Citations

Abstract Background The authors have reported a newly recognized hypoglyacemic disorder, non-insulinoma pancreatogenous hypoglycaemia syndrome (NIPHS), which is characterized by postprandial hyperinsulinaemic hypoglycaemia, negative 72-h fast, negative transabdominal and intraoperative ultrasonography, spiral computed tomography and coeliac axis angiography, positive selective arterial calcium stimulation test (SACST), absence of insulinoma but presence of islet hypertrophy and nesidioblastosis, and relief of symptoms from gradient-guided partial pancreatectomy. Experience with the next five patients with this disorder (6–10; 1998–1999) compared with the original five (1–5; 1996–1998) is reported. Results Wide age range, male predominance, postprandial occurrence of symptoms, negative radiological localization, nesidioblastosis/islet hypertrophy, but no insulinoma characterized all ten cases. Response to SACST indicated widespread islet hyperfunction in patients 1 and 5; limitation to distribution of the SPA and GDA in patients 3, 4, 6 and 8, to distribution of the SPA in patients 2, 7 and 9, and to distribution of the SMA in patient 10. Except for patient 5, pancreatic resection to the left of the SMV was performed when only the SPA was positive on the SACST. Patients 5 and 8 have had recurrence of symptoms; they resolved within 3 months for patient 5. Conclusion NIPHS can usually be treated successfully with gradient-guided partial pancreatectomy.

Abstract Background Calciphylaxis is a rare, painful, life-threatening problem of cutaneous necrosis and refractory healing seen in patients with uraemia and secondary hyperparathyroidism. The pathogenesis involves abnormalities in calcium and phosphorus metabolism, and acute deposition of calcium in tissues. Methods The clinical course of 16 patients diagnosed with calciphylaxis at this institution from 1994 to 1998 was reviewed. Results Fourteen women and two men, with a mean age of 56 (range 39–70) years, presented with chronic renal disease of various causes, hyperparathyroidism and characteristic skin lesions. All patients underwent intensive medical therapy, including haemodialysis (n = 16), parathyroidectomy (PTX) (n = 7) and skin debridement of cutaneous lesions (n = 8). Mean preoperative serum values in surgical (PTX) versus non-surgical patients were: calcium 9·9 and 9·3 mg dl−1 (P = 0·25); phosphorus 5·8 and 4·9 mg dl−1 (P = 0·04); calcium–phosphorus product 61·6 and 45·2 (P = 0·03); and parathyroid hormone (PTH) 56·8 and 5·9 pmol l−1 (P = 0·0001) respectively. Mean postoperative values for calcium (8·7 mg dl−1), phosphorus (4·2 mg dl−1), calcium–phosphorus product (30·5) and PTH (4·0 pmol l−1) in surgical patients changed significantly (P < 0·05). Median overall survival was 9·4 months with 15 patients now deceased. Median survival was 14·8 and 6·3 months for PTX versus no PTX (P = 0·22), 14·1 and 6·1 months for skin debridement versus no debridement (P = 0·08), 12·4 and 6·6 months for proximal versus distal skin lesions (P = 0·60), and 6·5 and 13·9 months for diabetic versus non-diabetic patients was (P = 0·11). Conclusion Calciphylaxis appears to have a female preponderance with a very dismal prognosis. A multidisciplinary approach using frequent haemodialysis to normalize serum calcium and phosphorus levels, and local debridement of skin lesions seems prudent. PTX cannot be recommended routinely in all such patients unless severe secondary hyperparathyroidism with poorly controlled metabolic disturbance mandates surgery. The exact indications for PTX for calciphylaxis remain unclear.

We describe a 63-year-old smoker who suffered from intractable facial pain secondary to an underlying lung neoplasm. Data from 30 previously reported and similar cases are also summarized. The clinical triad of a smoker suffering from periauricular pain and an elevated ESR should alert the clinician to the possibility of an occult lung mass. In these cases a computed tomography of the chest should always be obtained. Previously refractory pain typically responds to surgical resection of the mass and/or radiation therapy.

The syndrome of orthostatic (low pressure) headaches is well described and most commonly occurs following deliberate violation of the dura (e.g. lumbar puncture). This syndrome can also occur spontaneously and results from the leakage of CSF. We describe three patients who suffered from spontaneous CSF leaks secondary to bony pathology of the cervical spine, and propose that this may be a more common aetiology than originally thought. Often these patients are difficult to manage medically, and surgery may be necessary for symptomatic relief.

