Congenital syphilis unusually presenting with prematurity-related severe neonatal morbidities including meconium obstruction: A case report and review of the literature.

RATIONALE: Congenital syphilis (CS) can manifest as a variety of clinical presentations according to the severity in symptomatic infants during neonatal period. Preterm neonates with CS may have more clinical evidences of infection and be more severely affected by CS compared with term ones. With increasing survival of markedly premature infants for recent decades, CS may be a challenging problem in those with severe manifestations associated with combined pathophysiologies of CS and prematurity. PATIENT CONCERNS: A very low birth weight infant at 32 weeks gestation presented with an unusual CS presentation consisting of prematurity-associated severe neonatal morbidities including meconium obstruction, prolonged cholestatic jaundice with elevated liver enzymes, and disseminated intravascular coagulation with a bleeding diathesis, in addition to common or typical manifestations of CS. DIAGNOSES: Congenital syphilis. INTERVENTIONS: Therapy consisting of a complete course of parenteral penicillin, blood component therapy, proximal ileotomy with inspissated meconium evacuation and distal loop ileostomy, and other conservative treatments. OUTCOMES: Resolution with normal gastrointestinal function and improved liver function was observed. LESSONS: This case suggests that in premature infants CS may manifest as unusual severe neonatal morbidities that may result from combination of syphilitic pathologies and contributors or conditions associated with prematurity including multisystem immaturity.

La curiosa historia sobre los ojos de David Bowie / The curious story about the eyes of David Bowie

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Syphilis.

Syphilis is an infectious disease caused by a spirochete, Treponema pallidum; it is most commonly spread by sexual transmission. Syphilis is known as the "Great Imitator," as systemic manifestations are variable. It can involve any part of the eye, with syphilitic uveitis being the most common type. Congenital syphilis is characterized by Hutchinson's teeth, saddle nose deformity, deafness, and interstitial keratitis; pigmentary changes in the retina are varied and patchy.

Signos dentales de la sífilis congénita / Dental signs of congenital syphilis

La sífilis congénita es causada por una bacteria en forma de espiroqueta denominada Treponema pallidum; se transmite de la madre infectadaaal feto. Esta enfermedad causa distintas alteraciones sistémicas, dependiendoen qué fase de la sífilis se infecta el producto. El incremento de casos a nivel mundial hace necesario el estudio de su epidemiología y que se implementen programas de salud que atiendan a la población embarazada, con seguimiento por medio del control prenatal. Se estima que 1.5 por ciento de las mujeres embarazadas a nivel mundial la padecen. Las alteraciones dentales tienen su etiología en la infl amación provocada por la espiroqueta al momento de la amelogénesis; los signos dentales característicos de la sífi lis congénita son los incisivos de Hutchinson, molares de mora y, en menor medida, los caninos de Fournier. El propósito de este trabajo es hacer una revisión de la literatura, enfatizando en las características clínicas de la enfermedad que nos permitan realizar el diagnóstico de la sífilis congénita.

Congenital syphilis is caused by a spirochete bacterium called Treponema pallidum; it is transmitted from the infected mother to the fetus. This disease causes diff erent systemic alterations, dependingon which phase of syphilis the product is infected. The increase incases worldwide makes it necessary to study its epidemiology andto implement health programs that serve the pregnant population, with follow-up through prenatal control. It is estimated that 1.5% of pregnant women world wide suff er from it. Dental alterations have theiretiology in the infl ammation caused by the spirochete at the momentof amelogenesis; the characteristic dental signs of congenital syphilisare Hutchinson incisors, blackberry molars, and, to a lesser extent, Fournier’s canines. The purpose of this paper is to review the literature, emphasizing the clinical characteristics of the disease that may allowus to diagnose congenital syphilis.

Visual Diagnosis: A 2-month-old Boy With an Unusual Rash.

