Hewitson, R.P.

Rosenberg, A.B.

English

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12 lovember 1966 S.A. MEDICAL JOUR TAL 100LDOUBLE AORTIC ARCH IN AN INFANT OF THREE MONTHSA. B. ROSENBERG, B.sc. (STELL.), M.B., CH.B. (CAPE Tow), Senior Medical Officer, Department of Paediatrics,SomerseT Hospital .. A ID R. P. HEWITSON, F.R.C.S. (ENG.), F.R.C.S. (Em .), Thoracic Surgeon. SomerseT and RedCross War Memorial Children's HospitalsOf the various causes of respiratory difficulty in infancy,one of the less common, but nevertheLess remediable, istracheal obstruction due to abnormalities of the greatvessels. The following case illustrates some of the aspectsof this possibility that are worth bearing in mind whendealing with any child who wheezes.CASE REPORTA Coloured infant, aged 3 months, was admitted to theSomerset Hospital on 17 September 1965, because of ahistory of coughing for 5 days and for gastroenteritis.Past HistoryThe birth was a full-term normal delivery to a motherwith 4 children, who are all well. The child was born athome, and was given BCG at the regional clinic. Beforethis present complaint, there had been no complaints andthe child took all its feeds well, with normal bowel action.General ExaminationThe child was clinically anaemic, tachypnoeic, andweighed 8 lb. 11 oz. on admission. The pulse rate was1l0/min. and temperature (rectal) 99·4°P. Examination ofthe respiratory system showed a central trachea, good airentry in both lungs, despite diffusely scattered crepitationsand a fair amount of bronchospasm.All other systems appeared to be norma!. Haemoglobin9·5 G /100 m!., white cell count 11,900/cu.mm. X-ray ofthe chest at this stage showed partial pneumonic consolida-tion in both right upper and left lower lobes. There wasalso patchy consolidation in the right lower lobe. Treat-ment included a broad-spectrum antibiotic and a broncho-dilator.Investigations done included a Heaf test, PPD andMantoux test, which were all negative. Culture of thethroat swab showed a good growth of Staphylococcusaureus sensitive to the tetracyline that was being given.Gastric washings, examined for acid-fast bacilli, werenegative, and so was the sweat test.During the first week there was clinical improvementand this was confirmed radiologically, where the consoli-dation had largely cleared, leaving some paracardiacopacification on the right side. Weight gain during theensuing month was slow, and from time to time it wasnecessary to administer adrenaline intradermally, forsevere bronchospasm, audible for quite a distance from theside of the cot.With the slow clinical improvement, the right hilar areaand paracardiac region remained opacified on serial X-raysof the chest. At this stage it was decided to change theantibiotic in an effort to improve the condition. On 2November 1965 the radiologist reported the right hilardensity to be better defined, and to have a lobulatedappearance, in keeping with a gland which was com-pressing the right main bronchus at its origin.On 20 ovember 1965, after being picked up for feedingby a nurse, his head became acutely flexed forward.resulting in the child becoming cyanosed and momentarilyapnoeic. A day later, this recurred.Fig. I(a)Fig. l(b)Fig. I. Lateral X-ray views of chest (a) showing trachea indentedposteriorly and Cb) oesophagus outlined with barium similarly indented.A barium swallow was ordered in an effort to establishthe cause of these episodes. It showed, as was suspected, aslight narrowing of the oesophagus, in the region of theaortic knuckle, but there was no impediment to the freepassage of the barium. The oesophagus above and belowthis was normal in calibre and appearance, and there wasno displacement.Bronchoscopy was then performed under general anaes-thesia. This showed the lower trachea to be pushed for-ward and indented by a pulsatile swelling. On oesophago-I I1002 S.A. TYDSKRIF VIR GE EESKU TDE 12 ovember 1966scopy, there were suggestion of pulsation, both anteriorlyand posteriorly. A diagnosis of a double aortic arch wasthen made and the child was prepared for thoracotomy.Course and ManagementAt operation on 10 December 1965, a left lateral inter-costal thoracotomy was performed. The descending thora-cic aorta was found to be on the left with a large right-sided arch crossing the midline behind the oesophagus.The left subclavian artery was identified alongside theoesophagus. There was a long vessel forming an anterioror left arch, about 4 mm. in diameter; the anterior con-nection with the ascending aorta was buried under thethymus, but the posterior end joined the posterior archat the level of the left subclavian. This vessel was dividedwith no change in the pulses and the ends retractedreadily. The connective tissue on the left of the tracheaand oesophagus was also loosened. The chest was closedin the usual manner with one underwater-seal drain.Postoperatively the patient made a