Congenital orofacial teratoma in a 22-week foetus.

No abstract available.

Cutaneous Cephalic Neurocristic Hamartoma on the Head With Melanocytic, Cartilage, Blood Vessel, Neural, and Bony Tissue.

ABSTRACT: We report on a congenital tumor of the face and scalp in a male newborn, histologically proven to contain melanocytes, cartilage, and bone, vascular, and neural tissue as part of a pigmented congenital tumor. Thus, this tumor was classified as a cutaneous cephalic neurocristic hamartoma.

A case of congenital rhabdomyosarcoma.

A 6-month-old girl presented with a congenital orbital tumor diagnosed as congenital embryonal rhabdomyosarcoma. Given the location, complete surgical resection was impossible. Management with chemotherapy and proton therapy resulted in complete clearance. This case highlights the clinical and histologic features of cutaneous congenital embryonal rhabdomyosarcoma.

Cosmetically Challenging Congenital Melanocytic Nevi.

BACKGROUND: Congenital melanocytic nevi (CMN) have a 1% to 5% lifetime risk for malignant transformation, with 50% of transformations occurring before the age of 5 years.The aim of this study is to assess the risk of melanoma development in pediatric patients with facial CMN involving the eyebrows, eyelid margins, and nasal alae where a margin of CMN was not excised to preserve these structures. METHODS: A retrospective chart review of all pediatric patients with CMN from 1986 to 2014 was performed to review demographic information, diagnosis, and number of surgeries. Patients' clinical photographs were evaluated for residual nevi after completion of the treatment. RESULTS: More than 950 medical charts of patients with CMN of the face area were reviewed. We identified 32 patients (13; 41% male) that met the study criteria with pathology-confirmed diagnosis of facial CMN with an average age of 4.4 years (3.3 months-15.8 years) at the time of initial surgery. The CMNs were classified into small (1; 3%), medium (14; 44%), large (14; 44%), and giant (3; 9%) based on their projected adult sizes. No patients developed melanoma within the small residual lesions left over the eyebrows and eyelids and inside nostrils at an average follow-up time of 5.6 (1.0-14.4) years and average age of 9.6 (1.8-19.2) years at the time of last follow-up. CONCLUSIONS: Although a lifelong risk of malignant transformation of the residual CMN cannot be concluded, our results found no transformation in follow-up visits at an average age beyond the highest risk of melanoma development in childhood. We feel that leaving residual lesions on the face in areas of important anatomic structures for better cosmetic outcome is an acceptable risk.

Management of a Large Congenital Hemangioma Obstructing Visual Axis: A Case Report and Review of Literature.

Congenital hemangiomas comprise a subset of vascular tumors with clinicopathologic features that are distinct from the more common infantile hemangioma. The authors present a patient with a large congenital hemangioma involving the forehead and brow which obstructed the visual axis and created significant risk for deprivational amblyopia. Management of the congenital hemangioma involved customized headgear to clear the visual axis and early vascular embolization of feeder vessels with the subsequent successful surgical resection at 23 days of life.A large amblyogenic congenital hemangioma required a multidisciplinary approach involving early vascular embolization of feeder vessels and subsequent surgical resection at 23 days of life.

Neonatal Teratoma: Craniofacial Treatment.

Teratomas are rare congenital neoplasms. Head and neck locations of the tumor are uncommon with combined intracranial and extracranial extensions being even more rare. The authors present a case of teratoma involving the temporal, buccal, maxillary, orbital and extending to the intracranial regions, which was successfully managed by surgical resection.

Single-Stage Reconstruction of Eyebrow Defect Using a V-Y Advancement Pedicle Flap Based on the Orbicularis Oculi Muscle.

Eyebrows play an important role in face expression and facial mimics by virtue of muscle contraction. Defects or deformity of the eyebrows result in abnormal facial expressions, and may lead to aesthetic issues for patients. The objective of this study is to report the case of a patient, with a congenital skin pigmented nevus at the right side of the eyebrow treated with direct surgical resection and followed by immediate reconstruction of the eyebrow with a V-Y advancement pedicle flap based on the orbicularis oculi muscle.

