Anomalías vasculares de presentación neonatal / Vascular anomalies in the neonatal period

Objetivos. Las anomalías vasculares de presentación neonatal suponen un reto diagnóstico por la ausencia de semiología florida, de historia evolutiva y la conveniencia de restringir pruebas diagnósticas agresivas. El objetivo es revisar las características de los casos neo- natales presentados a nuestra Unidad de Anomalías Vasculares en los últimos 5 años. Material y métodos. Se recogen todos los casos de sospecha de anomalía vascular presentados a nuestra Unidad antes de 1 mes de edad entre 2010 y 2015. Se revisa el momento del diagnóstico en relación con la anomalía, las pruebas diagnósticas y los tratamientos efectuados con su cronología. Se comparan el diagnóstico de presunción y el de certeza, cuando lo hay. Resultados. Se incluyen 26 pacientes: 15 tumores vasculares, 2 de ellos con afectación visceral (6 hemangiomas infantiles (HI), 3 NICH, 4 RICH, 1 hemangioma en penacho, 1 tumor vascular hepático no especificado. 3 malformaciones venosas: 2 con RM equívoca y una malformación venosa hiperqueratótica. 4 malformaciones linfáticas: 3 macroquísticas y una microquística. 2 lesiones muy vasculares que se diagnosticaron posteriormente (fibrosarcoma y adiponecrosis) y no eran anomalías vasculares. Solo 3 pacientes tenían diagnóstico prenatal, las malformaciones linfáticas macroquísticas. Conclusión. El diagnóstico preciso de las anomalías vasculares durante el primer mes de vida es difícil, incluso con RM. En pocos casos se necesita un tratamiento precoz, por lo que conviene dar tiempo a la evolución, al menos durante unas semanas. Los diferentes tipos de tratamiento (observación, propranolol, biopsia, láser, embolización, exéresis) dependerán de la patología a tratar. Una observación continuada puede evitar procedimientos y riesgos innecesarios (AU)

Objective. Vascular anomalies in the neonatal period are a diagnostic challenge for the lack of evident signs, symptoms and follow-up, and the convenience of restricting aggressive diagnostic tests. The aim of this work is to review the characteristics of neonatal cases presented to our Vascular Anomalies Unit in the last 5 years. Materials and methods. All cases of suspected vascular anomaly presented to our unit before 1 month of age between 2010 and 2015 were reviewed, diagnostic tests and treatments carried out with chronology were analyzed. Presumptive diagnosis and final diagnosis (when available) were compared. Results. Fifteen vascular tumors were found, 2 with visceral involvement: 6 infantile hemangiomas (IH), 3 NICH, 4 RICH, 1 tufted hemangioma, 1 unspecified liver vascular tumor, 3 venous malformations (2 equivocal MRI and a hyperkeratotic venous malformation), 4 lymphatic malformations, 3 of them macrocystic, and 2 vascular lesions that were diagnosed of fibrosarcoma and sclerema neonatorum and they were not vascular anomalies. Only 3 patients with macrocystic lymphatic malformations had prenatal diagnosis. Conclusion. Accurate diagnosis of vascular anomalies during the first month of life is difficult, even with MRI. Only in a few cases early treatment is needed, so it is worth taking time to follow-up. Different types of treatment (observation, propranolol, biopsy, laser, embolization, and resection) will depend on the condition to be treated. A continuous observation can avoid unnecessary procedures and risks (AU)

Sclerema Neonatorum in a Full-Term Infant Showing Favorable Prognosis

Sclerema neonatorum (SN) is a rare disease characterized by firm, indurated, waxy skin lesions extending throughout the body, sparing the fat-free soles, palms, and genitalia. The prognosis of SN is generally very poor, with a high fatality rate. We report the case of a full-term infant with delayed onset of SN showing good prognosis. A 4-month-old Korean male infant presented with diffuse waxy, sclerotic skin lesions on the whole body, sparing the face, nipples, and genital area, which began developing at 2 months of age. Histopathologic findings of the sclerotic skin lesions showed wide, fibrous intersecting bands in the subcutaneous fat tissue. Only sparse infiltration of lymphocytes and histiocytes was observed in the fat lobules and septa. Based on clinical presentation and histopathologic findings, he was diagnosed with SN. The patient survived with conservative care and had mild improvement of the skin lesions on his follow-up visit at 12 months of age.

Delayed-Onset Sclerema Neonatorum in a Critically Ill Premature Infant.

