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Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: Cervical teratomas are rare congenital neoplasms that can cause neonatal airway obstruction if large., Case Presentation: The female Persian neonate displayed respiratory distress at birth, with a 7 cm × 8 cm cystic solid mass identified on the left side of the neck. Antenatal ultrasonography revealed polyhydramnios. Despite initial stabilization, the infant required intubation and mechanical ventilation due to persistent respiratory distress. Imaging confirmed a cystic mass compressing the trachea, ruling out cystic hygroma. Surgical resection on postnatal day 17 revealed a 10 cm × 10 cm solid cystic structure, histologically identified as an immature teratoma., Conclusion: Despite risks of poor fetal and postnatal outcome from large cervical teratomas, early surgical resection after airway stabilization can result in recovery. Proper multidisciplinary management of respiratory distress from such tumors is paramount., (© 2024. The Author(s).)

Infantile myofibromatosis is a rare myofibroblastic proliferative disorder characterized by firm, skin-colored to red-purple cutaneous and subcutaneous nodules; these are the most prevalent fibrous tumors observed in infancy. A premature male infant presented at birth with multiple subcutaneous firm skin-colored nodules measuring about 1-2cm each. Full body MRI and excisional biopsy of the right chest nodule confirmed the diagnosis. We review the case of infantile myofibromatosis and discuss its highly heterogeneous presentation and clinical course, as well as histopathology, genetic testing, and approaches to management.

This Review depicts the evolving role of MRI in the diagnosis and prognostication of anomalies of the fetal body, here including head and neck, thorax, abdomen and spine. A review of the current literature on the latest developments in antenatal imaging for diagnosis and prognostication of congenital anomalies is coupled with illustrative cases in true radiological planes with viewable three-dimensional video models that show the potential of post-acquisition reconstruction protocols. We discuss the benefits and limitations of fetal MRI, from anomaly detection, to classification and prognostication, and defines the role of imaging in the decision to proceed to fetal intervention, across the breadth of included conditions. We also consider the current capabilities of ultrasound and explore how MRI and ultrasound can complement each other in the future of fetal imaging., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Background: Linear nevus sebaceous syndrome (LNSS) is a rare genetic disease characterized by large linear sebaceous nevus typically on the face, scalp, or neck. LNSS could be accompanied by multisystem disorders including the central nervous system. Herein, we report gene mutational profile via whole exome sequencing of both lesional and non-lesional skin samples in a LNSS patient., Case Presentation: A 17-year-old girl presented with multisystem abnormalities, including large skin lesions, ocular disorders, abnormal bone development and neurological symptoms. A diagnosis of LNSS was established based on clinical manifestations, histopathological and imaging findings. The skin lesions were resected and no recurrence was noted at the time of drafting this report. Whole exome sequencing of genomic DNA revealed the following 3 mutations in the lesions of the index patient: KRAS (c.35G > A, p.G12D), PRKRIR (c.A1674T, p.R558S), and RRP7A (c. C670T, p.R224W), but no mutation was found in the healthy skin and peripheral blood sample of the index patient, or in the blood samples of her parents and sibling. PCR-mediated Sanger sequencing of DNA derived from lesional skin sample of the index patient verified KRAS mutation, but not PRKRIR (c.A1674T, p.R558S) and RRP7A (c. C670T, p.R224W). None of the 3 mutations was found in Sanger sequencing in skin lesions of 60 other cases of nevus sebaceous patients., Conclusions: Our findings show the relevance of KRAS mutation to LNSS, providing new clues in understanding related genetic heterogeneity which could aid genetic counselling for LNSS patients.

A 1-week-old female infant presented with a transilluminating neck lump that increased in size with crying. The presumptive diagnosis was lymphatic malformation, but imaging raised the possibility of an abscess or necrotic tumour. A biopsy revealed a likely developmental cyst with local inflammatory change. Microlaryngoscopy revealed a fourth branchial pouch sinus, which was cauterised. The sinus and neck lump resolved without need for further treatment., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Cervical teratomas are a rare form of fetal teratoma that can grow to massive size. Generally, these masses can be surgically excised after birth with excellent physical and functional prognosis because the benign variants respect anatomical borders. The primary complications of these masses are associated with compromise of the trachea and esophagus: upper airway obstruction and polyhydramnios. We report the first documented occurrence of superior vena cava syndrome and hypoxic ischemic encephalopathy associated with a massive, right-sided cervical teratoma. This case highlights that when cervical teratomas are right-sided and sufficiently large, they can extend inferiorly and compromise central venous return to the heart. This unique presentation would likely have required fetal surgical excision to avoid catastrophic cerebral injury.