Rapidly involuting congenital hemangioma with pustules: two cases.

Rapidly involuting congenital hemangiomas (RICHs) are rare tumors that usually present as well-defined bluish or violaceous plaques or tumors with scattered telangiectasias and central or peripheral pallor. We report two previously unreported cases of RICH with associated pustules.

An alternative method for facial resurfacing: supraclavicular skin prefabrication by perforator fascia flap.

Prefabrication of supraclavicular skin provides a useful source for flaps congruent with the face skin. Among various vascular sources that have been used for this purpose, anterolateral thigh fascia seems to represent a greater value because of having a long and strong vascular pedicle and negligible donor-site morbidity. In this regard, we present a technical report on using the lateral circumflex femoral artery perforator flap harvest technique in preparing an anterolateral thigh fascia flap for the prefabrication of the supraclavicular skin. The technique proved successful in resurfacing the facial skin of a young female patient with a giant congenital melanocytic hairy nevus on the left side of her face.

Treatment of a congenital melanocytic nevus on the forehead with immediate tissue expansion technique: a three-year follow-up.

The aim of this case report was to determine the method and basic principles of immediate tissue expansion using a Foley catheter in the forehead region for treating a medium-sized congenital melanocytic nevus. A 4-year-old child presented with a supraorbitally located forehead congenital melanocytic nevus, close to the midline. Total excision and reconstruction with intraoperatively expanded forehead skin below the hairline was performed. Intraoperative tissue expansion using a Foley catheter allowed us to obtain expanded and enhanced local tissue, which had tissue characteristics similar to those of the forehead skin, and thus closure of a medium-sized defect without distorting important anatomic structures such as the brow and frontal hairline was possible. Of the two methods of tissue expansion, immediate expansion using a Foley catheter is a good alternative in single-stage reconstruction of head and neck defects because it has the advantage of omnidirectional expansion.

Frontal congenital lipoma.

Frontonasal dysplasia (FND) is a rare syndrome characterized by malformations of the central portion of the face, especially of the forehead, nose, and philtrum. FND is associated with hypertelorism, a hidden encephalocele, and a cleft of the nose. Occasionally, affected individuals also experience abnormalities of the brain and craniofacial bones. In such cases, a frontal lipoma or calcification of the falx cerebri suggest the existence of a lipoma of the corpus callosum. We present the case of a male newborn with a congenital lipoma 15 mm in size located in the medial frontal line. Magnetic resonance imaging confirmed complete agenesis of the corpus callosum. While there is controversy about the association of frontal lipoma and lipoma of the corpus callosum and FND, we believe our case supports the concept that the changes are due to the same underlying pathogenic mechanism. Therefore, we recommend imaging of the central nervous system in newborns with a congenital lipoma located in the craniofacial midline.

Pathogenesis of infantile hemangioma.

Cutaneous vascular anomalies are congenital disorders of abnormal vascular development and growth. Infantile hemangioma is a common type of vascular anomalies characterized by the abnormal growth of blood vessels in the early proliferative phase, followed by the gradual spontaneous regression of the lesion in the involuting phase. Over the past decade, significant advances have been made in our understanding of the cellular and molecular mechanisms that control the development, growth, and regression of infantile hemangioma. In this article, we present a comprehensive review of the current knowledge of the pathogenesis of hemangioma as well as promising research horizons and implications for new therapeutic advances.

[Esthetic otoplasty in subjects presenting with facial neurofibromatosis].

The results of surgical treatment of a woman presenting with facial and cervical soft-tissue neurofibromatosis and a concomitant auricular defect are presented. The otoplasty was performed by means of sectoral dissection of the auricular skeleton as described by Trendelenburg with the subsequent correction of the surrounding facial and cervical tissues. Much attention is given to the step by step procedure of correction of the auricular deformity and the surrounding facial and cervical tissues in the patients with this pathology.

Microcystic adnexal carcinoma: the first reported congenital case.

