1428

atrioventricular block may be the presenting feature of anothermitochondrial disorder, Kearns-Sayre syndrome, in which

myocardial ultrastructural abnormalities have been demonstrated.6In LHON young males are at increased risk of sudden blindnessand this report raises the possibility that maternal relatives may alsobe at risk of arrhythmia and sudden death.

Departments of Medicine and Pathology,Royal Hobart Hospital,Tasmania, Australia,and Johns Hopkins Center

for Hereditary Eye Diseases,Baltimore, Maryland, USA

S. P. C. BOWERI. HAWLEYD. A. MACKEY

1. Wilson J. Leber’s hereditary optic atrophy, some clinical and aetiologicalconsiderations. Brain 1963; 234: 614-18.

2. Rose FC, Bowden AN, Bowden PM. The heart in Leber’s optic atrophy. Br JOphthalmol 1970; 54: 388-292.

3. Nikoskelainen EK, Wanne O, Dahl M. Pre-excitation syndrome and Leber’shereditary optic neuroretinopathy. Lancet 1985; 1: 969.

4. Zeviani M, Gellera C, Antozzi C, et al. Maternally inherited myopathy andcardiomyopathy: association with mutation in mitochondrial DNAtRNALeu(URR). Lancet 1991; 338: 143-47.

5. Vidaillet HJ, Pressley JC, Henke E, Harrell FE, German LD. Familial occurrence ofaccessory atrioventricular pathways (preexcitation syndrome). N Engl J Med 1987;317: 65-69.

6. Charles R, Holt MB, Kay JM, Epstein EJ, Russell Rees J. Myocardial ultrastructureand the development of atrioventricular block in Kearns-Sayre syndrome.Circulation 1981; 63: 214-19.

Successful pregnancy in severe diabetes

SIR,-We report pregnancy and normal delivery in a 27-year-olddiabetic patient being treated with intraperitoneal insulin infusion(CPII) by means of a programmable implantable Infusaid, model1000, device.

In September, 1989, this device was implanted as part of a safetyand feasibility study. The patient had brittle diabetes (highfrequency of moderate hypoglycaemic reactions complicated bytransient hemiplegia) despite continuous subcutaneous insulininfusion (CSII). Diabetes duration was 21 years with no late

complications. After implantation, hypoglycaemia frequencyimproved and neurological complications disappeared. Data beforeand after implantation were, respectively, HbAlc 63% vs 6%(normal 4-4%-6%); mean number of monthly hypoglycaemicepisodes (blood sugar below 3-6 mmol/1) fell from 45 to 20-7; andblood glucose 24 h standard deviation changed from 4-55 to 2.05mmol/l. In September, 1990, due to a technical problem, CPII wasdiscontinued and a transient CSII regimen was used for 3 weeks; amoderate hypoglycaemic reaction complicated by hemiplegia tookplace after about 1 week of this regimen.

In March, 1991, the patient unexpectedly became pregnant.HbAjc was 7-2% and she had no late complications. Because of hervery brittle diabetes and history of neurological complications afterhypoglycaemic events, we decided to pursue CPII with the implantfor as long as possible. Throughout pregnancy, the patient was seenevery 2 weeks to maintain optimum metabolic control and monitorthe pump. During her pregnancy HbAlC decreased from 6-9% to5-5%, blood sugar daily standard deviation varied between 2-94 and4-05 mmol/1, and monthly frequency of hypoglycaemic eventsincreased from 23 to 30, but no moderate or severe episodes werereported. She tolerated the pump well until 3 days before delivery,when she had some discomfort. Ultrasonography was done duringevery trimester. At the last examination labia majora hypertrophiaor hypospadias were suspected. Other indices were normal. Labourwas artificially induced at fetal maturation. At 37 weeks, thepump was filled with diluent and while fasting she receivedintravenous (iv) insulin and iv 10% glucose during labour. Aboy (birthweight 3410 g, length 50 cm, Apgar score 10) wasdelivered by forceps. He had hypospadias. His blood glucoseconcentration was 15 mmol/1 and blood calcium 2-15 mmol/1.No late hypoglycaemia or metabolic complications were seen.Mother and baby were discharged 5 days after delivery. Afterpump rinsing due to slowdown in infusion, the pump was filledwith insulin and the CPII regimen started again, without

complications.