Although originally described to assist airway management of fetal congenital malformations with life-threatening airway obstruction, the indications for an ex utero intrapartum treatment (EXIT) have expanded to include surgical resection of lesions that are potentially incompatible with life in the absence of uteroplacental circulatory support. We describe the case of an infantile fibrosarcoma (IFS) that presented with fetal hydrops and was successfully managed with an emergency EXIT that necessitated the initiation of a massive fetal blood transfusion both with and without the support of uteroplacental circulation.

Introduction: Congenital head and neck pathology may cause direct postnatal airway obstruction. Prenatal diagnosis facilitates safe delivery with pre- and perinatal airway assessment and management and Ex-Utero-Intrapartum-Treatment (EXIT) if necessary. Fetoscopic airway evaluation can optimize the selection of patients in need of an EXIT procedure., Methods: Description of 11 consecutive fetuses, born with a potential airway obstruction between 1999 and 2011 and treated at the University Hospitals Leuven, with a long-term follow-up until 2018. An algorithm including fetoscopic airway evaluation is presented., Results: In utero imaging revealed seven teratomas, one fourth branchial pouch cyst, one thymopharyngeal duct remnant, one lymphatic malformation and one laryngeal atresia. A multidisciplinary team could avoid EXIT in eight patients by ultrasonographic (n = 2) or fetoscopic (n = 6) documentation of accessible airways. Three patients needed an EXIT-to-airway-procedure. Neonatal surgery included tracheostomy during EXIT (n = 2) and resection of teratoma (n = 7) or branchiogenic pathology (n = 3). All patients do well at long-term (minimum 54 months) follow-up., Conclusions: Combining prenatal imaging and perinatal fetoscopy, EXIT-procedure and neonatal surgery yields an optimal long-term outcome in these complex patients. Fetoscopy can dramatically reduce the number of EXIT-procedures., (Copyright © 2019. Published by Elsevier B.V.)

The main, but not sole, indication for an Ex-utero Intrapartum Treatment (EXIT) delivery is an airway obstruction due to either laryngeal atresia or tumors in the head and neck region. Here we present our Institution's experience with eleven cases: three teratomas, four lymphatic malformations, two laryngeal atresias and two dermoid cysts. The EXIT procedure was used to secure the fetal airway while maintaining uteroplacental gas exchange and fetal hemodynamic stability through the umbilical circulation. Five fetuses required tracheostomy. Only one fetal death occurred due to extensive growth of a teratoma preventing us from establishing an airway. No other fetal or major maternal complication occurred. The EXIT procedure is a complex procedure and these rare cases should be referred to a center with a dedicated and experienced multidisciplinary team.

Background: Large fetal head and neck (HN) masses can be life-threatening at birth and postnatally owing to airway obstruction. The two most frequent congenital masses that may obstruct the airway are lymphatic malformation (LM) and teratoma. The aim of this paper was to evaluate the results of our experience in the management of giant congenital HN masses and to conduct a literature review., Methods: The study involved a consecutive series of 13 newborns (7 females) affected by giant HN masses. Prenatal diagnosis was achieved by means of ultrasound (US) and fetal magnetic resonance imaging (MRI). Delivery was performed by means of EXIT procedure in case of radiological evidence of airway obstruction. In the postnatal period all feasible therapeutic options (surgery, sclerotherapy, medical therapy) were discussed and adopted by a multidisciplinary team. Twelve patients underwent surgery and one received Rapamycin for one month, with consequent surgical resection owing to increasing size of the mass., Results: The histopathological diagnosis was LM in 11 cases and teratoma in 2 cases. Airway obstruction was solved in 11 cases; 2 LM patients required a tracheotomy because of persistent airway obstruction. Major complications were flap necrosis (one patient) and facial nerve palsy (2 cases). Recurrence occurred in 5 patients., Conclusions: The management of congenital HN masses is always challenging and necessarily requires an interdisciplinary approach. Current therapeutic options include surgery, sclerotherapy, medical therapy or a combination of them. When they are large enough to obstruct the airway, a patient-centered approach should guide timing and modality of treatment., Level of Evidence: IV., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: The purpose of this study is to determine which MRI parameters of fetal head and neck masses predict high-morbidity neonatal outcomes, including ex utero intrapartum treatment (EXIT) procedure., Materials and Methods: This retrospective study (2004-2016) included parameters of polyhydramnios (based on largest vertical pocket), mass effect on the trachea, mass midline extension, and morphologic grade and size of masses. The morbid cohort included those requiring an EXIT procedure, difficult intubation at delivery, or lethal outcome. Predictive modeling with a multivariable logistic regression and ROC analysis was then performed., Results: Of 36 fetuses, five were delivered by EXIT procedures, there was one neonatal death within 12 hours after delivery, and another neonate required multiple intubation attempts. The remaining 29 fetuses were delivered at outside institutions with no interventions or neonatal morbidity. The largest vertical pocket and mass effect on the trachea were selected as independent predictors by the logistic regression. The cross-validated ROC AUC was 0.951 (95% CI, 0.8795-1)., Conclusion: The largest vertical pocket measurement and mass effect on the trachea were the most contributory MRI parameters that predicted significant morbidity in fetuses with masses of the face and neck, along with other significant parameters. These parameters predict significant morbid neonatal outcomes, including the need for EXIT procedures.

