a case of PHACES syndrome witch was effectively treated using vincristine. A female infant was referred at 8 months of age for evaluation with a segmental mandibular haemangioma with rapid growing and facial disfigurement. The infant was initially placed on oral prednisone with clinical response but she developed obstructive sleep apnoea. Vincristine was started at about 24 months and continued for 4 months with marked decrease in the size of the haemangioma. The only side effect was constipation.

[The hemangioma-sternum bifidum malformation syndrome. Report of a patient]. / Espectro malformativo de hemangioma-esternón bífido. Reporte de un paciente.

We present a male patient with a facial haemangioma extending from the lower left lid to the malar region and the upper left lip. The patient also presents a 1 cm round cutaneous notch on the upper third of the sternum. There are not other physical anomalies. The characteristics of the clinical picture suggest the diagnosis of sternal cleft-haemangiomas malformative spectrum (OMIM 140850).

Prenatal diagnosis of a benign facial tumor.

We report a case of prenatal diagnosis of a benign fetal skin tumor on the chin made at 27 weeks of pregnancy by ultrasound scan. We report this case given the uncommon diagnosis and the unique fetal facial profile seen on ultrasound, resembling the image of an ancient Egyptian pharaoh which made us call it 'Ramses' sign' as a future mnemonic aid to sonographers.

Epignatus: reporte de un caso / Epignathus: report of a case

Se reporta una paciente que mostraba una entidad tumoral congénita extremadamente rara del complejo bucofacial, derivada de restos de tejido embrionario. El epignatus se define como un monstruo doble, en el cual el feto parásito o parte de él, se encuentra inserto en la mandíbula del autósito, aunque pueden ser también pólipos, quistes pilosos, quistes dermoides o teratomas. Esta malformación puede tener un gran tamaño e interferir la alimentación y/o la respiración. Se presenta una paciente con esta entidad, en la cual la disección necrópsica puso en evidencia la irresecabilidad de la tumoración, de haberse podido realizar la intervención quirúrgica

Epignatus: reporte de un caso / Epignathus: report of a case

Se reporta una paciente que mostraba una entidad tumoral congénita extremadamente rara del complejo bucofacial, derivada de restos de tejido embrionario. El epignatus se define como un monstruo doble, en el cual el feto parásito o parte de él, se encuentra inserto en la mandíbula del autósito, aunque pueden ser también pólipos, quistes pilosos, quistes dermoides o teratomas. Esta malformación puede tener un gran tamaño e interferir la alimentación y/o la respiración. Se presenta una paciente con esta entidad, en la cual la disección necrópsica puso en evidencia la irresecabilidad de la tumoración, de haberse podido realizar la intervención quirúrgica

[Cervico-facial heterotopic brain tissue with a mandibular deformity]. / Tejido cerebral heterotópico cervico-facial con deformidad mandibular.

Case report of a premature baby girl who presented with a right cervico-facial mass which caused severe respiratory difficulty. The patient underwent subtotal resection of the mass whose pathologic specimen report revealed heterotopic cerebral tissue compatible with encephalocele. A head and neck CT Scan performed could not evidence a connection between the mass and the cranial cavity, reason for which the possibility of encephalocele was disregarded. After subtotal resection of the mass, the tumor began to grow from soft palate and the patient died from respiratory complications at four months of age. Autopsy reported heterotopic cerebral tissue from neck and soft palate; right lung pneumonia and infection by CMV. Although the heterotopic cerebral tissue was reported as benign, the clinical characterization of this mass is compatible with a malignant behavior due to the aggressiveness of its growth.

Tejido cerebral heterotópico cervico-facial con deformidad mandibular / Cervico-facial heterotopic brain tissue with a mandibular deformity

Este es el caso de una paciente prematura que presentó una masa faciocervical derecha que le provocó dificultad respiratoria severa. La bebé fue sometida a excisión subtotal de la masa de cuyo tejido se realizó un estudio patológico donde se informó tejido cerebral heterotópico compatible con encefalocele. Por tomografía computadorizada de cabeza no se evidenció conección de la masa con la cavidad craneal, por lo que se descarta encefalocele. Falleció a los 4 meses con dificultad respiratoria. En la autopsia realizada se informó tejido cerebral heterotópico en cuello y paladar; neumonía pulmón derecho e infección por citomegalovirus. El tejido cerebral heterotópico a pesar de que se informa como benigno, en este caso se comportó como maligno por lo agresivo de su crecimiento