Postgrad Med J: first published as 10.1136/pgmj.46.539.578 on 1 September 1970. Downloaded from

578 Case reports high triglyceride and cholesterol levels some months oral contraceptives preciptitate pancreatitis in after the attacks in the absence of overt diabetes patients with normal serum lipids, it is possible suggests a primary hyperlipoproteinaemia rather that pancreatitis may be a real hazard in patients than a pancreatitis or hormone-induced abnormality. with a pre-existing hyperlipaemia. The mechanism of development of pancreatitis in hyperlipaemic states is obscure, the most favoured explanation being vascular sludging due to the Acknowledgments It is that We would like to thank Drs A. Swanepoel and B. Dreyer chylomicronaemia. possible oral contra- for permission to publish and Dr L. Krut and Dr S. Truswell ceptives, which have been claimed to increase the for carrying out the lipid examinations. This study forms tendency to vascular thrombosis and also to elevate part of a grant from the South African Council for Scientific the serum levels of certain blood clotting factors, and Industrial Research. may set the foundation for enhanced chylomicron aggregation and clustering. Alternatively the hor- References monal effect of the Pill may aggravate one or more FREDRICKSON, D.S., LEVY, R.I. & LEES, R.S. (1967) Fat of the mechanisms suggested for the development Transport in Lipoproteins-an integrated approach to of mechanisms and disorders. New England Journal of secondary pancreatitis-induced hyperlipaemia, Medicine, 276, 148. viz., acute diabetes, the release of triglycerides GLUECK, C.J., BROWN, W.V., LEVY, R.l., GRETEN, H. & from areas offat necrosis, pancreatic c cell damage, FREDRICKSON, D.S. (1969) Amelioration of hyperlri- increased release of glycerides from the liver into glyceridaemia by progestational drugs in familial Type-V the plasma, defective intravascular of hyperlipoproteinaemia. Lancet, i, 1290. clearing GREENBERGER, N.J., HATCH, F.T., DRUMMEY, G.D. & glycerides due to lipoprotein lipase inhibition or ISSELBACHER, K.J. (1966) Pancreatitis and Hyperlipaemia: indeed, the aggravation of the pre-existing defect A study of serum lipid alterations in 25 patients with in lipid metabolism (Marks, Bank & Louw, 1968). acute pancreatitis. Medicine, 45, 161. Fredrickson has found that oestrogens tend to raise MARKS, I.N., BANK, S. & Louw, J.H. (1968) The Diagnosis and to serum and Management of Pancreatitis. Progress in Gastro- progesterone reduce glyceride levels enterology, vol. 1. p. 412. Grune & Stratton, New York (Glueck et al., 1969). and London. The findings in the cases represented in the POULSEN, H.M. (1950) Familial lipaemia: A new form copyright. present paper suggest that pancreatitis be considered of lipoidosis showing increase in neutral fats combined in on oral who with attacks of acute pancreatitis. Acta Medica Scandi- patients contraceptives develop navica, 138, 413. abdominal pain. While there is no evidence that WANG, C., ADLERSBERG, D. & FELDMAN, E.B. (1959) Serum Lipids in Acute Pancreatitis. Gastroenterology, 36, 832.

Complete agenesis of the lung http://pmj.bmj.com/ H. A. SAMAAN F.R.C.S., F.R.C.S.E. Department of Thoracic Surgery, The Royal Infirmary, Edinburgh on September 29, 2021 by guest. Protected SCHNEIDER (1909-1913) divided pulmonary agenesis diagnosis is usually made accidentally in asympto- into three main degrees: matic patients by X-ray or by physical signs of (a) True agenesis-a group in which there is . Recognition of the true nature of complete absence of bronchi, alveolar tissue and the lesion is important, so that unnecessary inter- their blood supply. ference is avoided. (b) A group in which a rudimentary Cases of isolated pulmonary agenesis are rela- arose from the with no pulmonary tissue tively rare as the condition is more commonly investing its tip. associated with serious malformations of other (c) A group with a poorly developed main organs that do not permit prolonged life. Oyamada, bronchus invested by a fleshy mass of ill-developed Gasul & Holinger (1953) found a high association pulmonary tissue. between pulmonary agenesis and anomalies of musculo-skeletal, cardio-vascular, gastro-intestinal Agenesis may be unilateral or bilateral, involve and urogenital systems. and angio- the whole lung, or be lobar or segmental. The graphy may be required to establish the diagnosis. Postgrad Med J: first published as 10.1136/pgmj.46.539.578 on 1 September 1970. Downloaded from

