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Craniopagus separation requires careful planning and a multi-staged surgical approach, managed by a multidisciplinary team. Despite growing experience on surgical management, the long-term restoration of the cranial defect has been rarely discussed so far. Several alloplastic materials have been proposed as effective and safe solutions for cranioplasty even for pediatric patients, allowing for bone generation, good aesthetic results, and low complication rates. We report, for the first time, on a separated craniopagus child who underwent successful implant of a tailor-made system based on the combination of calcium phosphate ceramic reinforced with a titanium mesh.
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Procedimentos de Cirurgia Plástica , Titânio , Humanos , Criança , Crânio/cirurgia , Próteses e Implantes , Fosfatos de CálcioRESUMO
INTRODUCTION: Plexiform neurofibromas (PN) represent the main cause of morbidity in patients affected by Neurofibromatosis Type 1 (NF1). Until recently, surgery has been the main treatment option in these patients, but it is burdened with a low efficacy rate and a high incidence of side effects as well as recurrence. In recent years, MEK inhibitors (MEKi) such as selumetinib and trametinib have shown great promise. METHODS: We retrospectively describe a single center cohort of NF1 patients affected by PN1 and treated with MEKi since 2019 to 2021. Patients recruited in the study were affected by PN that were not eligible to complete surgical excision, symptomatic or with major cosmetic deformation or functional neurological deficits. RESULTS: Most patients experienced improvement in clinical symptoms and quality of life, with reduction or stabilization of lesions. However, no complete response was achieved. The most common adverse effects involved the skin, affecting every patient. Importantly, no life-threatening adverse effects occurred. CONCLUSIONS: In our experience, MEKi treatment has been shown to be both safe and effective in improving symptomatology and quality of life.
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Neurofibroma Plexiforme , Neurofibromatose 1 , Humanos , Neurofibroma Plexiforme/tratamento farmacológico , Neurofibroma Plexiforme/patologia , Neurofibroma Plexiforme/cirurgia , Estudos Retrospectivos , Qualidade de Vida , Neurofibromatose 1/tratamento farmacológico , Neurofibromatose 1/induzido quimicamente , Neurofibromatose 1/patologia , Inibidores de Proteínas Quinases/efeitos adversos , Quinases de Proteína Quinase Ativadas por Mitógeno/uso terapêuticoRESUMO
Vascular anomalies of the pediatric orbit represent a heterogeneous group that include both vascular tumors and vascular malformations. The disorder may initially be silent and then associated with symptoms and/or function damage, depending on the type of vascular anomaly and its extension. Vascular tumors include benign, locally aggressive (or borderline) and malignant forms while vascular malformations are divided into "simple", "combined" and syndromic, or "low flow" or "high flow". Both entities can arise in isolation or as part of syndromes. In this review, we describe the imaging findings of the vascular lesions of the orbit in the pediatric population, which are key to obtain a correct diagnosis and to guide the appropriate treatment in the light of the new genetic and molecular discoveries, and the role of the radiologist in their multidisciplinary management. We will also touch upon the main syndromes associated with orbital vascular abnormalities.
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The prevention of hospital-acquired pressure injuries (HAPIs) in children undergoing long-duration surgical procedures is of critical importance due to the potential for catastrophic sequelae of these generally preventable injuries for the child and their family. Long-duration surgical procedures in children have the potential to result in high rates of HAPI due to physiological factors and the difficulty or impossibility of repositioning these patients intraoperatively. We developed and implemented a multi-modal, multi-disciplinary translational HAPI prevention quality improvement program at a large European Paediatric University Teaching Hospital. The intervention comprised the establishment of wound prevention teams, modified HAPI risk assessment tools, specific education, and the use of prophylactic dressings and fluidized positioners during long-duration surgical procedures. As part of the evaluation of the effectiveness of the program in reducing intraoperative HAPI, we conducted a prospective cohort study of 200 children undergoing long-duration surgical procedures and compared their outcomes with a matched historical cohort of 200 children who had undergone similar surgery the previous year. The findings demonstrated a reduction in HAPI in the intervention cohort of 80% (p < 0.01) compared to the comparator group when controlling for age, pathology, comorbidity, and surgical duration. We believe that the findings demonstrate that it is possible to significantly decrease HAPI incidence in these highly vulnerable children by using an evidence-based, multi-modal, multidisciplinary HAPI prevention strategy.
