Сombination of pectus carinatum and Scheuermann–Mau disease in children: An empirical pattern or somite syndrome?

BACKGROUND: In recent years, the number of children with pectus carinatum tended to increase. The literature describes only a few cases of a combination of keeled chest deformity with a more serious pathology of the spine, i.e., Scheuermann–Mau disease.

AIM: This study aimed to examine the frequency, clinical manifestations, and timely diagnosis of combined structural lesions of the thoracic spine in children with keeled chest deformity.

MATERIALS AND METHODS: This observational single-center cross-sectional study included patients aged 5–17 years with pectus carinatum. Categorical values were described by indicating absolute values and percentages in the sample, and quantitative indicators with normal distribution were described using arithmetic means and standard deviations and 95% confidence intervals. Quantitative indicators without normal distribution were described using the median and interquartile range.

RESULTS: Scheuermann–Mau disease was detected in 11 (9.3%) of 118 children with pectus carinatum. “Pterygoid scapulae” was noted in 97 (82.2%) children with pectus carinatum, increased cervical lordosis in 93 (79.7%), and sloping, anteriorly adducted shoulders in 99 (83.9%), which significantly hampered the clinical assessment of the extent of thoracic kyphosis. At the time of examination, a rigid thoracic kyphosis was formed in a 16-year-old boy. In younger children (5–14 years old), Scheuermann–Mau disease had no clinical manifestations and was detected only during screening X-ray examination, whereas in the older age group (15–16 years), 3 of 4 adolescents complained of back pain.

CONCLUSIONS: The frequency of Scheuermann–Mau disease in children with pectus carinatum exceeds the average prevalence in the population. In patients with keeled chest pterygoid scapulae, excess cervical lordosis, and rounded shoulders are associated with the difficulty of the assessment of the magnitude of thoracic kyphosis. Asymptomatic progression of Scheuermann–Mau disease is typical in younger children with pectus carinatum, and pain syndrome and the clinical picture of the disease develop only by the age of 15–16 years. Thus, all patients with pectus carinatum and posture disturbance should undergo a screening X-ray examination of the thoracic and lumbar spine to detect Scheuermann–Mau disease and initiate treatment in time.