Cervical congenital malformations are relatively common in children. They can also be found in adults. The embryological development of the cervical region is closely related to the branchial clefts. This must be a diagnosis made by elimination; a cervical tumor must evoke the diagnosis of cancer. A cutaneous fistula or a cervical tumor, chronic or recent appearance in an inflammatory context, are the clinical signs. The thyroglossal duct cysts and the second branchial clefts cysts are the most common causes of median and lateral cervical cysts, respectively. Imaging contributes greatly to the orientation and diagnostic evaluation of the extent of the lesions. Treatment is initially based on antibiotic therapy and then on complete surgical excision, away from an infectious episode, the sole guarantee for the absence of local recurrence., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: Prompted by the limited data, we conducted this study to gather more information on dermoscopic features of CN in children, in order to optimize clinical care and management., Materials and Methods: All children with congenital nevi (CN) attending our Pediatric Pigmented Skin Lesion Unit during a 2-year period were included in the study. Clinical data were collected, and all children underwent clinical and dermoscopic examination. Dermoscopic patterns and specific features were recorded., Results: Three hundred and thirty CN were examined in a population of 276 children, aged from 6 months to 14 years. The majority (85.14%) had only one congenital naevus, and 43.12% had a family history of congenital nevi. Children with multiple congenital nevi were more likely to have a positive family history of a CN (P = 0.012). Only, in 23 children, neurological/developmental abnormalities were reported. Small CN were the commonest in our cohort (167) followed by the medium-sized (160), whereas large CN (>20 cm) were only three. Thirty-eight CN were located on the volar skin. The globular was the commonest dermoscopic pattern, followed by the reticular, whereas the parallel furrow pattern was the commonest pattern on palms and soles. CN on the trunk were more likely to be globular on the limbs, and reticular and homogeneous on the head and neck (P < 0.001). The commonest dermoscopic findings were haloed and target globules, blotches and perifollicular hypopigmentation, whereas globules and dots around cristae on volar skin. CN located on the limbs were more likely to demonstrate an atypical network (P = 0.001) and a target network with globules (P = 0.020), whereas haloed and target globules (P < 0.001), blotches (P = 0.023) and dots (P = 0.004) were found with an increased frequency in CN on the trunk., Conclusions: Given that there is much controversy on the management and accurate classification of CN, our findings may provide useful information., (© 2018 European Academy of Dermatology and Venereology.)

This case report describes a congenital ganglioneuroblastoma in a 38-day-old male Japanese Black calf. The cervical multinodular mass was present at birth and grew rapidly. The cut surface was pale gray-to-yellow and had a gelatinous appearance. Hemorrhagic cysts of various sizes were observed in the nodule. Histologically, the mass contained clusters of neuroblastic cells, ganglionic cells, and Schwann-like cells. Immunohistochemically, the ganglionic cells showed strong positivity for neuron-specific enolase, neurofilament, synaptophysin, and chromogranin A, whereas the Schwann-like cells strongly expressed S-100, glial fibrillary acidic protein, and vimentin. Ultrastructurally, neurosecretory granules resembling catecholamine were observed in the neuroblastic and Schwann-like cells. Based on the pathology, the diagnosis was congenital cervical nodular ganglioneuroblastoma.