The use of CPII allowed very good and stable glucose controlthroughout pregnancy despite brittle diabetes and the device waswell tolerated throughout pregnancy and during labour. Thisdevice facilitates the use of CPII, as we have shown before.’ Otherworkers2 have reported diabetic pregnancy managed by CPII withanother device, but multiple local infections and thus transient badcontrol episodes were recorded. Our technique offers a chance foryoung women with very brittle diabetes who wish to have a baby.

Services of Endocrinology and Nutritional Diseases,Gynaecology and Obstetrics,Urgent and General Surgery, and Endocrinology,

Hôpitaux Universitaires de Strasbourg,Hôpital Civil,67091 Strasbourg, France

N. JEANDIDIBRE. MIRANDAC. MATHELINS. BOIVINM. PINGET

1. Jeandidier N, Pmget M, Keipes M, Charton MN, Marescaux J, Réville PH. Longterm treatment of type I diabetes in 28 patients using two different implantableprogrammable pumps Transplant Proc (in press).

2. Fonseca VA, Menon RK, O’Brien MS, Fernando ON, Stephen R, Dandona P.Diabetic pregnancy managed with intraperitoneal insulin. Diabet Med 1987; 4:74-76

Octreotide

SiR,—Your April 4 editorial (p 837) points out the importance ofoctreotide in treatment of various pathological conditions. Theability of octreotide to suppress the exocrine pancreas suggests thatit also has potential in the treatment of pancreatic fistulas. You fail todiscuss this new indication of octreotide therapy. However,experience with use of octreotide in external pancreatic fistulas hasgreat clinical importance, since we do not have an effectivestandardised method for conservative treatment of this condition.Published data about the effect of octreotide in pancreatic fistulasare contradictory.1-3We have done a prospective trial to assess the efficacy of

octreotide in the management of high-output external pancreaticfistulas. A total of 11 male and 5 female patients (mean age 45-5years, range 28-58) with postoperative external, high-outputpancreatic fistulas were investigated. In 3 patients, a fistula

developed after pancreatic injury at abdominal surgery, and in 3others it did so after surgical removal of insulin-secreting islet celltumours. In the remaining 10 patients, pancreatic fistulas arose afterabdominal surgery for chronic pancreatitis or following its

complications (pseudocysts). The output of the fistulas was

constant before the administration of long-acting somatostatin andvaried between 190 and 570 ml per day (mean 335 ml per day).Fistula-related infection could not be clearly demonstrated in anypatient. The subjects had received total parenteral nutrition (TPN)since the appearance of fistulas, but the fistula output had notchanged. Octreotide (Sandostatin) therapy was begun at a mean of17 days (range 4-35 days) after appearance of the fistula. Thepatients received 0- 1 mg octreotide subcutaneously every 12 h. Onthe first day of treatment, mean fistula output had fallen to 45%(range 31-74%) of pretreatment output. By day 3, it had decreasedto 30% (range 0-74%) of the pretreatment value. The averagelength of treatment was 8 days (range 3-15). The fistula was closedin 14 of the 16 patients; surgical procedures were done in the twofistulas that did not heal. There was a significant negativecorrelation between fistula output and its amylase concentration(r = - 0-87, p < 0-01). An octreotide related side-effect could not bedemonstrated in any patient.These results show that octreotide has a consistent, clear effect in

decreasing fistula output, and indicate that it is equally successful inproducing favourable changes in the secretion of pancreatic fistulasirrespective of aedologv.

Department of Medicine and Surgery,Semmelweis University Medical School,1083 Budapest, Hungary

Z. TULASSAYL. FLAUTNERI. FEH&Eacute;RV&Aacute;RI

1. Miller BM, Traverso WL, Freeny PC, Abumrad NN. Failure of somatostatin or ananalog to promote closure of end pancreatic fistulae. Int J Pancreatol 1989; 4: 65-72.

2. Lansden FT, Adams DB, Anderson MC. Treatment of external pancreatic fistulaswith somatostatin. Am Surg 1989; 55: 695-98.

3. Prinz RA, Pickleman J, Hoffman JP. Treatment of pancreatic cutaneous fistulas with asomatostatin analog. Am J Surg 1988; 55: 36-42.