Slotted acetabular augmentation in childhood and adolescence.

During an 18-year period, 157 hips in 140 patients with hip dysplasia underwent a slotted acetabular augmentation (SAA or shelf) procedure. Indication for the procedure was pain in 71, progressive subluxation in 52, and acetabular insufficiency in 34 hips. Follow-up of 24-168 months (mean 60 months) was obtained in 108 hips in 98 patients. Good or excellent results were recorded in 83% of the hips. Unsatisfactory results were usually due to excessive breadth or insufficient thickness of the augmentation. The SAA is an alternative to the Chiari osteotomy when lateralization of the hip is not excessive.     

Meniscus injuries in childhood and adolescence. Causes--treatment--results

A brief outline of special morphological features of meniscus structure during growth is followed by an analysis of meniscus lesions during this age period. Between 1964 and 1981, we performed altogether 3,579 meniscectomies in our clinic; at that time, only 19 patients (0.5%) were between 3 and 12 years old, 223 cases (6.2%) between 13 and 18 years. Whereas dysplastic changes of menisci were the main causes for meniscectomy in childhood, traumatic lesions of the menisci were prominent in adolescence. A causal relationship to regular activity in sports is assumed. 188 cases (77.7%) could be controlled clinically after an average postoperative period of 8.5 and 10.0 years, respectively. The subjective results were altogether satisfactory. In 11 cases, x-ray examination showed arthrotic changes of the knee joint.     

Chronic pancreatitis beginning in childhood and adolescence.

We identified 10 patients with symptoms beginning before the age of 20 years in a group of 69 patients with proven chronic pancreatitis. Six of the 10 patients were women. There was a family history of pancreatitis in three patients and alcohol exposure in seven patients. Three patients were dependent on narcotics at the time of presentation. Six of the 10 patients had pancreatic duct dilatation to 10 mm or more in diameter during observation. These six patients underwent pancreaticojejunostomy, with clinical improvement in five patients. The median time of follow-up was 19 years from presentation. No patient developed diabetes and one developed malabsorption. Only three patients were free of pain, but four other patients had only mild episodes that rarely required hospital admission. One patient died of metastatic abdominal carcinoma of unknown origin 51 years after developing familial pancreatitis. Chronic pancreatitis beginning at a young age is sufficiently common to merit special awareness. It is compatible with prolonged survival, and pancreaticojejunostomy may help if the pancreatic duct reaches sufficient size. The disease does not seem to burn out with time.     

Leiomyosarcoma in childhood and adolescence

Background: Few series of leiomyosarcoma in patients <21 years of age have been reported. We reviewed our institutional experience with this neoplasm to learn disease characteristics, patterns of relapse, and outcome. Methods: The records of 21 patients with leiomyosarcoma admitted to our institution were reviewed retrospectively; 18 of these were diagnosed after 1970. Overall survival was estimated using the Kaplan-Meier method. Results: Ninety-five percent (20 of 21) were initially treated with a wide local excision that was complete with a negative microscopic margin in 10 (48%). There also was a strong correlation between grade and surgical margins. High-grade tumors were associated with a lower rate of complete resection. The majority underwent additional therapy. Radiation was used to treat both initial and recurrent disease in nine patients, with four of these undergoing brachytherapy. Thirteen patients were treated with adjuvant chemotherapy, most commonly doxorubicin (seven patients) and cisplatin (six patients). The median length of survival was 9.3 years, and there were nine disease-related deaths (43%). Of interest was the progressive decrease in survival with time. The 5-year overall survival rate was 79%; the 10-year rate was 49%. Three patients died of progressive disease >10 years after initial diagnosis. Conclusions: We conclude that leiomyosarcomas arising in childhood and adolescence are associated with a good initial chance of survival that decreases progressively over time. Known prognostic factors from larger adult series are consistent with the present data, but they are not provable because of the small number of patients. In particular, the grade was correlated with surgical margins. Presented at the 49th Annual Cancer Symposium of The Society of Surgical Oncology, Atlanta, Georgia, March 21–24, 1996.     

Suprasellar and sellar tumors in childhood and adolescence.

