Periumbilical skin length measurements in the newborn

Abstract

Normal limits of periumbilical skin were determined in 304 newborn babies in the first 24 h. Cranial umbilical skin (SD) was 12.36 (3.23) mm and caudal umbilical skin (SD) was 8.76 (3.10) mm on the average. It is recommended that cranial umbilical skin measurement should be included in the examination of dysmorphic neonates, and those with cranial skin length above 18.82 mm (>2 SD) should be carefully evaluated for Rieger syndrome.

Excessively redundant umbilical skin as a potential early clinical feature of Morquio syndrome and FKBP14‐related Ehlers–Danlos syndrome

“…Cullen described 60 variants of the umbilicus, which he classified into nine groups through examining 200 individuals, none of whom had redundant umbilical or peri-umbilical skin (2). The umbilical skin length is usually measured using a standard metal compass while applying gentle traction to the cord (9,10). O'Marcaigh et al studied the umbilical skin length in 104 neonates and the results were further confirmed by Toppare et al who studied 304 neonates (9, 10).…”

“…On the other hand, prominent and redundant umbilical skin has been almost exclusively described in the setting of Rieger syndrome in which it represents a core feature in conjunction with hypodontia and goniodysgenesis (7,8). Based on these results, Toppare et al recommended that a newborn with cranial umbilical skin length exceeding 18.82 mm [2 SDs above the mean (12.36 mm)] be evaluated for the presence of Rieger syndrome (10). Failure of umbilical skin to involute is believed to be the defect leading to this abnormality, so it is not a variant of umbilical hernia or other ventral body wall defects (7).…”

“…Failure of umbilical skin to involute is believed to be the defect leading to this abnormality, so it is not a variant of umbilical hernia or other ventral body wall defects . O'Marcaigh et al studied the umbilical skin length in 104 neonates and the results were further confirmed by Toppare et al who studied 304 neonates . Cranial and caudal skin lengths were found to be only significantly associated with postnatal age with which they were inversely correlated.…”

“…The decrease in cranial umbilical skin length is not significant after the first 9.5 hours of birth, which could be attributed to umbilical cord drying and umbilical skin retraction. Based on these results, Toppare et al recommended that a newborn with cranial umbilical skin length exceeding 18.82 mm [2 SDs above the mean (12.36 mm)] be evaluated for the presence of Rieger syndrome . Of note, these umbilical skin length values were taken from studies on term babies, so equivalent data are lacking for preterm and post‐term babies.…”

“…Of note, these umbilical skin length values were taken from studies on term babies, so equivalent data are lacking for preterm and post‐term babies. The umbilical skin length is usually measured using a standard metal compass while applying gentle traction to the cord . In this article we expand the list of differential diagnosis of umbilical skin redundancy to include not only Rieger syndrome, but also Morquio syndrome and the variant of EDS caused by mutated FKBP14 .…”