good recovery. Theconsolidation in the right upper zone gradually diminished.o further cyanotic spells were noted and the infantgained weight rapidly while in hospital. The drainage tubewas removed on the 2nd postoperative day and he wasdischarged on 28 December 1965.DISCUSSIONA complete vascular ring formed by the aorta and itsbranches is not a common occurrence. In 1962, it wasstated that less than 100 had been reported in the litera-ture.' Various combinations are possible; usually there are2 arches of which the left (or anterior) is the smaller; buta ductus or ligamen'um arteriosum may be the factorcompleting a ring and thus producing obstruction.Symptoms usually occur early-within the first fewmonths of life. There is evidence of progressive respira-tory obstruction, with inspiratory stridor and costal reces-sion. The neck may be kept extended in an effort tobreathe. The obstruction is not relieved by bronchodilata-tion. Respiratory infections are common and may occurrepeatedly. There may be increased respiratory difficultyduring feeding, as the oesophagus is enclosed as well asthe trachea, and distension of the former adds to theairway obstruction.The condition should be borne in mind and the diagno-sis usually can be made by contrast studies. The simplestis with contrast medium in the oesophagus, when theindentation is readily noted. Bronchoscopy and broncho-graphy may be performed and if readily available, cine-angiocardiography should demonstrate the anatomicalarrangement. This last is useful if there is any question ofassociated stenosis of one of the main vessels.A left thoracotomy gives adequate access to the vastmajority of these cases when the constricting ring can bedivided without compromising the blood supply to vitalorgans.We wish to thank Dc. R. Nurok, Medical Superintendent ofSomerset Hospital, and Dc. J. F. W. Mostert, Medical Super-intendent of the Red Cross War Memorial Children's Hospital,for permission to publish: Drs. I. Mirvish and G. Sutin, of theDepartment of Paediatrics, Somerset Hospital, for their guid-ance and encouragement; and Dc. C. Cywes, thoracic surgeryunit, Red Cross War Memorial Children's Hospital.REFERE 'CE1. Lindskog. G. E., Liebow. A. A. and Glenn, W. W. L. (1962):Thoracic and Cardiovascular Surgery with Related Pathology, p. 751.New York: Appleton-Century-Croft.MYASTHENIA GRAVIS IN A 2-YEAR-OLD TREATED BY THYMECTOMYG. J. SUTI , M.B., CH.B., M.R.C.P. (Eol .), D.C.H. R.C.P. & S. (E G.), Pari-iime Paediatrician, ID R. P.HEWITSON, ER.C.S., F.R.C.S. (EDIN.), Part-time Thoracic Surgeon, Somerset Hospital and Red Cross WarMemorial Children's HospiTal, Cape TownMyasthenia gravis is a rare illness in childhood. The usualapproach to therapy is conservative and there are only fewinstances documented where thymectomy has been under-takenY This report concerns an infant, who may be theyoungest patient on record, in whom progression of thedisease justified this radical procedure.C SE HISTORYM.P., a Coloured female infant, born on 15 November1963, was fir t seen by one of us (G.J.S.) on 24 September1965. About 3 months before this visit her parents hadnoted drooping of the left upper lid. which they sponta-neously volunteered had become more marked as the dayprogressed. A few days before being seen the right eyehad become similarly involved. She had also shown unduelethargy towards afternoon, and was taking to bed muchearlier than usual. On physical examination she had arather expressionless face, and bilateral ptosis of a markeddegree which she was unable to correct. The knee reflexeswere probably diminished, but there was no other evidenceof neurological deficit. She was admitted to the Somer etHospital where a dramatic response to 0-5 mg. of prostig-mine intramuscularly, confirmed the diagnosis of myas-thenia gravis.A satisfactory response was obtained to prostigmine.7·5 mg. orally twice a day, and she was dicharged on 15October 1965. The effect, however, gradually wore off,and when seen on the morning of 13 December 1965, shewas found to have a partial bilateral external ophthalmo-rlegia. which by afternoon had become total, when seenby Or. L. Schrire.She was admitted to the Red Cross War MemorialChildren's Hospital, Rondebosch, on 15 December 1965.Increased dosage produced a variable and inconstantresponse, and the impression of peripheral muscular in-volvement became more dominant. Thymectomy was per-formed on 29 December (R.P.H.). The thymus removedin toto was macroscopically normal, with no evidence ofneoplasia, lymphoid folliCles or germinal centres foundon histology (Or. C. E. Watson).Following operation, improvement was rapid and pro-gressive. After 48 hours, markedly increased responsive-

Double aortic arch in an infant of three months

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Double aortic arch in an infant of three months

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