Suprasellar and sellar region tumors in children constitute a diverse group of lesions. We advocate an aggressive surgical approach to these tumors for diagnostic and therapeutic purposes. With detailed knowledge of the microneurosurgical anatomy of this region, the neurosurgeon can choose from a number of operative approaches on the basis of tumor size and location. The benefits of radiation therapy for craniopharyngiomas and optic chiasmatic/hypothalamic tumors must be carefully weighted against the substantial long-term risks associated with irradiation of the developing brain in this region. During the next decade, the role of primary chemotherapy for germinoma and adjuvant chemotherapy for optic pathway gliomas will be determined. Also, the role of stereotactic radiation therapy, of great value to some children with recalcitrant tumors in the suprasellar region, may be better defined.     

Malignant melanoma in childhood and adolescence

A retrospective chart review of 25 patients under the age of 20 years with malignant melanoma, diagnosed between the years 1938 and 1984 at the University of Iowa Hospitals and Clinics, was conducted. Nine of the patients were under the age of 14 years, and 18 patients were females. There appeared to be a frequent delay in diagnosis of the primary lesion, occasionally incorrect initial diagnosis, and under-treatment in these young patients. Primary treatment varied from limited excision of the lesion to wide excision and regional lymph node dissection. Females appeared to have a better prognosis than males, and wide local excision with or without regional lymph node dissection was associated with the most favorable outcome. Overall 10-year survival in this group of patients was 56 per cent, a rate that compares well to the overall survival in adult patients with malignant melanoma.     

Granulomatous enterocolitis in childhood and adolescence

Fifty-two children and adolescents with granulomatous enterocolitis were followed from 1–24 yr with a mean of 6.9 and a median of 6.0 yr. The protean early manifestations of the illness sometimes presented years before the onset of bowel disease; This is true especially of failure to grow and fever of unknown origin. Granulomatous ileocolitis was present in 12 patients, ileitis and jejunitis in 8, and terminal ileitis in 32. Half of the patients on medical management are doing well. Of 19 patients operated upon, 12 did very well and four had recurrences. Appendectomy after an acute illness was followed by remission in only 3 of 15 patients; seven of these required another operation and four others had several hospitalizations with recurrent disease. This does not suggest that acute ileitis is a separate entity from chronic granulomatous disease. Three of the six patients with severe growth retardation had a spurt of growth after operation. It is felt that, although surgery does not stimulate growth, it offers a year or two of control of the disease to allow growth and, indeed, in a lower but respectable percentage of cases, a long-term respite from symptoms.     

Lupus nephritis in childhood and adolescence

Lupus nephritis in childhood usually presents after the age of 10 years, and presentation under 5 years is very rare. More males (FM ratio 4.51) are affected than in adult-onset cases, but the ratio is the same in prepubertal and pubertal children. The incidence of clinically evident renal disease is greater at onset than in adults (82%), the usual presentation being with proteinuria, 50% having a nephrotic syndrome. Half the children show World Health Organisation class IV nephritis in renal biopsies. Neuropsychiatric lupus is present at onset in 30%, may complicate 50% at some point and remains a major problem. Prognosis has improved greatly over the past 30 years, at least in part the result of immunosuppressive treatment. Treatment of the initial phase may be guided by the severity of the renal biopsy appearances, more aggressive treatment including cytotoxic agents, i.v. methylprednisolone and perhaps plasma exchange, although the value of exchange is not established. Controversy persists as to the most effective cytotoxic treatment in the acute phase, both oral and i.v. cyclophosphamide and azathioprine being used in different units. In the chronic maintenance phase it seems established both clinically and histologically that addition of a cytotoxic agent improves outcome, but again the drug and route of administration are contentious. Azathioprine has the advantage of being safe for pregnancy and not gonadotoxic, whilst i.v. cyclophosphamide has been demonstrated to improve results over prednisolone alone in controlled trials and has advantages in non-compliant patients. No trial comparing the two regimes has been carried out, and one is needed. Today children much less commonly go into renal failure, and the main causes of actual death (15% of patients over 10 years) are now infections and extra-renal manifestations of lupus, principally neurological. Morbidity of the disease and the treatment remain a major problem, especially when treatment exacerbates complications of the disease itself, such as infections, osteonecrosis, thrombosis, vascular disease and possibly neoplasia.     