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Úlcera por Pressão , Humanos , Criança , Úlcera por Pressão/etiologia , Úlcera por Pressão/prevenção & controle , Úlcera por Pressão/epidemiologia , Melhoria de Qualidade , Estudos Prospectivos , Doença Iatrogênica/prevenção & controle , Resultado do TratamentoRESUMO
OBJECTIVE: This study evaluates long-term outcomes in adults with Unilateral and Bilateral Cleft Lip and Palate (UCLP/BCLP) treated during the period 1992 to 1995 with tibial periosteal graft in primary repair. DESIGN: Retrospective study. SETTING: Department of Plastic and Maxillofacial Surgery, Children's Hospital Bambino Gesù (Italy). PATIENTS: The study included 52 patients with non-syndromic BCLP/UCLP who met the inclusion criteria. INTERVENTIONS: All patients underwent a standardized surgical protocol using a tibial periosteal graft as primary repair of the hard palate. MAIN OUTCOME MEASURE(S): Long-term outcomes on maxillary growth, residual oronasal fistula, and leg length discrepancy. RESULTS: About <2% of patients showed oral-nasal communication. Mean value of maxillary depth was 86° ± 4.5°. The lower value for maxillary retrusion was 76.8° in relation to the Frankfurt plane. At the x-ray control, 12.2% of patients showed leg discrepancy with a difference of always <2â cm. CONCLUSIONS: The rate of maxillary retrusion obtained was the same if compared to other techniques. Tibial periosteal graft reduces the risk of fistula and the need for reintervention after secondary bone graft. The study did not observe negative impacts on leg growth after 25 years.
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OBJECTIVE: More specific strategies are needed to support children requiring skin grafting. Our goal was to identify procedures that reduce operating times, post-operative complications, pain and length of hospital stay. Patient safety, optimal wound bed support and quick micro-debridement with locoregional anaesthesia were prioritised. Ultimately, a novel acellular fish skin graft (FSG) derived from north Atlantic cod was selected for use. METHOD: We admitted consecutive paediatric patients with various lesions requiring skin grafting for definitive wound closure. All FSGs were applied and bolstered in the operating room following debridement. RESULTS: In a cohort of 15 patients, the average age was 8 years and 9 months (4 years 1 month-13 years 5 months). Negative pressure wound therapy (NPWT) was given to 12 patients. Rapid wound healing was observed in all patients, with a wound area coverage of 100% and complete healing in 95% of wounds. Time until engraftment in patients receiving NPWT was reduced by about a half (to an average 12 days) from our standard experience of 21 days. Ten patients received locoregional anaesthesia and were discharged after day surgery. The operating time was <60 minutes, and no complications or allergic reactions were reported. Excellent pliability of the healed wound was achieved in all patients, without signs of itching and scratching in the postoperative period. This case series is the first and largest using FSG to treat paediatric patients with different wound aetiologies. We attribute the rapid transition to acute wound status and the good pliability of the new epidermal-dermal complex to the preserved molecular components of the FSG, including omega-3. CONCLUSION: FSG represents an innovative and sustainable solution for paediatric wound care that results in shorter surgery time and reduced hospital stays, with accelerated wound healing times.
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COVID-19 , Tratamento de Ferimentos com Pressão Negativa , Animais , Peixes , Humanos , Tratamento de Ferimentos com Pressão Negativa/métodos , Pandemias , Transplante de Pele/métodos , CicatrizaçãoRESUMO
A child with a maxillary Ewing sarcoma was operated for tumor asportation and reconstruction with free fibula flap. Adequate anticoagulation was achieved with lower doses of heparin and monitored with multiple ACT values. We used NIRS monitoring to avoid hypoperfusion. Post-operative pain relief was guarantited by local anestethic continous infusion.
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Vascular birthmarks are common in neonates (prevalence: 20-30%) and mostly incidental findings sometimes with spontaneous regression (salmon patch and nevus simplex). Capillary malformations are found in about 1% and infantile hemangiomas are found in 4% of mature newborns. Vascular malformations are classified according to their most prominent vessel type. The term "capillary malformation" (port wine stain) includes a wide range of vascular lesions with different characteristics; they may be isolated or part of specific syndromic conditions. Part of the infantile hemangiomas and of the vascular malformations may require treatment for functional or cosmetic reasons, and in rare cases, investigations are also necessary as they represent a clue for the diagnosis of complex vascular malformation or tumors associated with extracutaneous abnormalities. Complex vascular malformations are mostly mosaicism due to early somatic mutations. Genetic advances have led to identify the main pathogenic pathways involved in this disease group. Diffuse capillary malformation with overgrowth, Klippel-Trenaunay syndrome, CLAPO syndrome, CLOVES syndrome, and megalencephaly-capillary malformation belong to the PIK3CA-related overgrowth. Capillary malformation-arteriovenous malformation underlies a fast-flow vascular malformation, sometimes manifesting as Parkes-Weber syndrome. Recognition of these different types of capillary vascular stains is sometimes difficult; however, associated findings may orient the clinicians while genetic testing may confirm the diagnosis. Lymphatic malformation frequently manifests as large masses that compress and/or infiltrate the surrounding tissues, representing a neonatal emergency when airways are involved. Infantile hemangiomas may cause functional and/or permanent esthetical damage, depending on their localization (such as periorbital area, lip, nose); large (more than 5 cm) infantile hemangiomas with a segmental distribution can be associated with obstruction or malformations of the underneath organs with complications: PHACE syndrome, LUMBAR/SACRAL syndrome, and beard infantile hemangioma. In our review, we discuss controversies regarding the international classification and emerging concepts in the field of vascular anomalies. Finally, we discuss potential developments of new, non-invasive diagnostic techniques and repurposing of target therapies from oncology. Complex and/or life-threatening vascular tumors and malformations are extremely rare events and they represent a considerable therapeutic challenge. Early recognition of clinical signs suggestive for a specific disease may improve therapeutic outcomes and avoid severe complications.