Myoepithelial neoplasms of soft tissue represent a heterogeneous group of lesions, encompassing both benign entities as myoepithelioma (ME) and highly aggressive tumors as myoepithelial carcinoma (MEC). We describe a case of pediatric soft tissue MEC with peculiar features that may lead to misdiagnosis: congenital onset and presence of a benign component with predominant cystic structure. Few cases of congenital MEC have been reported, but the coexistence of MEC with ME is even more rare, accounting for less than 1% of myoepithelial tumors. Moreover, an extensive cystic appearance had never been described in either ME or MEC of soft tissue. Despite several predictors of poor prognosis, the patient has been showing a favorable clinical course since the administration of ICpE (ifosfamide, cisplatin, and etoposide) chemotherapy. This report provides valuable information in the differential diagnosis of cystic congenital tumors and supports a possible efficacy of adjuvant combined treatment for patients with localized disease after surgery.

Congenital melanocytic nevi (CMN) are nevi that are present at birth or arise within the first few weeks of life. They are often found on the trunk, head and neck and extremities. We report herein an unusual presentation of a CMN as a cerebriform tumour presenting as secondary cutis verticis gyrata on the scalp of an 18-year-old Filipino male., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Importance: The current standard of treatment for infantile hemangiomas (IHs) involves initial observation for regression throughout infancy and childhood, with or without medical management with β-blocker medications. Approximately 50% of the lesions respond almost completely to this regimen. However, the remaining 50% of the lesions, especially established focal IHs of the lip, nose, eyelids, forehead, cheek, and scalp, do not regress completely with this regimen or do so leaving a deformity; among these lesions, early surgical management may result in a superior aesthetic and functional outcome., Objective: To identify select focal head and neck lesions of IH that will likely not completely involute with medical management and that are ideal for a 1-stage surgical excision., Design, Setting, and Participants: In this case series, records of infants and children presenting to a tertiary care vascular anomalies center for management of IHs by the senior author were reviewed. Representative examples of focal IHs of the lips, nose, eyelids, cheek, and glabella demonstrating the tissue expansion effect were selected for presentation. Expert opinion based on more than 20 years of experience of the senior surgeon treating more than 2000 patients with focal IH and long-term clinical follow-up is also provided., Main Outcomes and Measures: Eradication of the IH while restoring aesthetic form and function to the face., Results: Five examples of patients with focal IHs of the lip, nose, eyelid, cheek, and glabella demonstrating the tissue expander effect who were successfully treated with surgery are presented. The 5 patients with these lesions ranged in age from 3 months to 5 years old, and all of them were female. One of these patients was treated with β-blockers, and another with steroids, with incomplete response to treatment prior to undergoing surgery. The tissue expander effect of a focal IH on adjacent, unaffected tissue facilitated excision of the lesion and primary closure without distortion of anatomical subunits in all 5 of these cases. Improved cosmesis with either improved or unaffected function was demonstrated., Conclusions and Relevance: Clinicians should consider early surgical intervention in infants with select focal infantile hemangiomas in lieu of prolonged observation or medical management. The psychological benefit of early removal of these disfiguring lesions has not been quantified, but is subjectively apparent to clinicians and the families of patients. Furthermore, the costs and unknown long-term sequelae of β-blocker medication, which is the current standard of treatment for IHs along with observation for regression, have not yet been quantified but will gain increasing salience in the current medical climate., Level of Evidence: 5.

Disseminated extrarenal malignant rhabdoid tumors of the head and neck are very rare, but aggressive tumors. Although the features on radiological imaging may be nonspecific, the imaging is useful for assessing the extent of tumor involvement. Key pathologic features are those of a cellular "blue cell tumor" with variable rhabdoid appearance. These cells express a combination of markers usually viewed as characteristic of diverse lines of differentiation, including EMA, cytokeratins, smooth muscle markers, and GFAP, and occasionally synaptophysin. At a molecular level, the entity is defined by mutations or alterations in the SMARB1/INI1 gene resulting in loss of INI1 expression. Diagnostic features include rhabdoid cells, expression of keratin with absence of desmin, S100 protein and CD34, and loss of INI1 expression. These features are exemplified in this sine qua non radiology-pathology correlation article.

In this review article we describe the ante- and perinatal management of fetal neck tumors. Although there are rare congenital anomalies, the clinical consequences for the fetus may be fatal and include airways obstruction, heart insufficiency, pulmonary hypoplasia and cosmetic effect. The right management allows to decrease the fetal and neonatal mortality and morbidity associated with the disease. It includes intrauterine therapy in some cases, mostly in a goitrus hypothyroidism of the fetus, but firstly, an assessment of the fetal airways patency with a subsequent, eventual Ex-Utero Intrapartum Treatment (EXIT).

