Picture of the Month

SPECIAL FEATURE

SECTION EDITOR: WALTER W. TUNNESSEN, JR, MD Picture of the Month

Felix G. Riepe, MD; Dirk Eichmann, MD; Hans C. Oppermann, MD; Heniz J. Schmitt, MD; Walter W. Tunnessen, Jr, MD

15-MONTH-OLD boy had a history of un- nine teeth (Figure 2). The tympanic membranes were explained bleeding from his gums for sev- hyperemic, and evidence of middle-ear fluid was pres- eral weeks and fever for 2 days. He had ent. Palpable prominence of the costochondral junc- been fed only cow’s milk and oatmeal tions of the chest wall was noted. since age 4 months. On physical exami- The results of laboratory examinations revealed a nor- nation he had almost no spontaneous movement. His legs mal white blood cell count, a hemoglobin level of 76 g/L, A 9 were held in a “frog leg” position (Figure 1), were swol- and a platelet count of 334ϫ10 /L. The serum calcium, len along the long bones, and were tender to palpation. phosphorous, copper, and alkaline phosphatase levels were His skin was dry and pale. Hemorrhages of the gingiva normal. Thyroid stimulating hormone, triiodothyronine, were obvious as were 2 blood-filled cysts of the lower ca- and thyroxine levels were also normal. The serum level of vitamin D was normal, but vitamin C levels were low, From the Departments of Pediatrics (Drs Riepe, Eichmann, and 28 µmol/L (reference range, 45-108 µmol/L). Chest x-ray Schmitt) and Diagnostic Radiology (Dr Opperman), Faculty of film showed a scorbutic rosary at the costochondral junc- Medicine, Christian Albrechts University Kiel, Kiel, Germany; tions with a “corner” sign noted in the proximal meta- and the American Board of Pediatrics, Chapel Hill, NC physis of the humerus. Lower extremity radiographs dem- (Dr Tunnessen). onstrated abnormalities (Figure 3).

Figure 1.

Figure 3.

Figure 2.