Congenital syphilis should be considered in the differential diagnosis of common newborn rashes, especially if the palms and soles are involved. As soon as the diagnosis is confirmed, a lumbar puncture should be performed to rule out neurosyphilis and intravenous antibiotics started pending confirmatory testing.

Recurrent Fever, Anemia, Arthralgia, and Genu Varum as Late Manifestations of Congenital Syphilis.

We report an unusual case of recurrent fever, inflammatory knee pain, genu varum, persistent anemia, and high erythrocyte sedimentation rate in a 28-month-old boy as late manifestations of congenital syphilis (CS). Despite standard penicillin treatment at the end of the first month of life, it recurred later in life, more than once. In the first relapse, manifested by a likely gumma lesion, the prior penicillin treatment plus a negative venereal disease research laboratory result unduly led to exclusion of CS. A second treatment with penicillin led to complete clinical resolution. Although rare, bow legs, recurrent fever, anemia, and inflammatory arthralgias may be manifestations of late CS. Congenital syphilis should be considered throughout early childhood, especially if history of syphilis infection is present. A negative venereal disease research laboratory result does not exclude late syphilis, present in nearly 30% of these patients. The possibility of atypical symptoms of this "great masquerader" should always be borne in mind.

Early detection of congenital syphilis.

Late congenital syphilis is a very rare clinical entity, and its early diagnosis and treatment is essential. Dental findings often provide valuable evidence for the diagnosis of late congenital syphilis. It occurs due to the transmission of the disease from an infected mother to her fetus through placenta. This long forgotten disease continues to effect pregnant women resulting in perinatal morbidity and mortality. Congenital syphilis is a preventable disease, and its presence reflects a failure of prenatal care delivery system, as well as syphilis control programs. We are reporting a case of late congenital syphilis with only Hutchinson's teeth.

[Early congenital syphilis: a case report]. / Sífilis congénita precoz: a propósito de 2 casos clínicos.

INTRODUCTION: Congenital syphilis (CS) is a multisystemic infection of the newborn (NB) which can produce severe symptoms, and in some cases, even be fatal. In recent years, the incidence of syphilis has increased worldwide and similarly, the cases of CS in neonates have increased. OBJECTIVE: To report two cases of early and severe presentation of CS, focusing on the importance of prevention of vertical transmission and monitoring of treated mothers. The diagnostic difficulties are discussed. CLINICAL CASES: Two premature newborns that were diagnosed with probable CS present in the newborn period are presented. In the first case, due to a high index of suspicion, but without confirmatory testing, treatment was started with good clinical response. In the second case, CS was confirmed through positive serology and the specific treatment was given. CONCLUSION: CS has significant diagnostic challenges as there is no test for early confirmation, therefore, a high index of suspicion might be key in the treatment and consequent prognosis. Due to the current epidemiology of the condition, it is also important to focus on preventive measures.

Higoumenakis' sign in the diagnosis of congenital syphilis in anthropological specimens.

Higoumenakis' sign (HS) is a diagnostic criterion referring to the enlargement of the sternal end of the (right) clavicle, frequently observed in patients with late congenital syphilis. Although indexed for several years in clinical medicine textbooks, it has not been extensively applied for the diagnosis of congenital syphilis among anthropological specimens. This is highly significant, since the other major palaeopathology findings refer almost exclusively to the skull and diagnosis thus becomes difficult if only peripheral skeletal remains are available for evaluation. The potential effectiveness of the proposed use of HS as a marker of syphilis in anthropology appears adequate, since descriptions very similar to that of HS have been reported for certain findings, although no attempt has been made to correlate them with the presence of the disease. Higoumenakis himself originally observed this sign in 86% of his patients with congenital syphilis, and this report was subsequently verified by other independent researchers. His attempt to explain the pathophysiology of the sign and its localisation, on the basis of anatomical, biological, and mechanical reasons, however, has been questioned. On the other hand, the application of the remaining markers of congenital syphilis is also problematic, due to sensitivity and/or specificity limitations, and other signs may not be detected due to inability to retain soft tissue samples in anthropological populations and a lack of reliable techniques for treponematous DNA amplification in such old samples. Thus, the fact that the onset of any of the signs of syphilis is not a constant finding justifies the authors' suggestion that HS should be checked for in any available anthropological specimen, because it is highly indicative of possible infection by Treponema pallidum.

