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Journal of the Royal Society of Medicine Volume 85 February 1992
duodenum or rarely common bile duct5. A less common complication is spontaneous rupture into the abdominal cavity with a development of pancreatic ascites5. Although it is customary to treat pseudocysts operatively, recent advances have allowed both percutaneous and endoscopic approaches to their management. The peptide hormone somatostatin reduces both exocrine and endocrine pancreatic secretions'. Recently somatostatin treatment has been shown to reduce the local complications of acute pancreatitis6, and to treat external pancreatic fistulae
successfully'. Therefore it was reasonable to try somatostatin therapy in this case of a ruptured pseudocyst which is, of course, an internal pancreatic fistula. The clinical biochemical and radiological improvements were dramatic. Whilst it is impossible to prove that these improvements were due to somatostatin treatment, it seems highly probable given the temporal relationships of these to the commencement of somatostatin treatment (Figure 2). This is the first report of the use of somatostatin treatment for a ruptured pancreatic pseudocyst. Another unusual feature ofthis case was the development of subcutaneous fat necrosis. After pancreatitis, fat necrosis occurring in the mesentery is common. Subcutaneous fat necrosis however is rare, but is well recognized
Neonatal cleft lip repair
M W H Erdmann FRCS N Waterhouse FRCS(Plast) Department of Plastic and Reconstructive Surgery, Charing Cross Hospital, Fulham Palace Road, London W6
as a complication of acute pancreatitis7. It has unknown aetiology7. Nevertheless it was interesting to note the temporal relationship of its improvement with the commencement of somatostatin treatment.
References 1 Williams ST, et al. Effect of octreotide acetate on pancreatic exocrine function. Am J Surg 1989;157:459-62 2 Ranson JHC. The role of surgery in the management of acute pancreatitis. Ann Surg 1990;211:382-93 3 Imrie CW, Shearer MG. Diagnosis and management of severe acute pancreatitis. In: Russell RCG, ed. Recent advances in surgery, vol. 12. Edinburgh: Churchill Livingstone, 1986:143-54 4 Stroud WH, Cullom JW, Anderson MC. Hemorrhagic complications of severe pancreatitis. Surgery 1981;90:657-65 5 Bradley EL III. Pancreatic Pseudocysts. In: Bradley EL IH, ed. Complications ofpancreatis. Medical and surgical management. Philadelphia: WB Saunders, 1982:124-53 6 Choi TK, Mok F, Zhan WH, Fan ST, Lai ECS, Wong J. Somatostatin in the treatment of acute pancreatitis: a prospective randomised controlled trial. Gut 1989;30:223-7 7 Wormsley KG. Diseases of the pancreas. In: Weatherall DJ, Ledingham JGG, Warrell DA, eds. Oxford textbook of medicine. Oxford: Oxford University Press, 1983:12.156-12.168
(Accepted 11 September 1991)
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Case presented to Section of Plastic Surgery, 8 May 1991
Keywords: cleft lip; ultrasound
A case is reported where a false negative antenatal ultrasound was performed to exclude a cleft abnormality. The reliability of ultrasound is questioned and the role of neonatal cleft lip repair is discussed.
Case report A newborn male neonate was referred with a diagnosis of a unilateral complete cleft lip and palate. He had no other congenital abnormalities. The parents' first child had also been born with a bilateral cleft lip and palate and has undergone numerous corrective surgical procedures. Subsequently the mother developed severe postnatal depression, for which she required prolonged psychotherapy. With the onset ofthe second pregnancy both parents sought antenatal counselling, and ultrasound scans at 16 and 18 weeks (Figure 1) were reported as normal. The parents were reassured and elected to continue with the pregnancy. At birth, when the cleft defect was apparent, the child was immediately rejected by both parents, with the father in turn rejecting the mother. In view of the fragmentation of the family unit it was decided to repair the cleft lip as a matter of urgency. The neonate was admitted to Charing Cross Hospital, where he underwent a standard Millard lip repair and was Correspondence to: Mr M W H Erdmann, 'Alderley', Lambridge Wood Road, Henley-on-Thames, Oxon RG9 3BP
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ital the same day. On transferred back to the review 2 weeks later both parents expressed their delight with the operative result, and were reunited as a family. Discussion The incidence of combined cleft lip and palate deformities in the UK is stated by Wile6n1 to be 1.47/1000 live births and has a fourfold preom in- males. The predicted recurrence of a cleft d cef mn children with one affected sibling is 3.2%, and with two affected siblings is 9%2. Only 3% of a clinic cleft population can be linked to identifiable syndromal aetiologic factors, such as chromosomal aberrations or teratologic syndromes secondary to drug and alcohol ingestion. The great majority of clefts fall into a 'multifactorial inheritance' category describing a strong familial tendency without Mendelian inheritance patterns.