Case reports 579

FIG. 1. Case I Postero-anterior film of (a) plain X-ray chest. (b) Lateral bronchogram. copyright. Case 1 num was displaced to the right. The heart was A male patient aged 35 years with four healthy posteriorly placed. Diaphragmatic movement was children. No history of congenital abnormalities normal. The cause of was not apparent. could be obtained in his family. At no right main bronchus was When he was a schoolboy he suffered from found and bronchography did not outline any of occasional bronchitis and his family doctor men- the right bronchial tree. tioned to his parents that his heart was beating on the wrong side. Because of the finding of dextro- A ventricular : right angiogram did http://pmj.bmj.com/ cardia he was not accepted for military service. not outline a right pulmonary artery. There was no In July 1958 when he was under treatment for abnormality of the cardiac chambers. The excretion aspirin poisoning, the cardiac impulse was observed urogram showed that the left kidney was in the to be on the right side of the chest. His only symptom normal position and the right was displaced, lying was mild exertional dyspnoea. Because of this and vertically and lateral to the left border of the mid- the abnormal radiographic appearance he was lumbar spine. referred for examination. No further investigation was undertaken and no

treatment was advised. on September 29, 2021 by guest. Protected On examination: He was obese but otherwise healthy-looking, 196 pounds in weight, not cyanosed or dyspnoeic. Pulse 70 per minute, regular. Blood Case 2 pressure 130/90 mmHg. The trachea was deviated An asymptomatic girl aged 12 years. She had a to the right, but expansion of the chest was equal normal birth but her mother suffered from hypere- on both sides. FEV1 was 1-8 litres and the FVC mesis. The child was first examined when she was 2-85 litres. The heart sounds were normal and were 9 months old because of episodes of bronchitis best heard on the right fourth intercostal space with tracheal rattles and an abnormal radiograph. one inch from the right sternal border. The electro- Radiographs of the chest showed that the heart cardiogram showed sinus rhythm. The left ventri- occupied the posterior part of the right hemithorax. cular complexes from V1 to V6 indicated right Bronchoscopy and bronchography confirmed the sided displacement of the heart. absence of a right bronchial tree. Radiographic examination showed that the right At angiography, no right pulmonary artery was hemithorax was smaller than the left. The mediasti- opacified. Postgrad Med J: first published as 10.1136/pgmj.46.539.578 on 1 September 1970. Downloaded from

580 Case reports When she was re-examined at the age of 12 she Abnormalities of pulmonary vessels may be was free from respiratory complaints. She had a associated with pulmonary agenesis, (Ferencz, 1961). slight scoliosis of the spine and corrective exercises Vascular compression of the trachea or bronchus were advised. detween a posteriorly situated pulmonary artery and anteriorly situated aorta has been reported (Maier & Gauld, 1953). Stenosis of the trachea caused either by compres- sion or rarely by intrinsic congenital stenosismaylead to episodes of hypoxia and greatly raised pulmonary arterial blood pressure (Killingsworth & Hibbs, 1939; Nelson et al., 1967). This in turn may cause reversal of blood flow through a persistent ductus arteriosus necessitating urgent operative closure. Other vascular anomalies include , absent pulmonary arteries to both lungs and intra-cardiac septal defects. It has been reported that in twins (Finkelstein, 1912, Yount, 1948) the agenesis may be on opposite sides. Complete unilateral pulmonary agenesis was stated by Smart (1946) to occur once in 15000 autopsies. The majority ofcases have been discovered at necropsy in infants. In the absence of other serious defects, the condi- tion is compatible with prolonged survival. Heerup

