Preterm Infant With Respiratory Distress and Sternal Cleft

Abstract

A preterm female infant presents with respiratory distress and sternal cleft associated with a skin defect (Fig).Surfactant was given after intubation and placed on mechanical ventilation. She was noted to have superior sternal cleft associated with a skin defect on the sternal and abdomen skin (Figure). Echocardiography revealed a ventricular septal defect and atrial septal defect. Cranial and abdominal ultrasonography results were normal.The diagnosis of aplasia cutis congenita was made.Aplasia cutis congenita (ACC) is defined as the congenital absence of skin. ACC represents a heterogeneous group of disorders that may occur in isolation or in conjunction with various syndromes. There is no single underlying cause. It is usually detected at birth and most commonly affects the scalp as a solitary lesion. The type of lesion may be classified into 1 of 9 groups. The features of these groups are presented in the Table.There was sternal cleft associated with ACC in our case. These findings are consistent with group 4. This category encompasses ACC that overlies any embryologic malformations, such as sternal cleft.Diagnosis of ACC is made based on the physical appearance of the infant’s skin. No specific laboratory test is needed. Chromosome analysis may be indicated if a pattern of abnormalities suggests a genetic disorder. Elevated α-fetoprotein levels in maternal serum and amniotic fluid, as well as elevated acetylcholinesterase levels in amniotic fluid, have been reported as possible early signs of ACC. However, these tests are neither sensitive nor specific and not currently used for diagnosis.Treatment for cases of ACC is usually conservative. Local therapy includes gentle cleansing and the application of bland ointment or topical antibiotic ointment to prevent desiccation of the defect. Antibiotics are only indicated if overt signs of infection are noted. When lesions are a bit larger on the scalp, such as larger than 3 to 4 cm, treatment may be controversial. Some authors continue to recommend conservative wound care; however, others advocate for more aggressive therapy, such as surgical correction, because of the risk of complications.JoDee M. Anderson, MD, MEd, Assistant Editor, Visual Diagnosis, Video Corner; Associate Professor, Division of Neonatal-Perinatal Medicine, Department of Pediatrics, Oregon Health & Science University, Portland, OR.