Chondrolipomas are mesenchymal tumors that found as mature cartilage tissue in a fat tissue. A 2-year-old boy was seen with a complaint of a mass of the neck. On physical examination of the child, there was a one-centimeter mass above the sternocleidomastoid muscle on the lateral neck. Pathological examination of this mass after excision was reported as chondrolipoma. Chondrolipomas are seen mostly in the breast and in the adult. They are rare tumors of the head and neck area and seen mostly the in oral cavity here. This is the first case of chondrolipoma with this age and localization combination in the literature. Chondrolipoma should therefore also be considered in the differential diagnosis of congenital masses located at lateral cervical area.

Background: Infantile haemangiomas are the most common soft tissue tumours of infancy and childhood, with an incidence rate of 4-10 %. These lesions commonly present between 2 and 10 weeks after birth and characteristically involute around 8-10 years of age. Most lesions occurring in the scalp are managed conservatively, with surgery reserved for those with complications., Case Description: A 5-year-old girl presented with a swelling over the left occiput noticed since birth, with a gradual increase in size and rapid progression since the last 1 year. She had undergone six sessions of intralesional steroids over the past 3 years. However, recent MRI and CT scans showed an increase in the swelling size with full-thickness erosion of the underlying calvarium without neuroparenchymal involvement. The patient underwent surgical excision of the lesion with an excellent outcome. Our literature review revealed only a single case of haemangioma which arose from the scalp and caused significant underlying calvarial erosion and intracranial extension., Conclusion: We present a case of an atypical infantile haemangioma arising in the subgaleal plane causing full-thickness erosion of the underlying calvarium.

Background: Malignant melanoma in childhood is infrequent and can arise within congenital melanocytic nevi. Spread of malignant melanoma to the bone marrow, especially in children, is extremely rare., Methods and Results: Reported is a case of a 5-year-old boy with a congenital large melanocytic nevus of the head and neck who presented with a short history of low back and leg pain, fever and cervical lymphadenopathy. Despite regular follow-up by a dermatologist and plastic surgeon and repeatedly negative histology of previous partial excisions, diffuse bone marrow infiltration with malignant melanoma was diagnosed. The primary site was identified in the post-excision area. The disease progressed rapidly on ipilimumab immunotherapy and led to death at four months from the diagnosis., Conclusion: Surveillance is indispensable in children with a predisposition to melanoma and nonspecific symptoms such as bone pain, gait impairment or cytopenia, should always be taken into account.

A 34 year old mother with a history of polyhydraminos and premature rupture of membranes presented for an ex utero intrapartum treatment (EXIT) procedure to deliver her 34 week gestation fetus. The fetus had been diagnosed with a large cervical mass which significantly extended into the right chest. The mass compressed and deviated the airway and major neck vessels posteriorly. Imaging also revealed possible tumor involvement with the superior vena cava and right atrium. The plan was for potential extracorporeal membrane oxygenation (ECMO) during the EXIT procedure (EXIT-to-ECMO) and the potential for traditional cardiopulmonary bypass (CPB) for mediastinal tumor resection. A Modified EXIT-To-ECMO with Optional Reservoir (METEOR) circuit was devised to satisfy both therapies. A fetal airway could not be established during the EXIT procedure and so the EXIT-to-ECMO strategy was utilized. The fetus was then delivered and transferred to an adjoining operating room (OR). Traditional cardiopulmonary bypass with a cardiotomy venous reservoir (CVR) was utilized during the establishment of an airway, tumor biopsy and partial resection. The patient was eventually transitioned to our institution's standard ECMO circuit and then transferred to the intensive care unit. The patient was weaned from ECMO on day of life (DOL) eight and had a successful tumor resection on DOL 11. The patient required hospitalization for numerous interventions including cardiac surgery at 4 months of age. She was discharged to home at 5 months of age.

Purpose: Infantile haemangiomas comprise the majority of vascular anomalies and are considered the predominant vascular tumour type 1. We performed this prospective study to evaluate the therapeutic response and propranolol tolerance in infants with ulcerated infantile haemangioma of head and neck region., Methods: Sixty-four patients with ulcerated infantile haemangiomas (IHs) of head and neck region, without any prior treatment and with age older than 1 month, were included in the study, after informed consent was obtained, and were randomly divided into groups A and B. Group A patients were given oral propranolol at a dose of 2 mg/kg per day in three divided doses as outpatients. Group B patients were given oral ibuprofen at a dose of 10 mg/kg 8-hourly and paracetamol at dose of 16.2 mg/kg 8-hourly. Documentation of gender, age, haemangioma location, duration of ulceration and pain was measured on the second and fifth day after commencement of treatment in both groups using the Children's Hospital of Eastern Ontario Pain Scale., Results: There was no difference in pain score between the two groups (P value 0.074). Mean duration of healing of ulceration in group A was 17.93 ± 2.22 days and in group B was 27.71 ± 2.33 days (P value <0.001). In group A, out of 28 patients, 8 (28.5 %) were complete responders, 16 (57.1 %) were partial responders and 4 (14.2 %) were non-responders., Conclusion: Propranolol is a valuable therapeutic alternative for treatment of ulcerated haemangiomas and effectively reduces pain.

