1992, The British Journal of Radiology, 65, 268-271

Case reports

asymmetrical linear increased cortical uptake crossing a joint to involve a contiguous bone (Drane, 1987). Linear scleroderma has rarely been associated with melorheostosis (17 previously reported cases) (Wagers et al, 1972; Soffa et al, 1975; Younge et al, 1979). Systemic findings of scleroderma have not been associated with melorheostosis (Soffa et al, 1975). However, our patient did have a history of oesophageal dysmotility and Raynaud's phenomenon, two entities that have been associated with the systemic disease (Gilliland, 1987). Overall analysis of clinical presentation, radiographic findings and scintigraphic correlation are able to distinguish this rare entity from other causes of osteosclerosis.

literature. American 1096-1111.

Journal

of

Roentgenology,

87,

JANOUSEK, J. PRESTON, D. F., MARTIN, N. L. & ROBINSON,

R. G., 1976. Bone scan in melorheostosis. Journal of Nuclear Medicine, 17, 1106-1108. LEE, S. H. & SANDERSON, J., 1989. Case report: Hypophosphataemic rickets and melorheostosis. Clinical Radiology, 40, 209-211. LERI, A. & JOANNY, J., 1922. Une affection non decrite des os:

hyperostose "en coulee" sur toute la longueur d'un membre ou "melorheostose." Bulletins et Memoires de la Societe Medicale des Hopitaux de Paris, 46, 1141-1145. MORRIS, J. M.,

SAMILSON,

R. L. & CORLEY,

C. L., 1963.

Melorheostosis: review of the literature and report of an interesting case with a nineteen year follow-up. Journal of Bone and Joint Surgery, 45, 1191-1206. MULLER, S. A. & HENDERSON, E. D., 1963. Melorheostosis with

linear scleroderma. Archives of Dermatology, 88, 142-145. RESNICK, D. & NIWAYAMA, G., 1981. Enostosis, hyperostosis,

References CAMPBELL, C. J., PAPADEMETRIOU, T. & BONFIGLIO, M., 1968.

Melorheostosis: a report of the clinical, roentgenographic, and pathological findings in fourteen cases. Journal of Bone and Joint Surgery, 50 A, 1281-1304. DRANE, W. E., 1987. Detection of melorheostosis on bone scan. Clinical Nuclear Medicine, 12, 548-551. FAIRBANK, H. A. T., 1948. Melorheostosis. Journal of Bone and Joint Surgery, 30B, 533-543. GARVER, P., RESNICK, D., HAGHIGHI, P. & GUERRA, J., 1982.

Melorheostosis of the axial skeleton with associated fibrolipomatous lesions. Skeletal Radiology, 9, 41-44. GILLILAND, B. C , 1987. Progressive systemic sclerosis (diffuse scleroderma). In Harrison's Principles of Internal Medicine, ed. by E. Braunwald, K. J. Isselbacher, R. G. Petersdorf, J. D. Wilson, J. B. Martin & A. S. Fauci (McGraw-Hill, New York), pp. 1428-1432. GREEN,

A. E.,

ELSWOOD,

W. H.

& COLLINS,

J. R.,

1962.

Melorheostosis and osteopoikilosis with a review of the

and periostitis. In Diagnosis of Bone and Joint Disorders, ed. by D. Resnick & G. Niwayama (Saunders, Philadelphia), pp. 2977-2983. SOFFA, D. J., SIRE, D. J. & DODSON, J. H., 1975. Melorheostosis

with linear sclerodermatous skin changes. Radiology, 114, 577-578. WAGERS, L. T., YOUNG, A. W. & RYAN, S. F., 1972. Linear

melorheostotic scleroderma. British Journal of Dermatology, 86, 297-301. WHYTE,

M. P.,

MURPHY,

W. A.

&

SIEGEL,

B. A.,

1978.

" m Tc-pyrophosphate bone imaging in osteopoikilosis, osteopathia striata, and melorheostosis. Radiology, 127, 439-443. WYNNE-DA VIES, R.,

HALL,

C. M. & APLEY,

A. G.,

1985.