Abstract Background Primary sarcomas of the liver are extremely rare in adults. Optimal therapeutic approaches remain unclear. Methods Twenty consecutive adult patients who had surgical treatment for primary hepatic sarcomas were reviewed. Patient age ranged from 23 to 80 years. Other than one patient with primary hepatic angiosarcoma who had a history of thorium dioxide colloid (Thorotrast) exposure 23 years before diagnosis, no predisposing causes were apparent. Nineteen patients had hepatic resection and one patient had an orthotopic liver transplant. No patient received neoadjuvant chemotherapy or radiotherapy but radiotherapy was delivered intraoperatively in one patient. Results Leiomyosarcoma was the most common histological type of sarcoma diagnosed (five of 20 patients), followed by malignant solitary fibrous tumour (four) and epithelioid haemangioendothelioma (three). Fourteen tumours were high-grade sarcomas and six were low grade malignancies. Thirteen patients developed a recurrence. Distant metastases (ten patients) and intrahepatic recurrence (six) were the predominant sites of initial treatment failure. Six patients received salvage chemotherapy. Histological grade was the only factor significantly associated with overall patient survival (P = 0·03). With complete resection, patients with high-grade tumours had a 5-year survival rate of 18 (95 per cent confidence interval 5–62) per cent compared with 80 (52–100) per cent for patients with low-grade tumours. The 5-year survival rate for all 20 patients was 37 (20–60) per cent. Conclusion Surgical resection is the only effective therapy for primary hepatic sarcomas at present. Better adjuvant therapy is necessary, especially for high-grade malignancies, owing to the high failure rate with operation alone.

Wilfred Harris was a London neurologist with a significant interest in the treatment of neuralgia. Harris' descriptions of what he called migrainous neuralgia were the first recorded of cluster headache in the English medical literature. He was probably one of the first to describe the cluster phenomenon itself and the effectiveness of ergotamine in treating acute attacks of cluster headache. His seminal contributions to the clinical and therapeutic spectrum of cluster headache are reviewed.

We report two cases of SUNCT that demonstrate the medically and surgically refractory nature of this disorder and support the hypothesis that the causative ‘lesion’ lies within the central nervous system. After both patients had failed medical therapies, the first underwent a glycerol rhizotomy, gammaknife radiosurgery and microvascular decompression of the trigeminal nerve. The second patient underwent gammaknife radiosurgery of the trigeminal root exit zone and two microvascular decompression surgeries. Neither patient benefited from these procedures. Currently, the first patient suffers from anaesthesia dolorosa and the second patient from unilateral deafness, chronic vertigo and dysequilibrium as a result of surgical trauma. These cases of SUNCT highlight the uncertainty regarding the role of surgery given the potential for significant morbidity. These cases also suggest that SUNCT originates and may be maintained from within the CNS and this central locus explains why SUNCT is not typically amenable to interventions aimed at the peripheral portion of the trigeminal nerve.

Spontaneous CSF leaks are increasingly recognized, and a broader clinical and imaging spectrum of the disorder is emerging. The headaches of CSF leaks are typically orthostatic, but sometimes especially with chronicity the orthostatic features are blurred into lingering chronic daily headaches. Additional types of headache are also increasingly recognized. Two patients with spontaneous CSF leaks presented with intermittent transient severe headaches provoked by Valsalva-type manoeuvres. Orthostatic features were absent and the patients were asymptomatic if they avoided the provoking manoeuvres. One patient had been treated for 6 years for benign exertional headaches and had failed many medical treatments, including courses of indomethacin. He was found to have a leak from cribriform plate. The second patient had been symptomatic for several months, had diffuse pachymeningeal gadolinium enhancement on head magnetic resonance imaging, spinal meningeal diverticula, and CSF leak at the thoracic spine level. Headaches that mimic benign exertional headaches are yet another mode of the still broadening clinical presentation of spontaneous CSF leaks.

Cerebrospinal fluid (CSF) leak is recognized to cause headaches that typically but not invariably have orthostatic features (present in upright posture, relieved by recumbency). Head magnetic resonance imaging (MRI) typically shows diffuse pachymeningeal enhancement. A 24-year-old woman, after resection of a right temporoparietal glioma, developed CSF leak from the craniotomy site, resulting in subgaleal fluid collection and associated with diffuse pachymeningeal gadolinium enhancement as well as posture-related headaches. The headaches, however, were present in a recumbent position and relieved after several minutes of being in an upright position. It is postulated that CSF leak took place when the patient was recumbent and ceased when she was upright, a position in which there is decrease in intracranial pressure. After cessation of the leak, along with disappearance of subgaleal collection of CSF, both the headaches and the pachymeningeal enhancement resolved.