Background: Cervicofacial tumors are rarely detected by prenatal ultrasound, and prenatal counseling is very difficult as not much information is available about this problem in the literature, other than cases reports., Objectives: The aim of this study was to know if fetal echocardiography examination is helpful in those cases., Material and Methods: Forty-four fetuses with cervicofacial tumors detected in utero by ultrasonography and referred to our unit were assessed and the cardiovascular profile score (CVPS) based on echocardiography examinations was calculated. The data were analyzed by the standard statistical tests and Pearson's χ2-test (significance level P=0.05)., Results: CVPS<8 was diagnosed in 10 fetuses (23%). Structural or functional cardiovascular anomalies were present in 17 fetuses (39%). The statistically significant correlation between the CVPS<8 and the poor outcome was confirmed (P<0.05)., Conclusion: We recommend fetal echocardiography and CVPS evaluation before counseling in fetuses with cervicofacial tumors as cardiovascular dysfunction diagnosed prenatally may seriously affect survival of the fetus as well as prenatal counseling and need for cardiac treatment.

Neurocristic hamartoma (NH) is a rare dermal melanocytic lesion that is formed due to the aberrant development of neural crest-derived melanocytes during their course of migration through the dermis at the time of embryogenesis. Here, we describe a case of NH in a 6-year-old boy who clinically presented with diffuse plaque-type blue nevus on his scalp with a contiguous extension into the cervical region and lymph node involvement. A subcutaneous nodule displaying a marked histological heterogeneity with lymph node involvement is a very unusual and diagnostically challenging presentation of NH. The importance of an accurate diagnosis of NH lies in the fact that malignant transformation can rarely occur within these lesions over an unpredictable time course and remain undetected, rendering clinical management difficult. Although our child had a benign course after a follow-up of 5 years despite lymph node involvement, the possible risk of development of malignant melanoma in such a lesion warrants long-term surveillance. This case report highlights the unusual clinical presentation and histopathological features of this rare entity along with a relevant review of the literature. The present case also underscores the concept that sentinel lymph node involvement in certain melanocytic lesions in children must not be mistaken for malignant melanoma.

Head and neck teratoid cysts are the least common congenital cysts in the head and neck region, accounting for approximately 1.8% of all dermoid cysts. Teratoid cysts exhibiting mesodermal elements may be lined by gastric, intestinal, respiratory, squamous, or cilitated epithelium. We present a case of huge submandibular and neck teratoid cyst in newborn with airway obstruction and feeding difficulty. Surgical extirpation is the treatment of choice. However, before operation, some other cystic diseases in the head and neck region needs to be excluded. Fine-needle aspiration biopsy was necessary in differential diagnosis of lesions before treatment, especially that the principle of treatment of those diseases is different.

Defects of embryologic development give rise to a variety of congenital lesions arising from the epithelium and are among the most common congenital lesions of the head and neck in the pediatric population. This article presents several congenital lesions of epithelial origin, including congenital midline cervical cleft, pilomatrixoma, dermoid, foregut duplication cysts, and preauricular sinuses and pits. In addition, the management of these lesions is reviewed., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Bone involvement has been described in tumors with melanocytic differentiation such as melanotic neuroectodermal tumor of infancy, and very rarely in cellular blue nevi and neurocristic cutaneous hamartoma. We present an unusual case of facial congenital melanocytic tumor that involved the underlying bones and maxillary sinus and led to unilateral blindness. A newborn with a large red bluish patch with peripheral brown and black macules overlying marked swelling on the left side of his face was presented. The tumor was shown by magnetic resonance imaging, scintigraphy, and histopathology to invade the underlying bones and maxillary sinus and to compress the left eyeball resulting in blindness. Histopathology, immunohistochemistry, morphometric computerized microscopy, molecular genetic mutation analysis, and fluorescent in situ hybridization studies were more congruent with a melanocytic nevus. An 8.5-year follow-up was uneventful, with spontaneous partial shrinkage of the tumor.