Cerebral aneurysms in childhood and adolescence

In this study, 24 aneurysms occurring in 23 patients under the age of 18 years (mean 12 years) are analyzed. The male:female ratio was 2.8:1, and the youngest patient was 3 months old. Mycotic lesions and those associated with other vascular malformations were excluded. Forty-two percent of the aneurysms were located in the posterior circulation, and 54% were giant aneurysms. Presenting symptoms included subarachnoid hemorrhage in 13 and mass effect in 11. Several of these aneurysms were documented to rapidly increase in size over a 3-month to 2-year period of observation. All aneurysms were surgically treated: direct clipping was performed in 14; trapping with bypass in four; trapping alone in four; and direct excision with end-to-end anastomosis in two. The postoperative results were excellent in 21 aneurysms (87%), good in two (8%), and poor in one. The pathogenesis of cerebral aneurysms is reviewed.     

Pituitary adenomas in childhood and adolescence

This report describes a group of 25 children and adolescents with biopsy-proven pituitary adenomas. They were found in 33% of patients encountered in our recent study of 74 parasellar tumor detected in patients under 20 years old. Earlier detection accounts for this apparent increase in frequency. This interpretation is supported by the rarity of signs of intracranial hypertension at presentation, as well as decreased incidence and severity of visual failure. More than 70% of patients have evidence of pituitary hypersecretion at presentation. Increased awareness of these endocrinological signs as well as improved hormonal assays facilitate both diagnosis and management. Hypopituitarism is rare; the important exception is suppression of gonadotrophic function that is known to occur early in the natural history of intrasellar pituitary adenomas. The low incidence of suprasellar and extrasellar extension is documented by both radiographic and operative findings. Transsphenoidal resection was employed in 92% of patients, with an estimated total resection in 73% of cases. Follow-up studies show a high quality of survival; no patient has severe neurological deficit and less than half of them require replacement therapy. The recurrence rate is 8%. Our findings do not support a previous report that pituitary adenomas in this age group are frequently extrasellar and invasive.     

Meniscal cyst and magnetic resonance imaging in childhood and adolescence.

We retrospectively reviewed 11 cases of meniscal cyst diagnosed at the Mayo Clinic from 1976 through 1990. Patient age ranged from 4 to 18 years (average 13 years). Two cases were diagnosed on clinical findings alone. Arthrograms were available in five cases. Magnetic resonance imaging (MRI) was used in four cases. These cases illustrate the difficulty in making the diagnosis of meniscal cyst. MRI is a useful tool for diagnosing meniscal cyst and aids in surgical planning. Because of the extensive differential diagnosis of the meniscal cyst, we recommend MRI in preoperative evaluation of soft tissue masses about the knee.     

Testicular germ-cell tumors in childhood and adolescence

Summary Testicular germ-cell tumors are relatively rare in childhood and adolescence, accounting for only 3.9% of all neoplasms. However, they have become a model for curable cancer. Furthermore, most of them have accurate serum markers [beta-human chorionic gonadotropin and alpha-fetoprotein], which provide in clinical stage I disease after semicastration a wait and see program. MAHO 82, 88, and 92 were cooperative studies on the treatment of testicular germ-cell tumors in childhood and adolescence. Between 1982 and 1993, 137 patients were registered. In all, 76 patients suffered from yolk-sac tumors (YST); 30, from differentiated teratomas (TD); 29, from malignant teratomas of either intermediate (MTI), undifferentiated (MTU), or trophoblastic type (MTT); and 2, from seminomas. All patients received semicastration. Chemotherapy was given to 53 patients on the basis of disease stage and histology. Standard therapy consisted of four courses of vinblastine, bleomycin and cisplatin. However, if viable tumor was suspected after two courses, delayed laparotomy was performed (seven patients). If there was then complete tumor regression, standard therapy was continued (four patients). If there was an incomplete tumor response, the patients received as salvage therapy three courses of etoposide (VP-16), ifosfamide, and cisplatin (three patients). Among the patients with YST, 73 had stage I disease and 3, higher-stage disease; 1 of these died due to tumor progression. In all, 56 patients were followed according to the wait and see policy; 9 of these needed a delayed standard chemotherapy. The rate of no evidence of disease (NED) was 98%. Among the patients with TD, 30 had stage I disease. The NED rate was 100%. Among the patients with malignant teratomas (MTI, MTU, and MTT), 13 had stage I disease. The NED rate was 100%. Ten patients had stage II disease and received chemotherapy. The NED value was 100%. Six patients had stage III disease; three died. Altogether, 26 of 29 patients survived disease-free. In summary, the probability for the disease-free survival of all 137 patients suffering from testicular germ-cell tumors is 97% after a median observation period of 60 months.