L. L. Morris

Statistical analysis of the incidence of physeal injuries.

The incidence of physeal injuries in nearly 2,000 bony injuries was 18%. They were commoner in adolescents and specifically more frequent in the upper limbs. The incidence of growth arrest was just over 1%, whereas the incidence of serious complication was <1%. The prognosis depends more on the site than the Salter-Harris classification. The proximal tibia is a common site for growth disturbance.

Partial and complete trisomy 9: Delineation of a trisomy 9 syndrome

SummaryTwo infants with trisomy involving chromosome 9 are described. One had complete trisomy 9 and the other karyotype 47,XX,+der(9),t(7;9)(p22;q32)mat. A trisomy 9 syndrome is delineated, consisting of features of the trisomy 9p syndrome and various other malformations. These include abnormalities of the cardiovascular and urogenital systems, cranial suture anomalies, dislocation of the hips and knees and early death. A possible relationship of some of these findings to regions of 9q involved in cases of partial trisomy 9 is suggested.

Skeletal age estimation in leg length discrepancy.

Sixty hand radiographs of children with known leg length discrepancy were reported independently in a “blind” manner by four radiologists using the Greulich and Pyle Atlas. Significant variation was found. Fifty percent of the children were assigned a skeletal age that differed by more than 1 year between radiologists; 10% varied by more than 2 years (p < 0.05). Female skeletal age was considerably understimated by an average of 11 months. Skeletal age estimation is one source of error in the timing of surgery for leg length equalization, especially when a single estimate is used. Skeletal age also appears to be more variable in children with leg length discrepancy.

Ectopic ureter with complete ureteric duplication in the female child

Twenty-two female children with complete ureteric duplication and ectopic ureter (two bilateral) were seen in a 15-year period. The most common clinical presentation was dribbling urinary incontinence with normal micturition. Other presentations included urinary infection and vaginal discharge. Four cases were diagnosed after antenatal recognition of ureterohydronephrosis. The clinical diagnosis was supported by various radiological investigations but ultrasonography (US) proved to be particularly reliable in diagnosing ectopic ureter. The most common sites of opening of the ectopic ureter were the urethral margin or the urethrovaginal septum, although in seven cases the site was not identified. Twenty-one kidneys were managed by upper pole heminephrectomy and three by ureteropyelostomy, removing as much of the ectopic ureter as possible via the renal approach. The distal ectopic ureter was removed via a separate suprapubic incision at the initial operation in four cases, and in two cases, delayed excision of the distal ectopic ureter was necessary. All surgical specimens were examined histologically and only two heminephrectomy specimens showed features of renal dysplasia. Dribbling urinary incontinence was cured in all cases, although in one patient the entire kidney was lost after heminephrectomy. Ectopic ureter should be suspected in girls with dribbling urinary incontinence. The diagnosis is best supported by US together with conventional radiology. The majority of cases can be managed by heminephrectomy, but when adequate function is demonstrated in the upper pole, ureteropyelostomy is recommended.

Osteopathia striata with cranial sclerosis : highly variable phenotypic expression within a family

We report a four‐generation family, with five individuals affected by osteopathia striata with cranial sclerosis (OS‐CS). The family illustrates the wide spectrum of gene expression in this autosomal dominant condition. Of particular note is the unusually severe expression in the proband, who exhibits virtually all of the reported associations of the syndrome. Proximal osteolysis of the fibula and congenital urological abnormalities, in the proband, and holoprosencephaly sequence, in the probands sister, have not previously been described in the syndrome.

Distal mucocolpos and proximal hematocolpos secondary to concurrent imperforate hymen and transverse viginal septum

A 12-year-old girl had cruciate incision of imperforate hymen draining a large mucocolpos. Her symptoms did not abate thereafter, and 3 months later a transverse vaginal septum was diagnosed. This was perforated and dilated under ultrasound (US) guidance draining a large hematocolpos. Results of examination under anesthesia after 3 months was satisfactory, and she has had normal periods in follow-up for 9 months. Imperforate hymen and transverse vaginal septum are known causes of mucocolpos and hematocolpos. However, the concurrent occurrence of the two abnormalities is unique.

Primary bone tumours of the hand. Report of 21 cases.

Twenty-one primary bone tumours of the hand in children from 8 paediatric hospitals are reported. Osteochondromas and enchondromas were not included. Our material consisted of 16 patients withcommon tumours (3 Ewings sarcoma, 5 aneurysmal bone cyst, 6 osteoid osteoma and 2 epidermoid cyst) and 5 patients withuncommon tumours (osteoma, simple bone cyst, haemangiopericytoma, capillary angiomatous tumour and benign ossifying fibroma or osteoblastoma). The X-ray diagnosis of thecommon tumours should have high concordance with histology, whereas that ofuncommon tumours is much more difficult and uncertain. The characteristic features of Ewings sarcoma are stressed as all our children with this tumour had a delayed diagnosis and a fatal outcome. Differential diagnosis with other short tubular bone lesions of the hand — specifically osteomyelitis — is discussed and the possibilities of microscopic diagnosis are stressed.

Magnetic resonance imaging of growth plate injuries: the efficacy and indications for surgical procedures

Abstract In 23 patients with growth plate injuries, magnetic resonance imaging (MRI) studies were performed a total of 31 times to evaluate the physis which showed plain radiographic evidence of possible damage. Fourteen patients clinically showed growth arrest, and 10 patients required a Langenskiold operation. In 3 patients who underwent this operation, subsequent premature total fusion of the physis adversely affected the postoperative results. We propose that the merging shape of the arrest line with calcification of the provisional zone of the metaphysis shown by MRI indicates poor viability of the physis. MRI provided useful information on the appearance of the growth plate and changes in the metaphysis, both of which affected the prognosis and the results of the surgical procedures.

Gastric duplication cyst causing gastric outlet obstruction

We report a rare case of gastric outlet obstruction in a newborn infant caused by a gastric duplication cyst. Ultrasound provided a non-invasive and conclusive diagnostic technique, which should be used as a baseline investigation for cases of suspected gastric outlet obstruction.

Congenital ossifying fibroma (osteofibrous dysplasia) of the tibia―a case report

Ossifying fibromas of the long bones of the leg are benign lesions occurring in the pediatric age group identical in histological appearance to the similarly named tumor of the jaw in adults. Most frequently presentation occurs after minor trauma with symptoms of a swelling of the tibia or fibula which may be painful. Pathological fracture or limp are also occasional presentations. Congenital cases are extremely rare. We describe an otherwise normal male neonate who presented at birth with a bowed right lower leg. The limb was 1 cm shorter than the other side, with tibia vara and a firm mass situated anteriorly. X-ray showed a mixed lytic and sclerotic lesion in the proximal metaphysis of the tibia. Biopsy showed collagenous stroma containing spindle cells and irregular trabeculae of woven bone rimmed by plump osteoblasts. As the appearances were typical of an ossifying fibroma (osteofibrous dysplasia) no surgical treatment was given. The patient was well with no growth of the tumor and with radiological evidence of healing at 1 year follow up. This case is presented to draw attention to the clinicopathological features of this unusual lesion which must be considered in the differential diagnosis of congenital lesions of the tibia.