Sclerema neonatorum is extremely rare in the 21st century. We report a premature infant managed in a neonatal intensive care unit with delayed development of sclerema neonatorum.

Sclerema neonatorum treated with intravenous immunoglobulin: a case report and review of treatments.

Sclerema neonatorum (SN) is a rare neonatal panniculitis that typically develops in severely ill, preterm newborns within the first week of life and often is fatal. It usually occurs in preterm newborns with delivery complications such as respiratory distress or maternal complications such as eclampsia. Few clinical trials have been performed to address potential treatments. Successful treatment has been achieved via exchange transfusion (ET), but its use in neonates is declining. Similar to ET, intravenous immunoglobulin (IVIG) enhances both humoral and cellular immunity and thus may decrease mortality associated with SN. We report a case of SN in a term newborn who subsequently developed septicemia. Biopsy showed subcutaneous, needle-shaped clefts without associated necrosis, inflammation, or calcifications. Treatment with IVIG led to notable but short-term clinical improvement. Sclerema neonatorum remains a poorly understood and difficult to treat neonatal disorder. Although IVIG did not prevent our patient's death, further studies are needed to determine its clinical utility in the treatment of this rare disorder.

Panniculitis in children.

This paper will give a comprehensive view of the most frequent panniculitides seen in childhood, with emphasis on the types exclusively found in infancy, and for all other types of panniculitides also found in adults. Aim of this paper is also to analyze the clinical differences between panniculitis in childhood and in adulthood, and to give reliable histopathologic criteria for a specific diagnosis. A review of the literature is here integrated by authors' personal contribution. Panniculitides in children is a heterogeneous group of diseases, as well as in adult life, characterized by inflammation of the subcutaneous fat. Only very few types of panniculitis are exclusively found in childhood, such as Sclerema neonatorum and subcutaneous fat necrosis of the newborn, while the vast majority of the other types may be found both in paediatric age and in adults. Furthermore, this paper will consider in detail panniculitis according to their frequency, such as Erythema nodosum, Lupus panniculitis, Cold panniculitis, panniculitis in Behçet disease, and poststeroid panniculitis. It will also describe rare forms of panniculitis, such as Eosinophilic panniculitis (a pathological entity debated by many authors), Subcutaneous panniculitis T-cell lymphoma, and the different forms of the so call "Lipophagic panniculitis", encompassing respectively the febrile relapsing panniculitis of Weber-Christian disease and the non-relapsing form of Rothmann-Makai disease. For each type of panniculitis considered concise information will be given about epidemiology, etiology, clinical findings, laboratory data, prognosis and therapy, while histopathologic findings will be described in detail.

Recurrent sclerema in a young infant presenting with severe sepsis and severe pneumonia: an uncommon but extremely life-threatening condition.

A one month and twenty-five days old baby girl with problems of acute watery diarrhoea, severe dehydration, severe malnutrition, and reduced activity was admitted to the gastrointestinal unit of Dhaka Hospital of icddr,b. The differentials included dehydration, dyselectrolytaemia and severe sepsis. She was treated following the protocolized management guidelines of the hospital. However, within the next 24 hours, the patient deteriorated with additional problems of severe sepsis, severe pneumonia, hypoxaemia, ileus, and sclerema. She was transferred to the Intensive Care Unit (ICU). In the ICU, she was managed with oxygen supplementation, intravenous antibiotics, intravenous fluid, including a number of blood transfusions, vitamins, minerals, and diet. One month prior to this admission, she had been admitted to the ICU also with sclerema, septic shock, and urinary tract infection due to Escherichia coli and was discharged after full recovery. On both the occasions, she required repeated blood transfusions and aggressive antibiotic therapy in addition to appropriate fluid therapy and oxygen supplementation. She fully recovered from severe sepsis, severe malnutrition, ileus, sclerema, and pneumonia, both clinically and radiologically and was discharged two weeks after admission. Consecutive episodes of sclerema, resulting in two successive hospitalizations in a severely-malnourished young septic infant, have never been reported. However, this was managed successfully with blood transfusion, broad-spectrum antibiotics, and correction of electrolyte imbalance.

A firm plaque on the back of a newborn.

We report a case of subcutaneous fat necrosis of the newborn (SCFN), a rare disorder in term or post-term neonates. Although it is often associated with hematological abnormalities such as anemia and hypercalcemia, SCFN in this patient presented with hyperbilirubinemia. The course of SCFN is generally benign and self-limiting, though may be associated with complications secondary to hypercalcemia.

