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Streeter's dysplasia.
Streeter's dysplasia, also known as annular constrictive rings, is a very rare abnormality that manifests itself at birth in the form of ring like constrictive bands in the upper or the lower limbs and occasionally in the trunk.[8], [11] These annular defects occur in varying degrees of severity ranging from superficial circumferential grooves in the skin to amputation of the whole or part of a limb.[3] Only a few cases of this rare disease have been found to be reported from India.[2], [3], [6], [7], [10], [11] In this communication, we are reporting four new cases of this dysplasia showing varying degrees of the severity of the disease.
Case 1 Guddo, a 5 year old child was seen in the Orthopaedic Services with the chief complaints of a constrictive ring above the left ankle joint since birth. The constriction was shallow at birth but was gradually becoming deep. The patient was the first child of his parents and was born at home, a full term normal delivery. Local examination revealed a deep constrictive ring just above the left ankle. There was no swelling, cyanosis or any sensory loss in the distal portion. X-ray revealed a deep groove just below the lower ends of the tibia and fibula. It was confined to the skin and the subcutaneous tissues [Fig. 1]. Surgical release of the band was advised but the parents took the child against medical advise. Case 2 A, 1 year old female child was born with a congenital amputation at the level of the lower part of the right leg. The stump had a fair amount of soft tissue between the bone ends and the terminal scar which covered the end of the stump and was retracted. The roentgenogram revealed amputation of both the bones of the right leg in their lower ends [Fig. 2]. The other limbs were normal. No surgical intervention was contemplated. Case 3 Chanda, a 4 year child was admitted in the hospital with the complaints of pain in the left leg of five months' duration and swelling of 4 months' duration. She also had fever to start with. The child was born of a full term normal delivery. The left foot was absent since birth. The amputation scar was normal. On local examination the left leg was found to be swollen and tender. There was amputation at the left ankle joint. X-ray showed chronic osteomyelitis of the left tibia. The fibula was normal. Rudimentary left calcaneum and talus were present [Fig. 3]. The child was treated with antibiotics and showed good improvement. Case 4 Rinkoo, a 2 year old girl, was admitted in the Pediatric Surgery services with the complaints of the presence of a constrictive ring above the left ankle joint since birth. She was born of a normal home delivery and her brothers and sisters were normal. The part distal to the constriction was swollen [Fig. 4]. The temperature of that part and sensations were normal. Skiagram revealed a constriction in the left, leg above the ankle joint. The bones however were normal. The constriction band was removed surgically and the child had an uneventful recovery.
Streeter's dysplasia, commonly known as congenital constriction bands of the extremities is a well recognised, though an uncommon condition. The incidence of congenital constriction bands of the extermities in general population is not known but judging from the number of reported cases in the literature, the condition seems to be very uncommon. Most often the experience of any worker is confined to one or two cases only. Pillay and Hesketh[5] reported the largest series of 40 cases from Singapore and concluded that the disease is more common in Malaya people of the Far East. BirchJensen[l] surveyed the Denmark population and found an incidence of 1:48500 in the upper extremities. In the lower extermities, the incidence is a little higher.[3] The usual sites for these annular grooves are the distal parts of the limbs and most of these occur in fingers, toes, forearms and the legs. Less commonly, these are found above the knees and elbows.[5] Still rarer site is the trunk. These rings may be very shallow involving the skin and subcutaneous tissues only or they may be deeper and involve the deep fascia. Very rarely, they may include the bones also.[9] All our cases were confined to the lower limbs with varying degree of severity. In two cases the groove was upto the deep fasia while in two cases, the bands were deep leading; to the amputation of the limbs. The amputation may occur after delivery or may even occur in the uterus. Streeter[8] described a 27 week old foetus which was having amputation of the right foot at delivery. The amputated part was delivered after the delivery itself. This is the only case on record in which the amputated part was recovered. These bands are regarded as congenital defects of an unknown origin.[4] Various theories put forward include pressure of the amniotic adhesions, primary constitutional defect of the germ plasm leading to faulty growth of the tissue involved and the chromosomal defects. Most of the workers including Streeter8 agree on the defective germ plasm theory. The diagnosis of the condition is mostly clinical. Radiology only helps to see the depth of the involvement and the state of the bones. According to Pillay and Hesketh5 when the limb has been amputated, the skiagram shows that the bone is also tapered off. This was present in two of our cases. Although the distal part usually does not show changes except oedema, cyanosis etc., neuropathic changes have been observed to develop distal to the lesions.[4] No surgical interference is indicated if there are no symptoms or signs of pressure over the blood vessels or the nerves. In the presence of pressure symptoms surgical release of the constriction is very essential to save the limb.
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