The radiologist says malrotation: does the surgeon operate?

The management of malrotation when it is an incidental finding is unclear. This retrospective study reports an analysis of radiological and operative findings in a series of 71 patients. There were no deaths. We report a false-positive rate of 15% for upper gastrointestinal contrast studies reported as showing malrotation. Our findings and a review of the literature demonstrate that in the asymptomatic child over 2 years of age, the evidence supporting mandatory correction of malrotation is weak.

Wilms tumor associated with polycythemia: case report and review of the literature.

PURPOSE AND METHODS: A case of polycythemia with a normal serum erythropoietin is described, which led to the diagnosis of Wilms tumor. The clinical features of the reported cases of Wilms tumor associated with polycythemia are reviewed. RESULTS: An asymptomatic 6-year-old boy with polycythemia, a normal serum erythropoietin, and no evidence of erythroid colony forming activity in his serum was found to have a Wilms tumor. After resection and chemotherapy, he has had no recurrence of either the polycythemia or the Wilms tumor. There have now been 10 cases of Wilms tumor reported: 7 patients were more than 16 years of age, 8 were boys, and 9 were clinical stage I with a favorable histology. CONCLUSIONS: Polycythemia is a rare manifestation of Wilms tumor that can occur in the absence of an elevated serum erythropoietin and has an association with male gender, older patient age, and low clinical stage. Children with unexplained polycythemia should be investigated for Wilms tumor, even if the serum erythropoietin level is normal.

Congenital spigelian hernia with cryptorchidism.

Congenital spigelian hernia (SH) is a rarity. The authors present two cases of congenital SH with undescended testis, and examine the significance of the concurrence of these events.

Omental cysts: an unusual cause of abdominal distension in children.

Congenital cystic lesions arising in the omentum are a rare finding in the paediatric age group. They are difficult to diagnose preoperatively as they have few distinct features; the diagnosis is often confirmed only at lalaparotomy. They are thought to arise due to a developmental abnormality of the lymphatic system. Two cases are presented and the literature reviewed. The treatment of choice is complete resection. Long-term follow-up is unnecessary due to their benign nature.

Spontaneous Clostridium septicum myonecrosis in congenital neutropaenia.

Spontaneous Clostridium septicum myonecrosis is an uncommon disorder that has been described in association with malignancy, immunosuppression and neutropaenia. Typical clostridial myonecrosis develops without a visible portal of entry and mortality is high. The pathogenesis is not completely understood but the clostridia may gain access to the circulation via areas of ileo-caecal ulceration secondary to enterocolitis, antibiotics or neoplasms. A 5 year old boy with congenital neutropaenia presented with spontaneous Clostridium septicum myonecrosis in the thigh. Limb salvage was achieved using antibiotics, hyberbaric oxygenation and selective debridement. The portal of entry may have been the gastrointestinal tract as colonic ulceration may occur in neutropaenia, and pre-morbid clindamycin administration may have encouraged overgrowth of colonic clostridia.

Local failure in childhood rhabdomyosarcoma and undifferentiated sarcoma: prognostic factors and implications for curative therapy.

In this retrospective review, the risk factors for local failure in childhood rhabdomyosarcoma and undifferentiated sarcoma were assessed in 49 patients managed by a multi-disciplinary team at the Prince of Wales Children's Hospital, Sydney, between 1970 and 1988. Average follow-up time was 6.1 years. Sixteen of 49 patients experienced local failure defined as local recurrence after complete excision (5/20) or progressive local disease following incomplete resection (11/29). Fourteen of 16 patients who experienced local failure have died. Using logrank analysis of time to local failure, we found significantly increased risk with "non-embryonal" histology (P = 0.032), residual tumour (P = 0.052, higher IRS group (P = 0.088), "inadequate radiotherapy" for residual tumour (P = 0.001), delay in definitive local treatment (P = 0.038) and Adriamycin-containing chemotherapy (P = 0.017). When these factors were examined by multivariate analysis (Cox regression), only the presence of residual tumour after resection, "inadequate radiotherapy" for residual tumour (P < 0.001), and delay in definitive local therapy (P < 0.037) were shown to have independent significant association with local failure. We conclude that local failure may be avoided by prompt local treatment by either complete surgical resection or adequate radiotherapy.