(1927) described a patient who lived till the age of 72 copyright. and Liidin & Wertheman (1939) another who lived to 58. The single lung is much larger than normal. FIG. 2. Case II. Postero-anterior plain X-ray of chest. It fills the space that should be occupied by the absent lung and leads to displacement of the mediastinal Discussion contents away from itself. Bilateral The vital capacity may be within the normal complete pulmonary agenesis was Smart recorded as three clinical reported by Schmit (1893) in a foetus born after 8 range. import, signs: months' 1. Symmetrical chest with almost equal expansion http://pmj.bmj.com/ . Chaireaux & Ferreira (1958) of both sides. described a full-term male infant whose trachea ended 2. Gross of trachea to blindly. The pulmonary artery joined the aorta displacement one side. below the left subclavian artery (persistent left 3. Rotation of whole . sixth aortic arch). The infant lived 15 min. No Spirometric tests show that the enlargement of the is due to pulmonary veins or bronchial arteries were present single lung and not to and there was a left superior vena cava which entered degenerative emphysematous changes. the left atrium. & Gardner Minor congenital abnormalities may be present Tuynman (1952) des- on September 29, 2021 by guest. Protected cribed a similar case in which development had in the other lung, in the chest wall and diaphragm. proceeded to the stage when primitive bronchial The factors responsible for pulmonary agenesis buds were present (26 day stage). The pulmonary are not fully understood. artery joined the thoracic aorta at the point where The following have been recognized: the ductus arteriosus normally joins the pulmonary (a) Genetic (Neill, Ferencz, Sabiston & Sheldon, artery. 1960) reported the case of a father and daughter with Morgagni (1762) was the first to describe pul- right pulmonary agenesis. Finkelstein (1912), Yount monary agenesis. Unilateral agenesis has been (1948) recorded agenesis in twins. reviewed by Smart (1946), Burger (1947), Wexels (b) Injury, threatened abortion and bacterial (1951), Jimenez-Martinez et al. (1965) and Nelson, infection during first three months of McMillan & Bharucha (1967). (Field, 1946). Absence of the right lung is less common than (c) Viral infection following rubella in pregnancy absence of the left (70%). Males are more affected (Campbell, 1961, Butler, et al., 1965; Forrester, than females (Lucas, Dotter & Steinberg, 1953). Lees & Watson, 1966). Postgrad Med J: first published as 10.1136/pgmj.46.539.578 on 1 September 1970. Downloaded from