Melorheostosis. In Atlas of Skeletal Dysplasias (Churchill Livingstone, New York), pp. 508-511. YOUNGE, D., DRUMMOND, D., HERRING, J, & CRUESS, R. L.,

1979. Melorheostosis in children. Journal of Bone and Joint Surgery, 61B, 415-418.

Sonographic detection of neonatal hydrometrocolpos By Arpan K. Banerjee, MB, BS, MRCP, 0. Clarke, DCR(R), DMU and L. M. MacDonald, MB, BS, FRCR Department of Diagnostic Radiology, St Thomas' Hospital, London SE1 7EH, UK (Received 22 March 1991 and in final form 9 September 1991, accepted 20 September 1991) Keywords: Sonography, Neonate, Hydrometrocolpos

Hydrometrocolpos is the result of an obstruction of the genital tract by an intact hymen, a midplane transverse septum, cloacal anomalies or vaginal atresia. This obstruction allows an accumulation of secretions from the infant cervical mucous glands in the vagina and uterus stimulated by maternal oestrogen (Wilson et al, 1978; Nyberg, 1990). The incidence is reported to be between one in 16 000 births (Westerhout et al, 1964) and one in 30000 births 268

(Hill & Hirsch, 1985). Although several cases of hydrometrocolpos have been reported in the world literature (Mahoney & Chamberlain, 1940; Spencer & Levy, 1962; Reed & Griscom, 1973), there are very few reports of the ultrasonographic appearances of this condition (Sailer, 1979; Sawhney et al, 1990). We present two cases of hydrometrocolpos which showed classical ultrasound features and review the literature on the subject. The British Journal of Radiology, March 1992

Case reports

• .. < •. J ' ^ i f r * "

Figure 3. Ultrasound scan of pelvis (sagittal section) showing a large cystic mass posterior to the bladder. Initial ultrasound showed multiple echoes within the hydrometrocolpos. Figure 1. Ultrasound scan of pelvis (sagittal section) showing a large cystic mass with multiple internal echoes. Case reports Case 1 A 1-day-old white girl, the product of a normal pregnancy and delivery, presented with a fluctuating mass protruding from the vagina. Clinical examination revealed a palpable

abdominal mass and a swollen labia. An ultrasound of the abdomen and pelvis was performed using a 5 MHz probe. A 6 cm x 4 cm cystic left adnexal mass was demonstrated with no posterior acoustic enhancement. The mass contained several internal echoes (Fig. 1). The bladder was not distended and therefore not seen, but both kidneys were normal. The vaginal membrane was incised and 150 ml of milky white fluid was expelled. A follow-up scan of the uterus showed a normal-sized uterus 2.5 cm x 2 cm with a prominent midline endometrial echo (Fig. 2). The patient remains well and symptom free. Case 2 A baby girl, the product of a full-term normal delivery, presented at birth with a pelvic mass, having a prenatal diagnosis of a pelvic mass and bilateral hydronephroses. Post-natal ultrasound examination confirmed the bilateral hydronephroses and demonstrated that the pelvic mass was a hydrometrocolpos. Initially the dilated uterus and vagina contained fluid with echogenic material, but on subsequent scans the mucus was replaced by clear fluid (Fig. 3). Subsequent investigations revealed a common cloacal exit and the ureters inserted into the hydrometrocolpos (Fig. 4), resulting in bilateral hydronephroses. Division of the vaginal septum allowed drainage of both the bladder and uterus thus relieving the hydronephroses. Corrective surgery was achieved on this child and normal ultrasound of the kidneys, bladder and uterus was performed at a recent appointment, aged 5 years.

Figure 2. Long midline scan showing a normal uterus following incision of the vaginal membrane behind the full bladder. Vol. 65, No. 771

Discussion Hydrometrocolpos usually presents soon after birth with a lower abdominal mass and a bulging introitus. Although the condition is relatively rare, with only 79 reported cases as of 1965 (Ceballos & Hicks, 1970), it apparently represents 15% of abdominal masses in newborn girls. The ultrasonographic features of the condition include a cystic adnexal pelvic mass with a sharp posterior surface but little posterior acoustic enhancement (Sawhney et al, 1990). Internal echoes within the cystic structure are seen as a result of reflections from the mucoid debris. Occasionally internal echoes may not be 269

