Nutcracker esophagus is essentially a manometric diagnosis characterized by high-amplitude, often prolonged duration of peristaltic contractions in the distal two thirds of the esophagus. Its association with noncardiac chest pain and/or dysphagia has been recognized and reported by numerous esophageal motility laboratories. There are very few long-term studies of the natural history of this abnormality. We report a patient who presented with dysphagia and, on initial investigation, was found to have classical nutcracker esophagus. On reinvestigation three years later, however, he had developed achalasia of the cardia. The transition from nutcracker esophagus to achalasia has not previously been reported.

Exomphalos major can be successfully treated non-operatively, allowing immediate enteral feeding and early discharge while granulation takes place. In this series, exomphalos major has an incidence of 1 in 26,000, mortality is 46% and severe pulmonary hypoplasia is present in 54% of infants.

Of 1200 patients referred to the esophageal laboratory at Guy's Hospital for investigation of suspected esophageal motility disorders, 61 (5.1%) were diagnosed as diffuse esophageal spasm. Twenty of these patients whose symptoms were severe did not respond to conservative treatment and were treated by balloon dilatation. Results were good in 14 and poor in six patients, which included one esophageal perforation. Diffuse esophageal spasm was diagnosed where more than 30% nonperistaltic activity was demonstrated by manometry. Lower esophageal sphincter pressure and relaxation were normal in all cases except one. Gastroesophageal reflux was present in four of five poor responders who were examined by 24-h ambulatory pH monitoring, and in only one of 10 good responders. Three of the six patients in whom balloon dilatation was successful proceeded to full-length myotomy, with relief of symptoms in two. The indications for, and results of, balloon dilatation in this condition are discussed, and a new radiological sign is described.

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Objective To describe the clinical features, treatment and outcome of functioning adrenocortical tumours in children, and to assess the value of tumour size as a marker for malignant potential. Patients and methods Twenty‐one children (mean age at presentation 4.9 years, range 0.6–11; 12 girls and nine boys) who presented with a functioning adrenal mass over a 29‐year period (1972–2000) were assessed. Clinicopathological features and outcome information were obtained from a structured review of the case‐notes. Before 1980 the diagnosis and staging was mainly obtained by intravenous pyelography and plain X‐ray. Since 1980, ultrasonography, computed tomography and more recently magnetic resonance imaging were the methods of choice. Results The mean (range) duration of symptoms before diagnosis was 31 (2–108) months. The symptoms and signs at presentation comprised virilization (18 children), Cushingoid features (three), precocious puberty (three) and hypertension (four). Three children had malignant neoplasms, one presented with metastasis and of the other two, one died from the disease within 6 months, despite adjuvant chemotherapy and radiotherapy. The third girl is disease‐free 11 years after complete excision of the neoplasm. Eighteen children had a benign neoplasm and all are alive and free of recurrence. In most of the children surgical extirpation was relatively simple, blood loss minimal, recovery uneventful and the hospital stay short (mean 5 days). The virilizing effects of all the neoplasms gradually resolved after surgery within a median (range) of 27 (6–108) months. Conclusions Functioning adrenocortical neoplasms in children are rare, with a peak incidence in the first decade, are predominantly unilateral and more common in girls. Most are benign, but both benign and malignant lesions can present with virilizing and Cushingoid features. The malignant neoplasms have an extremely poor prognosis, especially if excision is incomplete, despite adjuvant chemotherapy and radiotherapy. Although there is an association between tumour size and malignancy, this cannot be used as a reliable individual discriminator.

Ovarian teratomas in prepubertal females are uncommon, but familial ones are exceedingly rare. We report an ovarian teratoma in an 8-year-old girl, her mother, and her maternal grandmother. The risk of a metachronous tumor and subsequent complications (such as torsion) in the contralateral ovary remain unclear. There is no clear guidance on follow-up management of patient and family members in the literature. We have reviewed the literature and discuss the challenges for the pediatric surgeon arising from such cases.

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