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Mucormycosis is an opportunistic and aggressive fungal infection that mainly affects immunocompromised patients who generally suffer from diabetes mellitus, immune impairment, hematological disease. It is a life-threatening infection and the management is not standardized. The literature proposes aggressive and early surgical approach, even at the expense of mutilation. We report a case of rhino-orbital mucormycosis in a child with myeloblastic leukemia and the successful treatment using the instill negative pressure wound therapy combined with reconstructive surgery in order to reduce mortality and to avoid disfigurement. KEY WORDS: Amphotericin B, Apex syndrome, Forehead flap, Instill NPWT, Myeloaplasia Mucormycosis.
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Leucemia , Mucormicose , Anfotericina B/uso terapêutico , Antifúngicos/uso terapêutico , Criança , Humanos , Leucemia/tratamento farmacológico , Mucormicose/complicações , Mucormicose/cirurgiaRESUMO
Background and Objectives: Previous literature has disclosed that facial attractiveness affects the esthetic evaluation of nose and lip deformity on frontal and lateral photographs. However, it has never been debated if the removal of the external facial features on three-dimensional (3D) models ("cropped assessment bias") could provide a considerable usefulness in the interpretation and comparison of the results. Additionally, it has been assumed on two-dimensional (2D) studies that esthetic assessment biases with respect to observer gender, and it is not acknowledged if and to the extent that "gender assessment bias" may be influenced by a three-dimensional layout. The aim of this study is to investigate if facial traits and observers' gender may affect the esthetic ratings of unilateral cleft lip and palate (UCLP) patients after soft tissue reconstruction. Materials and Methods: Three-dimensional images of ten UCLP patients' images were acquired before the intervention (T0), one-month (T1) and six-months (T2) postoperative. Geomagic® software (version 2014; 3D Systems, Rock Hill, SC, USA) was used to remove the external facial features of 3D surface models. Five-point scale developed by Asher-McDade et al. was used to rate both nasolabial attractiveness and impairment for full-face (FF) and cropped-face (CF) 3D images. Forty-three judges (21 males, 22 females) were enrolled for the esthetic evaluation. Intraclass correlation coefficient (ICC) was used to test intra- and inter-examiner reliability; a value of 0.7 was set as the minimum acceptable level of reliability. Results: When comparing the 2 sets of observations (FF and CF), the ICC ranged from 0.654 to 0.823. Concerning gender assessment bias, the ICC ranged from 0.438 to 0.686 and from 0.722 to 0.788 for males and females, respectively. Concerning inter-examiner reliability, ICC for questions 2-7 ranged from 0.448 to 0.644 and from 0.659 to 0.817 at T0 and T2, respectively. Conclusions: The removal of external facial features provides subtle differences on the esthetic assessment of UCLP patients. Moreover, based on our data, examiners' gender differences may affect esthetic assessment of UCLP patients. Despite the subjectivity of esthetic judgments, a reliable, validated and reproducible scoring protocol should consider the influence of gender differences on 3D esthetic assessment of UCLP patients.