Risk factors for sclerema neonatorum in preterm neonates in Bangladesh.

BACKGROUND: This study presents a retrospective analysis of risk factors for sclerema neonatorum in preterm neonates in Bangladesh. METHODS: Preterm neonates admitted to Dhaka Shishu Hospital in Bangladesh were enrolled in a clinical trial to evaluate the effects of topical treatment with skin barrier-enhancing emollients on prevention of sepsis and mortality. Four hundred ninety-seven neonates were enrolled in the study and 51 (10.3%) developed sclerema neonatorum. We explored risk factors for sclerema neonatorum by comparing patients with and without sclerema neonatorum. Diagnosis of sclerema neonatorum was based on the presence of uniform hardening of skin and subcutaneous tissues to the extent that the skin could not be pitted nor picked up and pinched into a fold. Cultures of blood and cerebrospinal fluid were obtained in all neonates with clinical suspicion of sepsis. RESULTS: In multivariate analysis, lower maternal education (OR: 1.94; 95% CI: 1.02-3.69; P = 0.043), and signs of jaundice (OR: 2.82; 95% CI: 1.19-6.69; P = 0.018) and poor feeding (OR: 4.71; 95% CI: 1.02-21.74; P = 0.047) on admission were risk factors for developing sclerema neonatorum. The incidence rate ratio of sepsis in neonates who developed sclerema neonatorum was 1.81 (95% CI: 1.16-2.73; P = 0.004), primarily due to Gram-negative pathogens, and risk of death in infants with sclerema neonatorum was 46.5-fold higher (P < 0.001, 95% CI: 6.37-339.81) than for those without sclerema neonatorum. CONCLUSIONS: Sclerema neonatorum was a relatively common, grave condition in this setting, heralded by poor feeding, jaundice, and bacteremia, and signaling the need for prompt antibiotic treatment.

Predictors of mortality in infants with sclerema presenting to the Centre for Diarrhoeal Disease, Dhaka.

BACKGROUND: Sclerema is an uncommon, life-threatening condition, usually of newborns, with a case-fatality rate ranging from 50 to 100%. Very little is known about factors influencing outcome. AIM: To identify clinical and biochemical predictors associated with fatal outcome of sclerema in infants with diarrhoea. METHODS: Thirty infants with sclerema admitted to the Special Care Unit of the Dhaka Hospital of ICDDR,B with diarrhoea from May 2005 to end April 2006 were studied prospectively. Nine infants who died (30%) were considered to be cases while the 21 who survived constituted the comparison group. Hypothermia, severe malnutrition, septic shock, serum ammonia and CRP levels were considered to be predictors of death. Differences in proportions were compared by the chi(2) test and mean differences were compared using Student's t-test or the Mann-Whitney test, as appropriate. RESULTS: The mean age of the 30 infants was 2.1 months (range 12 d to 8 m). Fatal cases were more likely than survivors to be associated with severe underweight, a positive blood culture and higher serum ammonia and serum CRP levels. After adjusting for possible confounders in logistic regression analysis, the likelihood of death was higher in infants admitted with septic shock or who developed it soon after admission (OR 17.96, 95% CI 1.5-0220.4, p=0.024). CONCLUSIONS: Sclerema is associated with a high fatality rate and scleremic infants with diarrhoea who present with septic shock are at a greater risk of death.

Factors associated with sclerema in infants with diarrhoeal disease: a matched case-control study.

AIM: To identify clinical and biochemical factors associated with sclerema in infants with diarrhoeal illness, and their outcome. METHODS: In this case-control study, we enrolled 30 infants with clinical sepsis with sclerema (cases) and another 60, age- and sex-matched infants with clinical sepsis but without sclerema (controls) from among those admitted to the special care unit (SCU) and longer stay unit (LSU) of the Dhaka Hospital of International Centre for Diarrhoeal Disease Research, Bangladesh (ICDDR,B) for their diarrhoeal illness from May 2005 through April 2006. Sclerema as the dependant variable while hypoxia, hypothermia, C-reactive protein (CRP) level, serum total protein and prealbumin level were the major independent variables compared in the analysis. Differences in proportions were compared by the chi-square test and differences of mean were compared by Student's t-test or Mann-Whitney test, as appropriate. RESULTS: The case-fatality was significantly higher among the cases than the controls (30% vs. 2%, CI 2.9-565.5). After adjusting for confounders, infants with sclerema were more likely to be hypothermic (OR 11.6, 95% CI 1.1-126.5), and have lower serum total protein (OR 1.12, 95% CI 1.04-1.21) and prealbumin (OR 1.5, 95% CI 1.1-2.3). CONCLUSION: Diarrhoeal infants having clinical sepsis presenting with hypothermia, lower serum protein and prealbumin are prone to be associated with sclerema.

Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management.

OBJECTIVE: To review published literature on sclerema neonatorum (SN) in order to clarify its clinical presentation, histological features and management compared with two other diseases: subcutaneous fat necrosis of the newborn (SCFN) and scleredema. STUDY DESIGN: PubMed database was searched using the key words Sclerema neonatorum. A total of 55 articles from peer-reviewed journals were reviewed and summarized. RESULT: SN, SCFN and scleredema are diseases of the subcutaneous adipose tissue. SN is characterized by hardening of the skin that gets bound down to the underlying muscle and bone, hindering respiration and feeding and is associated with congenital anomalies, cyanosis, respiratory illnesses and sepsis. Histology of the skin biopsy shows thickening of the trabeculae supporting the subcutaneous adipose tissue and a sparse inflammatory infiltrate of lymphocytes, histiocytes and multinucleate giant cells. SCFN has circumscribed hardening of skin on bony prominences with necrosis of adipocytes in subcutaneous tissue and a dense granulomatous infiltrate on histology. Scleredema is characterized by hardening of the skin along with edema; histology shows inflammatory infiltrate and edema in skin and subcutaneous tissues. SN has a high case fatality rate whereas SCFN and scleredema are self-limiting and lesions resolve within a few weeks to months. Exchange transfusion may improve survival in SN. CONCLUSION: The histological features of skin biopsy should be used to establish diagnosis of SN, SCFN and scleredema as disease-specific treatment is imperative in SN due to high fatality.

Bebé colodión: manejo y proceso diagnóstico / Collodion baby: management and diagnosis

El «bebé colodión» es una situación clínica poco frecuente que se presenta en el neonato y que es compartida por varias enfermedades y síndromes. La alteración de la barrera epidérmica hace que el manejo y soporte del neonato sean fundamentales en los primeros días de vida, para luego llegar a un diagnóstico adecuado. Presentamos un nuevo caso de esta entidad en la que la colaboración del neonatólogo y el dermatólogo es fundamental

Collodion baby is a rare clinical condition affecting the newborn that is common to several diseases and syndromes. The alteration of the skin barrier makes management and care key factors in the first few days of life, after which the efforts are focused on reaching an accurate diagnosis. We present a new case of this clinical entity in which collaboration between the neonatologist and the dermatologist is essential

Sclerema neonatorum after therapeutic whole-body hypothermia.

Hypothermia is being studied as a neuroprotective therapy after asphyxia. This report is about a term newborn with severe asphyxia who underwent systemic hypothermia (34.5 degrees C) for 72 h. He survived without apparent brain damage but developed sclerema on his back, in the area in contact with the cooling mattress. The sclerema resolved without scarring after three months.

[Adiponecrosis subcutanea neonatorum in congenital pneumonia]. / Adiponecrosis subcutanea neonatorum bei konnataler Pneumonie.

In the course of a connatal pneumonia, a 7-day-old female newborn developed symmetrical subcutaneous nodules on her back, shoulders, and upper arms. These skin lesions were accompanied by hypercalcemia. Histological examination confirmed the putative clinical diagnosis of subcutaneous fat necrosis of the newborn. We discuss the differential diagnoses, therapeutic strategies, and prognosis of this uncommon disorder of the fat tissue.

A Case of Generalized Subcutaneous Fat Necrosis of Newborn / 대한피부과학회지

Subcutaneous fat necrosis of newborn is an uncommon disorder characterized by firm subcutaneous plaques and nodules usually appearing shortly after birth. A female newborn with brain death developed diffuse hardening of trunk, extremity, cheek and buttock in the first 5 weeks of the life. A clinical diagnosis of sclerema neonatorum was made. But histologic examination revealed patchy areas of fat necrosis with crystallization, infiltration of histologic cells and several multinucleated giant cells containing needle-shaped clefts. Calcium deposits were also demonstrated within the necrotic area. So, we diagnosed this case as subcutaneous fat necrosis of newborn with generalized hardening of the skin.