Introduction: Head and neck teratoma is a rare entity. Its prognosis mostly depends on the risk of neonatal respiratory distress, its extension and potential malignancy. Surgical management must be as complete as possible to avoid recurrences and malignant transformation. The authors present a retrospective analysis of 6 cervicofacial teratomas and a review of the literature. The aim of the study was to analyse prenatal, neonatal and postnatal management of teratoma., Materials and Methods: Charts of children presenting with a head and neck teratoma, managed by our maxillofacial and plastic surgery unit, were analysed and antenatal, clinical, biological, radiological and pathological characteristics were collected. Surgical treatment, recurrences and surgical outcomes were analysed., Results: Six patients were included: 2 with a cervical teratoma, 2 with a facial teratoma and 2 with intraoral teratomas. In 2 cases, the lesions were diagnosed antenatally and both patients required neonatal resuscitation. All the patients underwent early surgery, and 3 with complete excisions. All patients with an initial incomplete excision eventually presented a recurrence and therefore second look surgery. No malignant transformation was noted., Conclusion: Early prenatal diagnosis is crucial to neonatal care. Early surgery and meticulous follow-up are critical in the long-term favourable outcome., (Copyright © 2014 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Unusual congenital or early-onset skin lesions on the scalp often pose a diagnostic challenge particularly as the clinical evaluation may be hampered by dense hair growth. Thus, this paper provides a concise review on developmental abnormalities and nevi with exclusive or predominant scalp localization. Aplasia cutis congenita occurs as an isolated finding, in association with genetic syndromes, nevi and anomalies or as a consequence of intrauterine trauma and teratogens. A hairless area with a narrow surrounding rim of hypertrichosis (hair collar sign) may point to occult cranial dysraphism, especially if accompanied by further suggestive signs as port-wine stains, large hemangiomas, dimples, congenital dermoid cysts, and sinuses. Many diverse entities may hide behind cutis verticis gyrata with the primary essential form being rare and representing a diagnosis of exclusion. In contrast to former belief, benign adnexal tumors arise in a nevus sebaceus considerably more often than basal cell carcinomas and other malignant epithelial tumors. Provided that tumor development is not suspected, excision of a nevus sebaceus nevus is indicated primarily for aesthetic-psychosocial reasons. However, surgical treatment is considerably easier in small children. Nevus sebaceus may be a cutaneous marker for several complex syndromes whereas nevus psiloliparus presents almost always in connection with encephalocraniocutaneous lipomatosis. Congenital melanocytic nevi of the scalp tend toward clinical regression, so that surgical intervention in large lesions should be carefully considered. In contrast, the threshold for excision of blue nevi and other conspicuous melanocytic nevi on the scalp should be low, especially since they are difficult to monitor.

Congenital hemangiomas (CH) are benign vascular neoplasms that proliferate in utero and have completed development by birth. Two subtypes of CH are recognized: rapidly involuting congenital hemangiomas (RICH) and non-involuting congenital hemangiomas (NICH). Involution of the RICH subtype often begins in the first weeks of life. NICH does not involute, allowing the distinction between RICH and NICH. We report a case of an infant with RICH occurring on the scalp, examined at birth and followed for 26 weeks.

Congenital melanoma is extraordinarily rare, and 3 types have been described: transplacental metastases from the mother, de novo congenital melanoma, and melanoma occurring in association with a congenital melanocytic nevus. We describe 2 reports of array comparative genomic hybridization analysis of de novo congenital melanoma. The first patient was male, and the second was female; both had a scalp lesion present at birth, which grew quickly. The scalp mass from patient 1 showed a heterogeneous, anaplastic melanocytic neoplasm with large size and depth, high mitotic rate, ulceration, and necrosis. The scalp mass from patient 2 showed a broad melanocytic neoplasm with single cell and junctional nested proliferation at the dermal-epidermal junction and cellular, confluent aggregates of highly atypical melanocytes in the dermis with high mitotic rate. Patient 1 had lung and liver metastases detected by radiologic imaging and was treated with cisplatin, vinblastine, and dacarbazine but expired at the age of 5 months. Patient 2 developed a metastasis to the right neck with similar histologic features, and pulmonary metastases were also detected by imaging. Patient 2 is currently alive at the age of 4 years. Array comparative genomic hybridization analysis of the first case revealed loss of chromosomes 3p26.3-p21.31, 5p15.33-q23.1, 11q15.5-q13.2, 14 (complete deletion), and 15q11.1-q22.31. The second case displayed gains in chromosomes 1q21.1-q44, 2p25.3-p11.1, 2q11.1-q37.3, 6p25.3-p11.1, 7p22.3-p11.2, 7q11.1-q36.3, 8p23.3-p11.1, 8q11.1-q24.3, 9p24.3-p11.2, 9q12-q34.3, 11q13.2-q13.4, 13q11-q34, 18p11.32-p11.21, 19p13.3-p11, 19q11-q13.43, 20p13-p11.1, and 20q11.21-q13.33. In both cases, the presence of multiple chromosomal aberrations corroborated the diagnosis of melanoma.