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Fenda Labial/cirurgia , Estética/psicologia , Reoperação/normas , Fenda Labial/psicologia , Fissura Palatina/psicologia , Fissura Palatina/cirurgia , Humanos , Imageamento Tridimensional/métodos , Período Pós-Operatório , Reoperação/métodos , Reprodutibilidade dos TestesRESUMO
PURPOSE: To report a surgical algorithm for the treatment of bilateral cleft lip stigmata. METHODS: The investigators designs a retrospective study composed of patients with bilateral cleft lip stigmata. The surgical approach is on the basis of the severity of the deformity and of course the age of the patient. It consists in Simple scar revision with orbicularis muscle synthesis; Two dermal flaps tunneled in the central vermilion; Abbe flap. The investigators analyzes early and late complications, and performs the evaluation of the cosmetic appearance by a parent or patient himself/herself, a surgeon and a blinded third party observer. RESULTS: The sample was composed of 351 patients. At short-term follow-up, viability of the flaps, function and morphology are good. At long-term follow-up, we observed significant improvement of the characteristics and profile of the patient's face and a normal function of the lips. We recorded a good scarring and a high satisfaction rate by evaluation of patients/ surgeon/ blinded third party observer. CONCLUSION: The results of this study suggest that a surgical algorithm on the basis of the severity of the deformity and of course the age of the patient can represent an option of choice for most patients with bilateral cleft lip stigmata. KEY WORDS: Abbe flap, Bilateral cleft lip stigmata, Cleft lip, Dermal flaps, Orbicularis muscles.
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Cicatriz/cirurgia , Fenda Labial/cirurgia , Cirurgia Plástica/métodos , Adolescente , Fatores Etários , Criança , Pré-Escolar , Estética , Músculos Faciais/cirurgia , Seguimentos , Humanos , Lactente , Lábio/crescimento & desenvolvimento , Satisfação do Paciente , Estudos Retrospectivos , Método Simples-Cego , Retalhos Cirúrgicos , Adulto JovemRESUMO
BACKGROUND: Infantile hemangioma is the most common vascular tumor in newborns, with an incidence from 12 to 23% among preterm infants with low weight at birth and a female to male ratio of 3:1. The head and neck is the most frequently affected area (60%), and the scalp is a typical site for such large lesions. OBJECTIVE: We describe some clinical and medical aspects in comparison with the surgical approach to giant infantile hemangioma of the scalp. METHODS: The indications to treatment are discussed. An outcome basis evaluation, by reviewing some clinical cases, is provided to help readers better understand when and how to undergo surgery safely. CONCLUSION: Early excision of huge infantile hemangioma of the scalp is the treatment of choice if feasible within 5 months of age.
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Neoplasias de Cabeça e Pescoço/cirurgia , Hemangioma Capilar/cirurgia , Síndromes Neoplásicas Hereditárias/cirurgia , Couro Cabeludo/cirurgia , Neoplasias Cutâneas/cirurgia , Feminino , Neoplasias de Cabeça e Pescoço/congênito , Hemangioma Capilar/congênito , Humanos , Lactente , Síndromes Neoplásicas Hereditárias/congênito , Neoplasias Cutâneas/congênitoRESUMO
In this article, we present a review of the literature, and we focus on 2 particular cases of cancer of the salivary glands accessory in pediatric patients The accessory parotid is the site of congenital and acquired lesions. In adults, the acquired lesions are often neoplastic and are usually similar to those seen in the main parotid gland. The disorders in children are less well defined, as only a few cases have been reported.The accessory parotid gland, or accessory parotid, is a nodule of normal salivary tissue separated from the main parotid gland, located on the masseter muscle, to which it is bound by an extension of the masseteric fascia and connected to the Stensen duct at that level. In contrast to the extensive literature on acquired lesions of the accessory parotid in adults, very few cases of malignant or benign lesions of the accessory parotid in children could be found in the literature. A review of several articles reporting lesions of the accessory parotid in adult patients, reporting 3 or more cases each for a total of 71 patients, showed 24 malignant neoplasms, 39 benign neoplasms, and 8 nonneoplastic lesions. Lesions of the accessory parotid are quite rare in children but should be considered when facing mass located in the cheek.
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Neoplasias das Glândulas Salivares/diagnóstico , Neoplasias das Glândulas Salivares/cirurgia , Glândulas Salivares Menores/patologia , Adolescente , Criança , Diagnóstico Diferencial , Diagnóstico por Imagem , Feminino , Humanos , Neoplasias das Glândulas Salivares/patologia , Retalhos CirúrgicosRESUMO
Dermatofibrosarcoma protuberans (DFSP) is a low-grade, mesenchymal, spindle cell tumor. In addition to the classical form characterized by a storiform pattern of tumor cells, pigmented (Bednar's tumor) and myxoid variants can be observed. Classical DFSP and Bednar's tumor are easily diagnosed. The myxoid variant represents a diagnostic challenge. Pigmented and myxoid variants are rare and thus far have never been reported in association in congenital DFSP. We came across a unique DFSP that was, at the same time, congenital, pigmented, and myxoid. The tumor was surgically excised with broad free margins and no recurrence. The differential diagnosis with other entities such as giant cell fibroblastoma, CD34-positive plaque-like dermal fibroma, superficial plaque-like CD34 DFSP, and neurocristic hamartoma is discussed. The recognition of this hybrid variant of congenital DFSP is important to avoid under- or overtreatment.