Case reports

Hirsch, 1985). A plain lumbar radiograph may help in the diagnosis, if a pre-sacral teratoma or meningocoele is considered a possibility. Hydrometrocolpos may be accompanied by hydronephrosis, as demonstrated in Case 2, when the abnormality of the uterus and vagina is accompanied by a congenital renal abnormality. It may also be accompanied by a small bowel obstruction and plastic peritonitis (Ceballos & Hicks, 1970). Other congenital anomalies associated with hydrometrocolpos include cardiac defects and polydactyly as part of the Kaufman-McKusick syndrome (Cartari et al, 1987). If the hydrometrocolpos is secondary to vaginal atresia and not just an imperforate hymen, there may be an associated imperforate anus or rectovaginal fistula. Other urinary abnormalities such as a persistent urogenital sinus, polycystic kidneys, renal hypoplasia and urethrovaginal fistula may also be found (Reed & Griscom, 1973; Janus & Godine, 1986). The treatment of hydrometrocolpos consists of incision of the hymen and drainage of the fluid. Ultrasound is an appropriate method of monitoring progress. Plain abdominal radiography, intravenous urography and other contrast investigations may be necessary to identify fully the complicated hydrometrocolpos. References CARTARI, A., TACCONI, M. L., BERINCARI, N., PICARAZZI, A.,

CECCOLI, D. & GAUDINO, S., 1987. Rare syndromes. The

Kaufman-McKusick syndrome. A review of 44 cases reported in the literature. Annales de Genetique, 30, 70-74. CEBALLOS, R. & HICKS, G. M., 1970. Plastic peritonitis due to

neonatal hydrometrocolpos. Radiologic and pathologic observations. Journal of Paediatric Surgery, 5, 63-70. Figure 4. Lateral abdominal radiograph following intravenous and rectal contrast. A large soft-tissue pelvic mass with contrast enhancement (due to insertion of the ureters into the hydrometrocolpos) is seen between the colon and the bladder (the catheter is a feeding tube). A thick muscular uterine wall is seen on this figure and Fig. 3.

DAVIS, G. H., WAPNER, R., KURTZ, A. B., CHHIBBER, G., FITZSIMMONDS, J. & BLOCKLINGER, A. J., 1984. Antenatal

diagnosis of hydrometrocolpos by ultrasound examination. Journal of Ultrasound in Medicine, 3, 371-374. HILL, S. J. & HIRSCH, J. H., 1985. Sonographic detection of

foetal hydrometrocolpos. Journal of Ultrasound in Medicine, 4, 323. JANUS,

seen, especially if the diagnosis is made antenatally, when the echo-free pelvic mass may be seen lying between the bladder and spine. Cephalic extension into the fetal abdomen and caudal extension into the vagina may be seen (Davis et al, 1984). The differential diagnosis of cystic abdominal masses arising from the pelvis in a female infant includes a distended bladder, pre-sacral teratoma, an anterior meningocoele, an ovarian cyst and a duplication cyst of the rectum. Ovarian cysts and duplication cysts may be unilocular or multilocular, and if multilocular this helps in differential diagnosis. As the initial ultrasound examination of hydrometrocolpos frequently displays a non-cystic mass containing echogenic material, haemorrhage into an ovarian cyst or torsion of a cyst should be considered (Nussbaum et al, 1988). Ovarian tumours and possibly a distended rectum may also produce a similar appearance (Hill & 270

C.

&

GODINE,

L.,

1986.

Newborn

with

hydrometrocolpos and ambiguous genitalia. Clinical significance. Journal of Clinical Ultrasound, 14, 739-741. MAHONEY,

P.

J.,

&

CHAMBERLAIN,

J.

W.,

1940.

Hydrometrocolpos in infancy. Congenital atresia of the vagina with abnormally abundant cervical secretions. Journal of Paediatrics, 17, 772-780. NUSSBAUM, A. R., SANDERS, R. C , HARTMAN, D. S., et al, 1988.

Neonatal ovarian cysts: sonographic-pathologic correlation. Radiology, 168, 817-821. NYBERG, D. A. 1990. Intraabdominal anomalies. In Diagnostic Ultrasound of Foetal Anomalies. Text and Atlas, ed. by D. A. Nyberg, B. S. Mahoney & D. H. Pretorius (York Book Medical Publishers, Chicago), pp. 342-395. REED, M. H. & GRISCOM, N. T., 1973. Hydrometrocolpos in

infancy. American Journal of Roentgenology, 118, 1. SAILER, J. F., 1979. Haematometra and haematocolpos. Ultrasound findings. American Journal of Roentgenology, 132, 1010-1011. SAWHNEY, S., GUPTA, R., BRERRY, M. & BHATNAGAR, V., 1990.