Microcystic adenexal carcinoma is a rare, locally aggressive, malignant appendage tumor also known as sclerosing sweat duct carcinoma. Since widespread recognition of microcystic adenexal carcinoma as a distinct clinicopathologic entity, approximately 300 total cases have been reported in the literature, with only eight previous cases reported in children under the age of 18, with no reported cases in patients younger than 6 years old. Our patient is unique in that the lesion was present at birth, making this the youngest case of microcystic adenexal carcinoma reported.

Long-term outcomes of surgical management in the patients with facial congenital melanocytic nevi.

BACKGROUND: The challenges of management of facial congenital melanocytic nevi (CMN) are the balance of the risk of malignant transformation, surgical management and the long-term evaluation of the functional and cosmetic outcomes. OBJECTIVE: To present information on the long-term surgical management outcomes of patients with facial CMN, which may be applicable for future clinical and surgical approaches for these lesions. MATERIAL AND METHOD: A follow-up study was performed of patients with facial CMN that were clinically evaluated by the author at Srinagarind Hospital, Khon Kaen University, between 1993 and 2011. The clinical records, photographs, surgical managements and outcomes were analyzed. RESULTS: Among the 20 patients assessed, the female-to-male ratio was 2.33 to 1 with one patient being a twin. Most (95%) had a single and ten a large CMN. The surgical treatments of these lesions included: serial excision, excision with full thickness skin grafts, excision with composite graft, excision with local and regional flaps, and excision with tissue expanders and flap. The long-term outcome revealed that no patient's condition changed or developed into melanoma. Most of the patients had an acceptable cosmetic and functional outcome. One patient had a hypertrophic scar on the face, one a mild ectropion of the lower eyelid and 2 hyperpigmentation of the skin-grafted area. CONCLUSION: The author presents a number of surgical techniques that may be used for decision-making in surgical management of each CMN. For the most part, analysis of the characteristics of the CMN, comprehensive evaluation of the anatomic composition of the defects and application of good reconstructive methods will provide acceptable long-term surgical outcomes and reduce the psychological impact to parents and patients. Early surgical removal of large CMNs is recommended and long-term follow-up until adulthood are the two essentials.

Giant congenital nevi of the scalp and forehead treated by skin expansion.

Congenital giant melanocytic nevi of the scalp and forehead are rare lesions present at birth. These lesions are associated with risk of malignant transformation, but they primarily represent a psychological problem to both patient and parents and merit early excision and reconstruction. In this study we report our own experience: seven patients, aged 8 months to 9 years, with congenital pigmented nevi involving forehead and scalp, and a 4-year old patient with congenital pigmented nevus of periorbital region and nose were treated successfully with excision and expanded skin flap reconstructions. The mean expansion procedures were 2 (range, 1 to 3), with an average of 8,8 injections for each expansion procedure (range, 6 to 11). In only one patient simultaneous expanders were placed in the scalp and forehead. Follow-up ranged from 4 months to 15 years. We had no rupture, extrusion or infection of the skin expanders. Complications included eyebrow ptosis and asymmetry in two patients underwent correction at a final procedure. In our opinion tissue expansion is an excellent technique for the treatment of giant nevi of the scalp and forehead because it offers the best aesthetic and functional outcomes.

Prenatal diagnosis of severe epignathus in a twin: case report and review of the literature.

A prenatal ultrasound diagnosis of epignathus in a dichorionic-diamniotic twin pregnancy is reported. A complex mass protruding from the fetal face was seen at week 19. Amniocentesis resulted in a 46,XX fetus with elevated alpha-fetoprotein (α-FP). An increase in tumor size and severe polyhydramnios ensued. Selective feticide performed at 22 weeks led to untreatable uterine contractions with iatrogenic abortion and early neonatal mortality of the healthy cotwin. Without development of polyhydramnios and tumor growth, weekly scan and transvaginal cervical assessment would have been carried out and cesarean section planned at around 32 weeks. Necroscopy and histology aided the ultrasound-based prenatal diagnosis.