0141-0768/92/ 020108-02/$02.00/0 © 1992 The Royal Society of Medicine
Journal of the Royal Society of Medicine Volume 85 February 1992 Ultrasound in the prenatal diagnosis of cleft defects in the fetus was first popularized by Christ and Meininger in 19813. However, its accuracy in the detection of cleft abnormalities has only recently been investigated46. Pilu et al.6 analysed 223 patients at risk for fetuses with craniofacial malformations and noted a false negative rate of 1% at ultrasound. Fetoscopy can be useful in visualizing cleft defects directly, but is associated with an unacceptably high miscarriage rate of 3% in an otherwise non-life threatening deformity7. In order to maintain the high standard of ultrasound diagnosis demanded in the first two trimesters ofpregnancy, it is important to identify tertiary centres of expertise in this field. High resolution ultrasound is becoming increasingly available, and is capable of detecting most structural abnormalities of the fetus8. It is in the interests of the child that the obstetric ultrasound department is overseen by a specialist in perinatal medicine, and that initial counselling is undertaken only by those with expertise in paediatric plastic surgery. Magnetic resonance imaging (MRI) may prove superior to ultrasound, as it is non-invasive and provides the best definition of soft tissues of any imaging technique to date. Its risks and benefits for fetal studies have not yet been determined. Cleft lip repair in neonates exacts a high degree of surgical and anaesthetic skill which must be supported by paediatric neonatal care. Fetal cranio-facial surgery has not yet been attempted in humans, although the psychological and social
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benefits of being born with a repaired defect may be considerable. Animal models suggest advantages including reduced scarring and improved health and nursing. In summary, this case highlights the family dynamics leading to the necessity for neonatal lip repair and also the limitations of antenatal ultrasound in the diagnosis of cleft defects. References 1 Wilson ME. A ten-year survey of cleft lip and cleft palate in the South West region. Br J Plast Surg 1972;25:224 2 Curtis E, Frazer FC, Warburton D. Congenital cleft lip and palate: risk factors for counselling. Am J Dis Child 1961;102:853 3 Christ JE, Meininger MG. Ultrasound diagnosis of cleft lip and palate before birth. Plast Reconstr Surg 1981;68:854-9 4 Hegge FN, Franklin RW, Watson PT, Calhoun BC. Fetal abnormalities commonly detectable on obstetric ultrasound. J Reprod Med 1990;35:392-7 5 Saltzman DH, Benacerraf BR, Frigoletto FD. Diagnosis and management of fetal facial clefts. Am J Obstet Gynecol 1986; 155:377-9 6 Pilu G, Reece EA, Romero R, Bovicelli-L, Hobbins JC. Prenatal diagnosis of craniofacial malformations with ultrasonography. Am J Obstet Gynecol 1986;155:45-50 7 Brock DJ. Early diagnosis of fetal defects. Curr Rev Obs & Gynec 1982;2:149-56 8 Pearce JM, Campbell S. The use of ultrasound in the diagnosis of fetal abnormalities. Progress Obs & Gynaec 1984;4:52-81
(Accepted 11 September 1991)
Simultaneous occurrence of mucopolysaccharide type II disease (Hunter's syndrome) and systemic lupus erythematosus in a paediatric patient
Paper presented to Section of Paediatrics, 22 February 1991
A R Bedford Russell BSc MRCP M D Bain MB ChB MRCP1 R S Periera MB ChB MRCPath2 Departments of 1Child Health and 2Immunology, St George's Hospital Medical School, Cranmer Terrace, London SWI 7 ORE Keywords: mucopolysaccharide type I disease; Hunter's syndrome; systemic lupus erythematosus
Two rare disorders were simultaneously diagnosed in a 3-year-old Asian boy: mucopolysaccharide type II disease (MPSII) and systemic lupus erythematosus (SLE). A 30-yearold male has been described where these two conditions have co-existed1, but this report is the first in a child.
Case report The 3-year-old boy presented with painful abdominal distension and a skin rash since infancy, which had become worse in recent months. Two months previously he had developed swollen eyes, painful swollen joints, and an intermittent fever. His condition had improved on two occasions, when he had been treated with oral corticosteroids. At presentation he was receiving a reducing dose of betamethasone. The parents were non-consanguineous and from the Indian subcontinent. He was their first and only child and there was no family history of connective tissue or mucopolysaccharide disorder.
Figure 1. Coarse facies with malar rash
The clinical findings are demonstrated in Figures 1 and 2. The skin rash included elements of purpura and urticaria on his lower legs. The pertinent results of his investigations were: haemoglobin 9.3 g/dl; erythrocyte sedimentation rate 121 mmn/hr; urea 5.6 mmol/l; creatinine 44 mmol/l; albumin 28 gll; nuclei anti-nuclear antibody (ANA), positive; ANA (Hep-2-cells), positive; crithidia immunofluorescence (IF), strongly positive; rheumatoid factor positive at 1: 160;
0141-0768/92/ 020109-02/$02.00/0 O 1992 The Royal Society of Medicine