Hydrometrocolpos. Diagnosis and follow-up by ultrasound. A case report. Australasian Radiology, 34, 93-94. The British Journal of Radiology, March 1992

1992, The British Journal of Radiology, 65, 271-273 SPENCER, R. & LEVY, D. M., 1962. Hydrometrocolpos: report

WILSON, D. A., STACY, T. M. & SMITH, E. I., 1978. Ultrasound

of three cases and review of the literature. Annals of Surgery, 155, 558-571.

diagnosis of hydrocolpos and hydrometrocolpos. Radiology, 128, 451-454.

WESTERHOUT, F. C , HODGMAN, J. E., ANDERSON, G. V. et al,

1964. Congenital hydrocolpos. American Obstetrics & Gynecology, 89, 957-961.

Journal

of

An unusual osteochondral body in association with calcium pyrophosphate dihydrate crystal deposition disease By A. C. Jones, BSc, MRCP, *B. J. Preston, FRCR and M. Doherty, MA, MD, MRCP Rheumatology Unit, City Hospital, Nottingham NG5 1 PB and 'Department of Radiology, University Hospital, Nottingham, Clifton Boulevard, Nottingham NG7 2UH (Received 19 June 1991, accepted 23 July 1991) Keywords: Osteochondral body, Chondrocalcinosis, Knee, Pyrophosphate arthropathy

Chronic pyrophosphate arthropathy is a recognized subset of osteoarthritis (OA) characterized by deposition of calcium pyrophosphate dihydrate (CPPD) crystals (McCarty, 1976). Typically a disease of the elderly, there is symptomatic and radiographic involvement of characteristic sites, including the patellofemoral, radiocarpal and glenohumeral joints (McCarty & Haskin, 1963; Resnick & Resnick, 1983). CPPD deposition in cartilage (chondrocalcinosis) occurs particularly in the articular and the meniscal cartilages of the knee, the symphysis pubis and the triangular ligament of the wrist (McCarty & Haskin, 1963; Resnick & Resnick, 1983). It has been postulated that sporadic pyrophosphate arthropathy may represent a hypertrophic response to joint insult (Doherty et al, 1984), with CPPD crystal formation being a marker of high extracellular pyrophosphate production reflecting increased cellular biosynthetic activity (Caswell etal, 1983). Osteochondral bodies, arising in synovium by enchondral ossification of islands of chondroid metaplasia, are a common accompaniment to OA and may be particularly florid in the subset of pyrophosphate arthropathy (McCarty & Haskin, 1963; Resnick & Resnick, 1983). Such bodies form by a process of layered growth (Milgram, 1977b), and possibly reflect a generalized hyperplastic response {i.e. attempted repair) by joint tissues. We present a patient with CPPD arthropathy who developed an osteochondral body that showed features both of ossification and chondrocalcinosis.

episodes of acute synovitis. CPPD crystals, identified by compensated polarized light microscopy, were repeatedly isolated from the knee synovial fluid. The patient had also experienced episodes of diuretic-induced gout affecting the olecranon bursa and the first metatarsophalangeal, ankle and knee joints (confirmed by identification of monosodium urate crystals). Diuretics had been given for episodes of ischaemic congestive cardiac failure; all symptoms of gout resolved when they were withdrawn. There was no family history of arthropathy. On examination he was obese and had clinical signs of osteoarthritis of the knees with bony deformity and crepitus. Biochemically there was no evidence of predisposing metabolic

Case report An 83-year-old man presented with a 3 year history of symptomatic knee arthropathy associated with multiple Address correspondence to A. C. Jones, Rheumatology Unit, City Hospital, Nottingham NG5 1PB. Vol. 65, No. 771

Figure 1. Lateral radiograph of the osteochondral body demonstrating the dense, possibly trabeculated core.

271

Sonographic detection of neonatal hydrometrocolpos.

1992, The British Journal of Radiology, 65, 268-271 Case reports asymmetrical linear increased cortical uptake crossing a joint to involve a contigu...
1MB Sizes 0 Downloads 0 Views