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Craniosynostosis and Rickets

Inman, Page C. B.S.; Mukundan, Srinivasan Jr Ph.D., M.D.; Fuchs, Herbert E. Ph.D., M.D.; Marcus, Jeffrey R. M.D.

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Plastic and Reconstructive Surgery: April 2008 - Volume 121 - Issue 4 - p 217e-218e
doi: 10.1097/01.prs.0000305381.61117.2f
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Sir:

In the 1940s, rickets was thought to be the “most common disease of early childhood.”1 After the revelation that rickets was a disease of vitamin or mineral deficiency, it was thought to be “cured” by subsequent supplementation.1 Since the 1970s, however, the number of reported cases of rickets in children has been steadily increasing in the United States. Conceivably, decreased sun exposure, more breastfeeding, and fewer vitamin prescriptions written for infants have led to its resurgence, or at least to a re-emergence of this condition, once thought to be cured.

Rickets results from a deficiency in calcium, phosphate, vitamin D, alkaline phosphatase, or inhibitors of mineralization, such as aluminum or biphosphonates. Without these minerals, hypomineralization of the bone occurs, and disorganized chondrocyte proliferation occurs at the growth plate.2 This impairs the strength of these bones and bowing occurs. As a result, affected children often present with parietal and frontal bossing, craniotabes, the “rachitic rosary” (beading along the costochondral junction), and bowing of the distal radius, ulna, femur, or tibia.

Craniosynostosis in conjunction with rickets has rarely been reported in the United States as a result of vitamin D–resistant rickets, X-linked hypophosphataemic rickets, toxic amounts of vitamin D, long-term antacid use in an infant, or presumed genetic factors.

In 2004, a 2-year-old African-American boy with a turribrachycephalic head was brought to the attention of the Duke University Cleft and Craniofacial Program. A head computed tomography scan revealed nonsyndromic bilateral coronal and metopic craniosynostosis with calvarial thumbprinting, suggesting the presence of elevated intracranial pressure (Fig. 1).

Fig. 1.
Fig. 1.:
Preoperative oblique three-dimensional computed tomography scan.

To correct the elevated intracranial pressure, the patient underwent reconstruction of the anterior two-thirds of the cranial vault. The procedure was uneventful, and the desired degree of correction was obtained (Fig. 2). Normal craniofacial development was documented over the ensuing 14 months.

Fig. 2.
Fig. 2.:
Postoperative three-dimensional computed tomography scan of the two-thirds cranial vault reconstruction.

Currently, the cause of craniosynostosis is unknown, but rickets is a preventable disease. A recent study reports that out of 166 cases of rickets in children, 83 percent were in African-American children. Furthermore, 96 percent of those patients were breast-fed.3 Of note, African-American adults require six times more sun exposure than Caucasian adults to generate adequate vitamin D levels, so it only follows that African-American children would require more sunlight as well.1 Yet another study has found that the incidence of premature infants who developed rickets was increased in mothers who breast-fed (40 percent), as opposed to the incidence of rickets occurring in the children who were bottle-fed (16 percent).4

In the United States, craniosynostosis secondary to rickets is rarely reported; however, there is a documented association between the two entities. Craniosynostosis should be included in the differential diagnosis of any rickettic child with increasing head circumference. Moreover, it is important to pay attention to the vitamin D intake of children, especially those with darker skin coloration, those with limited sun exposure, and those who have been breast-fed for an extended period of time without vitamin D supplementation.

Page C. Inman, B.S.

Srinivasan Mukundan, Jr., Ph.D., M.D.

Herbert E. Fuchs, Ph.D., M.D.

Jeffrey R. Marcus, M.D.

Division of Plastic and Reconstructive Surgery

Department of Radiology

Division of Neurosurgery

Cleft and Craniofacial Program at Duke

Duke University Medical Center

Durham, N.C.

DISCLOSURE

The authors have no financial interest linked to the publication of this communication. The skull images in this report were produced by a helical craniofacial MDCT on a 16-slice scanner (GE Healthcare, Waukesha, Wis.) using the standard pediatric craniofacial protocol: 2.5-mm slice thickness; pitch, 0.875:1; 140 kVp; and 170 mAs. The images were reconstructed from projection data at 1.25-mm increments using a standard reconstruction kernel. Virtual morphometry was performed off-line on a Vitrea workstation (Vital Images, Minnetonka, Minn.). Images were interpreted by the neuroradiology department and reviewed with plastic surgeons and neurosurgeons.

REFERENCES

1. Harrison, H. E. A tribute to the first lady of public health (Martha M. Eliot): V. The disappearance of rickets. Am. J. Public Health Nations Health 56: 734, 1966.
2. Lacey, D. L., and Huffer, W. E. Studies on the pathogenesis of avian rickets: I. Changes in epiphyseal and metaphyseal vessels in hypocalcemic and hypophosphatemic rickets. Am. J. Pathol. 109: 288, 1982.
3. Weisberg, P., Scanlon, K. S., Li, R., et al. Nutritional rickets among children in the United States: Review of cases reported between 1986 and 2003. Am. J. Clin. Nutr. 80 (Suppl.): 1697S, 2004.
4. Takada, M., Shimada, M., Hosono, S., et al. Trace elements and mineral requirements for very low birth weight infants in rickets of prematurity. Early Hum. Dev. 29: 333, 1992.

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