[Clinical features of 121 infants below 3 months of age with congenital syphilis].

OBJECTIVE: To investigate the clinical features and diagnosis of infants under 3 months of age with congenital syphilis. METHOD: Data of 121 infants below 3 months of age with congenital syphilis were collected from January 1997 to May 2007 at the authors' hospital. Their clinical features, laboratory findings and treatment were reviewed. RESULT: Clinical manifestations of congenital syphilis in the infants were diverse. The disease involved multiple systems such as the skin, respiratory, digestive, blood, and nervous system, skeleton and so on. Among the 121 infants, the manifestations included increased leukocyte count (89.3%), skin damage (59.5%), hepatomegaly (56.2%), splenomegaly (41.3%), anemia (38.8%), fever (28.9%), pathologic jaundice (24.0%), growth retardation after birth (12.4%) and bone damage (54.3%), etc. The rate of misdiagnosis was 17.4 percent, and the kinds of disease misdiagnosed were up to 10. CONCLUSION: Clinical manifestations of congenital syphilis in infants under 3 months of age were complicated and nonspecific, therefore doctors misdiagnosed it very easily. Clinicians should attach great importance to it. At the same time, the restoration of antemarital examination, vigorously carrying out screening for syphilis and strengthening the management of immigrants are necessary to prevent congenital syphilis.

Influence of mother VDRL titers on the outcome of newborns with congenital syphilis.

Congenital syphilis still represents a significant public health problem worldwide, and particularly in developing countries. Despite years of research on different clinical and immunological features, many physiopathological aspects still lacks of knowledge, one of them the role of immune response against Treponema pallidum by infected mothers on the birth outcomes, e.g. birthweight. In this study we analyzed if the mother VDRL titers were significantly associated with the birthweight of newborns with congenital syphilis. We observed a highly significant association between both variables, finding at the linear regression that with higher mother VDRL titers, the newborn birthweight was lower (p=0.0345). We identified that higher VDRL titers are associated with lower birth weights, although the physiopathological reasons to explain this still remains unclear.

Maternal syphilis: pathophysiology and treatment.

Despite the long history of medical interest in syphilis and its effects on pregnancy outcome, many fundamental questions about the pathophysiology and treatment of syphilis during pregnancy remain unanswered. However, understanding has been advanced by recent scientific reports such as those which delineate the complete sequence of the genome of the syphilis spirochaete, provide a more precise description of fetal and neonate infection by use of rabbit infectivity tests and describe the gestational age distribution of fetal death secondary to syphilis. It appears that fetal syphilitic involvement progresses in a rather predictable fashion, and although there is disagreement about the optimal prenatal treatment regimen, programmatic efforts to prevent fetal death must provide seropositive pregnant women with a recommended treatment early in pregnancy, and certainly before the third trimester.

Case report of an SGA infant with jaundice, direct hyperbilirubinemia, hepatosplenomegaly, thrombocytopenia, and a negative VDRL.

This case report highlights the presentation of syphilis in a small-for-gestational age (SGA) female infant who was born to a mother with no prenatal care. In the first 2 days of life, the infant developed hepatosplenomegaly, conjugated hyperbilirubinemia, thrombocytopenia, and hemolysis. After extensive diagnostic investigation, the case was solved on the infant's day of life (DOL) 12. In retrospect, the health care team had obtained a diagnostic result that could potentially have solved the case on DOL 2. Although this infant suffered no long-term sequelae secondary to the delayed diagnosis, the review of this case presentation may assist other clinicians in an early identification of a potentially lethal diagnosis.