Congenital short gut, malrotation, and dysmotility of the small bowel.

A familial syndrome of congenital short small bowel, malrotation, and functional bowel obstruction is presented and illustrated in three male siblings with one long-term survivor. Including the present three cases there have been 18 similar cases found in the literature. This disorder appears to be a sex-linked recessive inheritance. Early barium meal and follow through in siblings of patients with this condition will detect malrotation that should be surgically corrected in order to prevent mid-gut volvulus. Intravenous alimentation and early introduction of enteral feeding is important, not only to maintain nutrition but to encourage adaptation of the associated short and dysmotile bowel. These measures aim to improve the outcome of this frequently, but not invariably, fatal disorder.

Torsion of the testis.

Torsion of the testis demands early diagnosis and urgent operative treatment. This study of all patients with torsion of the testis who were admitted to The Prince of Wales Children's Hospital, The Prince of Wales Hospital and The Prince Henry Hospital, Sydney, between 1965 and 1987, reinforces the need for urgent diagnosis and treatment and evaluates the diagnostic value of the sign of the contralateral horizontal testis. One hundred and thirty-one patients were studied; the majority was in the adolescent age-group. If operation were delayed beyond 24 hours without spontaneous relief of the symptoms or manipulative detorsion being performed, then at follow-up 5.5% of testes were normal; however, if operation were performed within 24 hours of the onset of symptoms, 90% of testes were normal at follow-up. In all cases in which it was sought, the sign of the contralateral horizontal testis was found, which confirms the bilaterality of the condition and that a high investment of the tunica vaginalis predisposes to torsion of the testis.

Management of oesophageal stenosis in epidermolysis bullosa dystrophica.

Seven patients with epidermolysis bullosa dystrophica and chronic and recurrent oesophageal lesions such as spasm, strictures, and complete occlusion were studied. Dysphagia could be cured with drugs if it was caused by bullae formation or spasm. If oesophageal strictures were present, endoscopy and bouginage with corticosteroid prophylaxis during the quiescent phase of the disease was a safe and useful procedure. We have also given corticosteroids, which reduced the oedema caused by bullae formation and oral phenytoin, which reduced epithelial detachment by inhibiting collagenase activity. Verapamil counteracted oesophageal spasm and pureed food during periods of dysphagia reduced blistering of the upper oesophagus.

Male pseudohermaphrodite reared as a female.

A 31-year-old male pseudohermaphrodite has been brought up as a female. The parents had told their child not to discuss the problem of ambiguous genitalia with anyone. As a teenager masculinization had occurred. Chromosome analysis of both peripheral blood and gonadal tissue was 46XY. The internal gonads were testes surrounded by a rim of ovarian stroma. A rudimentary cervix opened into a small vagina, as did the urethra in a valve-like fashion. The phallus was amputated during surgical reconstruction of the external genitalia to create a phenotypic female and the vagina was enlarged. Oestrogen therapy resulted in breast development.

Management of children with chronic femoral artery obstruction.

Chronic obstruction of the femoral artery in children is a complication of diagnostic angiography, cardiac catheterization and umbilical artery catheterization. We have seen 22 cases and have operated on 7. The indications for operation are claudication, muscle wasting, and shortness of the lower limb. The diagnosis is confirmed by a Doppler profile and the abnormal vascular anatomy demonstrated by angiography via the contralateral femoral artery. We have done 4 ilio-femoral, two femo-femoral and one femoro-popliteal bypass grafts utilizing the long saphenous vein. Results have been good in five, the other two grafts having thrombosed.