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Dermatofibrossarcoma/diagnóstico , Neoplasias Cutâneas/diagnóstico , Pele/patologia , Células Estromais/patologia , Biópsia , Dermatofibrossarcoma/congênito , Dermatofibrossarcoma/patologia , Diagnóstico Diferencial , Humanos , Lactente , Masculino , Neoplasias Cutâneas/congênito , Neoplasias Cutâneas/patologiaRESUMO
The potential for malignant transformation of a giant congenital melanocytic nevus (GCMN), although rare, should be considered, especially in proliferative lesions that have nodules on their surface, as they can clinically and histologically mimic melanoma and generally have a poor prognosis. We present the case of a newborn boy with a giant nevus of uneven blackish color on the back with variable degrees of intensity of pigmentation, almost entirely covered by nodules of large and variable sizes, soft texture, and a tendency to ulcerate and bleed. The unusual clinical and histologic appearance made the case particularly interesting and featured a not well-defined biologic behavior, thus deserving a radical surgical approach to fulfill diagnostic and therapeutic aims.
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Nevo Pigmentado/patologia , Índice de Gravidade de Doença , Neoplasias Cutâneas/patologia , Dorso/patologia , Epiderme/patologia , Humanos , Lactente , Recém-Nascido , Masculino , Nevo Pigmentado/cirurgia , Neoplasias Cutâneas/cirurgiaRESUMO
Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome characterized by involvement of tissues of ectodermal and mesodermal origin such as skin, eye, adipose tissue, and brain. Since 1970, when Haberland and Perou had described the first patient, 54 cases of ECCL have been reported in literature. We report on three new boys with ECCL. In addition to their typical dermal, ocular and central nervous system anomalies, one of them had a spheno-ethmoidal osseous lesion. Histopathological evaluation confirmed the benign nature of the lesion and was consistent with fibrous dysplasia. The aim of our study is to review clinical records and brain imaging studies of these three new patients with ECCL and compare these findings with those reported in literature to better define the phenotypic spectrum and radiological findings in ECCL.
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Oftalmopatias/complicações , Oftalmopatias/diagnóstico por imagem , Displasia Fibrosa Óssea/complicações , Displasia Fibrosa Óssea/diagnóstico por imagem , Lipomatose/complicações , Lipomatose/diagnóstico por imagem , Síndromes Neurocutâneas/complicações , Síndromes Neurocutâneas/diagnóstico por imagem , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Criança , Pré-Escolar , Olho/diagnóstico por imagem , Olho/patologia , Oftalmopatias/diagnóstico , Oftalmopatias/patologia , Humanos , Lipomatose/diagnóstico , Lipomatose/patologia , Imageamento por Ressonância Magnética , Masculino , Síndromes Neurocutâneas/diagnóstico , Síndromes Neurocutâneas/patologia , Fenótipo , Pele/patologia , Tomografia Computadorizada por Raios XRESUMO
Epithelioid osteosarcoma (OS) is a rare sub-type of OS with an aggressive behavior. An epithelioid OS was diagnosed in an 8-year-old female with painful swelling of the left jaw. After two courses of chemotherapy (cisplatin/methotrexate/doxorubicin), the patient presented a progressive disease. After hemimandibulectomy, 13 courses of post-operative chemotherapy (cisplatin/methotrexate/doxorubicin/ifosfamide) were performed. Histological and ultra-structural examination showed a high grade neoplasm consisting of sheets of epithelioid cells with focal osteoid formation. The patient is alive and in complete remission 42 months from diagnosis.
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Células Epitelioides/patologia , Neoplasias Maxilomandibulares/diagnóstico , Osteossarcoma/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Cisplatino/administração & dosagem , Doxorrubicina/administração & dosagem , Feminino , Humanos , Ifosfamida/administração & dosagem , Neoplasias Maxilomandibulares/tratamento farmacológico , Metotrexato/administração & dosagem , Osteossarcoma/tratamento farmacológico , Resultado do TratamentoRESUMO
Omphalocele is the most common congenital defect of the abdominal wall. Mortality rate is between 20 and 70% and early closure of the abdominal wall, within 10 days of life, is vital to the successful outcome of the surgical treatment. The authors describe the use of two bipedicled flaps of abdominal skin to correct the defect of the midline as soon as the reduction of all viscera has been accomplished.