Background: The presence of a branchial fistula with communication both internally and externally: a 'true' branchial fistula is rare, and may arise in the context of autosomal dominant conditions such as branchiootic syndrome and branchiootorenal syndrome., Study: We discuss the case of a true branchial fistula, which recurred after initial surgical excision, in a patient with branchiootic syndrome. The residual tract was dissected in a second operation through stepladder neck incisions and removed in toto via an intraoral approach. No renal abnormalities were detected on investigation with ultrasound., Discussion: Incomplete excision of a branchial sinus is likely to cause recurrence however intraoperative visualisation of the tract can can sometimes prove challenging. An combined intraoral and external approach aids delineation and tract definition when there is a true branchial fistula and can therefore facilitate a complete excision. Suspicion of an hereditary aetiology should be raised in patients with bilateral or preauricular features, or a positive family history, which may then prompt additional renal and genetic investigation., (Copyright © 2014 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Introduction: A retrospective clinical trial was conducted to evaluate the clinical features and treatment outcomes and to determine the incidence of complications in children with congenital neck lesions (CNLs) treated at our institution with a special emphasis on thyroglossal duct remnant (TGDR), branchial cleft anomaly (BCA), and dermoid cyst (DC)., Materials and Methods: This series had 72 patients with CNL. The diagnosis of CNL was made by physical examination, ultrasound (US) in most, and for a potential extension of the mass computed tomography (CT) or magnetic resonance imaging (MRI) in a few patients and confirmed by histopathological examination in all of the children., Results: Of the patients in this series, 39 (54.2%) children had thyroglossal duct remnant (TGDR). The most common surgical procedure (n = 36) in these children was Sistrunk's procedure. Four children (10.3%) with TGDR had associated anomalies including Turner syndrome and Morgagni hernia. During the study period, 25 (34.7%) children with branchial cleft anomaly (BCA) were treated and most of these were second branchial anomalies. There were eight children (11.1%) with dermoid cyst (DC)., Conclusion: TGDR is the most common CNL and is presented clinically rather late with regard to BCA and DC in this series. Surgical resection is optimal choice of therapy in CNLs not only for aesthetic reasons but also for the recurrent infections and the potential danger of malignancy. Definitive surgery may be associated with high morbidity, especially recurrence. Associated anomalies may be observed, especially in children with TGDR. Although the Sistrunk's procedure is a safe and successful technique, life-threatening complications should also be kept in mind during the management of these lesions and early and adequate surgical treatment is suggested., (Copyright © 2014 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

We present a case of congenital malignant melanoma of the scalp in a neonate. The child was born through caesarean section with a swelling, the size of a tennis ball, on the posterior scalp. At presentation to the clinic at 25 days after birth, the swelling had significantly increased in size and ulcerated. An excision was carried out but, because of extensive haemorrhage and haemodynamic instability, the procedure was limited to subtotal resection. Later on, completion of the excision and flap coverage of the wound were performed. After an initial stable course of a few months, the child came back with local recurrence. A re-excision was planned but the child developed pneumonia resulting in sepsis leading to the demise of the child. The report adds to the literature by describing a rare entity and challenges of managing large vascular scalp lesions with complete excision and defect coverage.

Aim: Our aim is focused on the field of action of plastic surgery in the cephalic malformation and on description of the surgical indications and techniques for their correction., Material of Study: We looked at 27 patients from 2006 to 2012 with cephalic district deformities. All patients underwent surgical and rehabilitative treatments., Results: After a median follow-up from one to five years, in most cases we reached both morphological and functional reconstruction, alone or in a surgical team together with other surgical disciplines., Discussion: The correction of craniofacial malformations makes use of a surgical discipline particularly demanding, which must associate a basic surgical training with a learning techniques specific to the area and a knowledge of craniofacial malformation., Conclusions: The surgical treatment of craniofacial malformations can be seen only through a joint neurosurgical and plastic-maxillo-facial surgery, guided by knowledge of the malformation, under the close supervision of anesthetists and pediatricians. This surgery is not limited to remodel the morphology but has its bases on the recovery of the functions, maintained by rehabilitation treatment.