Large diaphragmatic defect: are skeletal deformities preventable?

Abstract

The differences in some anthropometric parameters (weight, BMI, and TI) and in the skeletal deformity suggest that the CDH not only disturbs normal lung growth, but also seems to have implications on some other aspects of somatic development. Whether these changes could be related to the type of diaphragmatic reconstruction or rather to the size of the defect remains uncertain.

Self-assessed physical health among children with congenital diaphragmatic hernia

“…It is unclear to what extent the patch repair per se may contribute to chest wall deformities and scoliosis, as there is a strong correlation between severity of the malformation and size of the defect [17,37]. In present study there was a significant correlation between chest wall deformities and the severity of the malformation but in reported frequency of scoliosis the difference were only significant between Groups 1 and 2.…”

“…However, CDH is still associated with high mortality and morbidity among survivors. In follow-up studies a significant morbidity has been described, including failure to thrive, gastrointestinal disease, pulmonary morbidity, cardiovascular disease, hearing loss, neurocognitive defects [7][8][9][10][11][12][13][14], chest asymmetry, and scoliosis [15][16][17]. Surgical complications in CDH survivors are common and can occur asymptomatic many years after the repair [18,19].…”

Musculoskeletal deformities following repair of large congenital diaphragmatic hernias

“…[3][4][5][6][7][8][19][20][21][22][23][24] In children with large defects, scoliosis has been described in between 4% and 50% of children, and pectus deformity in between 14% and 80% of those undergoing patch or flap repair. Nasr et al compared chest wall deformities in patients repaired by flap and patch and found no statistical difference across treatment types.…”

“…1 Multiple studies have shown that patients who undergo CDH repair with patch or muscle flap have an increased incidence of chest wall deformities, with a scoliosis rate that ranges from 4-50% and a pectus deformity rate that ranges from 14-80%. [2][3][4][5][6][7][8] Repairing large defects with autologous tissue in the form of a split abdominal wall muscle flap is the treatment of choice at our institution, and we have previously reported a low recurrence rate associated with this repair type (4.3%). 9 The question arises as to whether this type of repair increases abdominal wall weakness or hernia and chest wall deformities.…”

Musculoskeletal abnormalities in congenital diaphragmatic hernia survivors: Patterns and risk factors: Report of a Japanese multicenter follow‐up survey

“…Recently, several studies reported musculoskeletal deformities in CDH survivors, but these studies were conducted in a small number of patients, and the frequency of deformities varied widely. Chest deformities are present in 40–50% of survivors after CDH repair, while scoliosis and pectus excavatum (PE) are noted in approximately 30% and in 20% of survivors, respectively . These deformities are usually mild and rarely have therapeutic consequences in the majority of CDH survivors, but, as more patients with severe CDH survive due to the use of improved treatment modalities, the number of patients with severe musculoskeletal deformities may increase in the future.…”