Case reports 581 (d) Diet deficiency in Vitamin A during pregnancy Agenesis of the lungs with patent ductus arteriosus in rats & 1949. treated surgically. Journal of Thoracic and Cardiovascular experimental (Wilson Warkany, Surgery, 50, 59. The prognosis in congenital absence of one lung KILLINGSWORTH, W. & HIBBS, W.G. (1939) Agenesis of the depends on the presence or absence of other associ- lung. American Journal of in Children, 58, 571. ated congenital abnormalities, together with in- LUCAS, D.S., DOTTER, C.T. & STEINBERG, I. (1953) Agenesis creased to infections. of the lung and patient ductus arteriosus with reversal of vulnerability pulmonary flow. New England Journal of Medicine, 249, 107. I-IDIN, M. & WERTHEMANN, A. (1930) Lungenveranderungen Acknowledgments nach experimenteller Rontgenbestrahlung. Strahlenthera- I am grateful to Mr A. Logan, Consultant Thoracic pie, 38, 684. Surgeon, The Royal Infirmary, Edinburgh, for permission and MAIER, H.C. & GAULD, W.J. (1953) Agenesis of the lung with assistance to publish details of these two patients who were vascular compression of tracheo-bronchial tree. Journal under his care in the Thoracic Unit. of Paediatrics, 43, 39. MORGAGNI, J.B. (1769) The seats and causes of diseases investigated by anatomy (Translated by B. Alexander) A. References Miller, London. BURGER, R.A. (1947) Agenesis of the lung. American Journal NEILL, C.A., FERENCZ, C., SABISTON, D.C. & SHELDON, H. of Diseases of Children, 73, 481. (1960) The familial occurrence of hypoplastic right lung BUTLER, N.R., DUDGEON, G.A., HAYES, K., PECKHAM, C.S. with systemic arterial supply and venous drainage. & WYBAR, K. (1965) Persistence of rubella antibody with Bulletin of Johns Hopkins Hospital, 107, 1. and without embryopathy. British Medical Journal, 2, NELSON, C.S., MCMILLAN, I.K.R. & BHARUCHA, P.K. 1027. (1967) Tracheal stenosis, pulmonary agenesis and patent CAMPBELL, M. (1961) Place of maternal rubella in the ductus ateriosus. Thorax, 22, 7. aetiology of congenital heart . British Medical OYAMADA, A., GASUL, B.M. & HOLINGER, R.H. (1953) Journal, 1, 691. Agenesis of the lung. American Journal of Diseases of CHAIREAUX, A.E. & FERREIRA, H.P. (1958) Bilateral pul- Children, 85, 182. monary agenesis. Archives ofDisease in Childhood. 33, 364. SCHMIT, H. (1893) Einfall von vollstandiger agenesiebeider FERENCZ, C. (1961) Congenital abnormalities of pulmonary Lungen. Virchow's Archives, 134, 25. vessels and their relation to malformation of the lung. SCHNEIDER, P. & SCHWALBE, E. (1913) Die morphologie der Pediatrics, 28, 193. missblidungen des menschen und der Tiere, 3, 763. FIELD, C.E. (1946) Pulmonary agenesis and hypoplasia. Fisher, Jena. Archives oJ Disease in Childhood, 21, 61. copyright. FINKELSTEIN, H. (1912) Hypoplasie und agenesie der lunge. SMART, J. (1946) Complete congenital agenesis of lung. In Lehrbuch der Sauglings-Krankheiter. Fischer's medizin, Quarterly Journal of Medicine, 15, 125. Berlin. TUYNMAN, P.E. & GARDNER, L.W. (1952) Bilateral aplasia FORRESTER, R.M., LEES, V.T. & WATSON, G.H. (1966) of the lung. Archives of Pathology (Chicago), 54, 306. Rubella syndrome: escape of a twin. British Medical WEXELS, P. (1951) Agenesis of the lung. Thorax, 6, 171. Journal, 1, 1402. WILSON, J.G. & WARKANY, J. (1949) Aortic arch and cardiac HEERUP, L. (1927) Et Tibfalde af venstresidig Lungemangel. anomalies in the offspring of vitamin A deficient rats. Hospitalstidende, 70, 1165. American Journal of Anatomy, 85, 113. JIMENEZ-MARTINEZ, M., PEREZ-ALVAREZ, J.J., PEREZ-TRE- YOUNT, F. (1948) Agenesis of right lung in each of identical VINO, C., RUBIO-ALVAREZ, V. & DE RUBENS, J. (1965) twins. Arizona Medicine, 5, 48. http://pmj.bmj.com/ Congenital abdominal coarctation with renal artery hypoplasia, hypertension and rheumatoid arthritis N. NAGARATNAM* W. M. T. WEERASINGHE

M.D., M.R.C.P.(Glasg) M.B.B.S.(Cey) on September 29, 2021 by guest. Protected Physician House Physician Government Hospital, Kegalle, Ceylon STENOSING LESIONS of the abdominal aorta are rare. This paper reports a case of congenital abdominal They are of varied aetiology, and may be due to coarctation with renal artery hypoplasia and hyper- congenital anomalies, atherosclerosis, dissecting tension associated with rheumatoid arthritis in a aneurysm and acquired lesions of the inflammatory girl of 13 years. type ofspecific and of non-specific origin. Abdominal coarctation can occur above or below the level of Case report the renal arteries. If the renal arteries are involved A girl, aged 13 years was admitted on 17 April a syndrome of Goldblatt hypertension is produced. 1967 with a history of swelling of her joints of 3 *Present Address: Government Hospital, Gampaha, years' duration, headache and effort dyspnoea of Ceylon 1 years' duration.