(REPRINTED) ARCH PEDIATR ADOLESC MED/ VOL 155, MAY 2001 WWW.ARCHPEDIATRICS.COM 607

Figure 1. The infant initiated little spontaneous movement and held his legs eralized demineralization with a ground-glass appear- slightly flexed and abducted. ance to the bones. The cortex is thinned. A white line, so- Figure 2. The gingiva are spongy and bleeding with blood-filled cysts in both called Fra¨nkel sign, is apparent at the ends of metaphyses, lower canine teeth. representing widening of the provisional zones of calcifi- Figure 3. A radiograph of the right lower extremity demonstrates cation and its increased density. The epiphyseal centers osteoporosis with a “corner” sign at the distal femur and rings of ossification show central rarefaction and are also surrounded by a white around the proximal tibia epiphysis and distal femoral epiphysis. line of calcification, referred to as the Wimberger ring or halo-ossification center. Transverse bands of diminished nfantile scurvy (Moeller-Barlow disease) is rarely re- density adjacent to the Fra¨nkel sign are known as scurvy ported in developed countries except in association lines. Lateral metaphyseal spurs (corner sign) between the I with global malnutrition.1-3 The rarity of occurrence provisional zone of calcification and the cortex are the re- is responsible for the frequent delayed recognition of this sult of metaphyseal infarctions. The corner sign is more disorder. A deficiency of vitamin C (ascorbic acid) is re- pathognomonic of scurvy than the other bony findings. sponsible for the manifestations of scurvy. Vitamin C is a Irregular calcification and widening of the costochondral cofactor for numerous enzymes that are critical to several junctions results in the scorbutic rosary. Periosteal hem- body functions. The most common manifestations of scurvy orrhages cannot be detected on radiography in scurvy un- are due to the decreased production and increased fragil- til they become calcified during healing. ity of collagen, a result of faulty hydroxylation of proline and lysine in forming a precise triple-helical collagen struc- DIFFERENTIAL DIAGNOSIS, DIAGNOSIS, ture.4,5 Vitamin C also plays a number of roles in hemato- AND TREATMENT poiesis, including the promotion of iron absorption and as a cofactor for the conversion of folic acid to folinic acid. Bleeding manifestations and bone pain may suggest acute Humans cannot synthesize vitamin C and must rely on in- leukemia. Bone pain and refusal to walk may lead to con- testinal absorption from ingested nutrients. sideration of osteomyelitis, septic arthritis, and rheu- matic disorders. CLINICAL MANIFESTATIONS The diagnosis of scurvy is based on a combination of clinical and radiographic findings. A dietary history com- Most cases of scurvy are seen between ages 6 and 24 patible with insufficient intake of vitamin C should be pres- months. Neonatal scurvy is rare, except in infants of moth- 6,7 ent. Accurate laboratory measurement of vitamin C lev- ers with extreme hypovitaminosis C. Breast milk con- els is difficult because of the instability of vitamin C. tains sufficient amounts of vitamin C unless the mother’s 8 Healing occurs rapidly with the oral administration diet is deficient in this vitamin. The first clinical mani- of 100 to 200 mg/d of vitamin C. As healing occurs, the festations of scurvy are often associated with acute febrile intake of vitamin C may be reduced to 50 mg/d until com- illnesses that seem to increase the need for vitamin C. plete clinical and radiologic resolution has taken place.9 Initial manifestations of scurvy are vague and in- clude irritability, decreased appetite, and delayed devel- Accepted for publication January 14, 2000. opment. As effects of vitamin C deficiency progress, af- Reprints: Felix G. Riepe, MD, Klinikum der Christian- fected children lie still with little movement because of Albrechts Universitit, Klinik far Allgemeine Pa¨diatrie, generalized tenderness, most apparent in bones as a re- Schwanenweg 20, 24105 Kiel, Germany. sult of subperiosteal hemorrhages. Swelling may be noted along the shafts of long bones. Pseudoparalysis may be apparent as a result of the bone pain. Infants often hold REFERENCES their legs in a “frog leg” position and dislike being handled, 1. Yilmaz S, Karademir S, Ertan U, et al. Scurvy: a case report. Turk J Pediatr. 1998; often refusing to walk. 40:249-253. Gingival hemorrhages may occur along with spongy, 2. Najera-Martinez P, Rodriquez-Collado A, Gorian-Maldonado E. Scurvy: a study hemorrhagic swellings of the mucous membranes over- of 13 cases. Bol Med Hosp Infant Mex. 1992;49:280-285. laying teeth. Petechial hemorrhages may occur in the skin 3. Young LW, Schiliro G, Russo A. Radiological case of the month: scurvy: almost historic, but not quite. AJDC. 1979;133:323-324. spontaneously or, more commonly, below the site of tour- 4. Gershoff SN. Vitamin C (ascorbic acid): new roles, new requirements? Nutr Rev. niquet application, the Rumpel-Leede sign, as a mani- 1993;51:313-326. festation of capillary fragility. Swelling may be palpated 5. Halliwell B. Ascorbic acid: hype, hoax, or healer [editorial]? Am J Clin Nutr. 1997; along the costochondral junctions of the rib cage, resem- 65:1891-1892. bling the rosary seen in rickets. 6. Clow CL, Laberge C, Scriver CR. Neonatal hypertyrosinemia and evidence for deficiency of ascorbic acid in arctic and subarctic peoples. CMAJ. 1975;113:624-626. 7. Go´mez DB, Warmann S, Scholl S, Glu¨er S. Neonatal scurvy [in German]. Mon- RADIOGRAPHIC FINDINGS atsschr Kinderheilkd. 1999;147:570-572. 8. Macy IG. Composition of human colostrum and milk. AJDC. 1949;78:589-594. Changes in the long bones, particularly around the knee, 9. Moran JR, Greene HL. The B vitamins and vitamin C in human nutrition. AJDC. are most diagnostic of scurvy. The earliest finding is gen- 1979;133:308-314.

(REPRINTED) ARCH PEDIATR ADOLESC MED/ VOL 155, MAY 2001 WWW.ARCHPEDIATRICS.COM 608