LYMPHATIC CYST OF THE EAR

1281

CLINICAL AND LABORATORY NOTES CONTINUOUS VENOUS HUM IN BILHARZIAL CIRRHOSIS OF THE LIVER BY M. R. KENAWY, M.D. Cairo REGISTRAR TO THE MEDICAL UNIT OF KASR-EL-AINI HOSPITAL, CAIRO

UNIVERSITY,

EGYPT

CIRRHOSIS of the liver is endemic in Egypt among the cultivator class. It is caused by infestation with bilharzia ova, usually of the mansoni type, and their deposition in the liver. In the last two years I have been able to collect 6 cases of bilharzial cirrhosis, with or without splenomegaly, in which a continuous venous hum could be easily heard over a localised The murmur has area over the xiphoid process. the following characters : (a) it is a continuous hum which increases in loudness during inspiration and decreases during expiration ; (b) it is louder in the sitting or standing position than while the patient is recumbent ; (c) it is localised and not propagated and not heard at the back ; and (d) it is not associated with any cardiovascular abnormality. As there has been much controversy about the source of such a hum, I thought the following record might be helpful :

inspiration and its greater loudness in the standing or sitting position, where the action of the diaphragm_ is more free and thus allows a greater rise of intraabdominal pressure, afford further corroborative evidence. Its disappearance after splenectomy as well as its complete absence on auscultation of the back excludes the possibility of stenosis of the vena cava by " a perivenous hepatic fibrosis " such as Dr. J. L. Bates suggested in THE LANCET of May 8th

(p. 1108). CONCLUSIONS

(1) The continuous venous hum previously recorded in Hanot’s cirrhosis and Banti’s disease also. occurs in endemic (bilharzial) cirrhosis of the liver in Egypt, with or without splenomegaly. (2) Six cases have been collected in the last two years. In one of them the hum disappeared after splenectomy. (3) Its disappearance was probably due to removal of some venous communication during the operation. My thanks are due to Prof. H. B. and encouragement.

BY MIN SEIN, M.B.

male, aged 20, was admitted to hospital on July 29th, 1936, complaining of an abdominal swelling for a year.

experience in the other 5 cases there is relationship between the hum and the presence of ascites, nor had specific treatment with antimony (tartar emetic) any effect on the hum. The degree of anaemia and its further improvement under treatFrom my

no

ment also had no effect. In the case recorded above the hum disappeared entirely after splenectomy. This suggests that during the removal of the spleen the source of the hum was also removed. Since the hum occurs in cases of cirrhosis of the liver without splenomegaly, it could not be assumed that the spleen was the actual source, and most probably it arose in some venous communication which was severed during the operative manipulations. The increased loudness of the murmur during

Kasper, M. (1932) Zbl. Bakt. 126, 252. Long, E. R. (1935) J. Amer. med. Ass. 104, 1883. Pagel, W. (1930) Beitr. Klin. Tuberk. 76, 414. —

(1936) J. Path. Bact. 42, 417.

Roulet, F. (1936) Acta Davos, 1, 1. Sata, A. (1899) Beitr. Path. Anat. Suppl. Weigert, C. (1886) Virchows Arch. 104, 31.

III.

for kind advice

LYMPHATIC CYST OF THE EAR

A

He gave a history of diarrhoea and terminal hsematuria two years before. He was pale and was stunted in growth. The abdomen looked distended ; the liver was enlarged three fingers-breadth in the mammary line and hard in consistence ; the spleen was enlarged down to left iliac fossa. Nothing abnormal was detected in heart or lungs, and there was no free fluid in the abdominal cavity. Over the xiphoid process a continuous venous hum with the characters described above could be heard. The stools contained Bilharzia mansoni ova; the urine contained albumin and blood. Renal function normal. Wassermann reaction negative. A blood count showed : hemoglobin 50 per cent., red cells 3,800,000, and white cells 3200 (polymorphs 75 per cent., lymphocytes 25 per cent.). Blood pressure 100/65. Sigmoidoscopic examination showed a slight granularity of the mucous membrane and nothing else abnormal. The patient was treated with iron and a full course of tartar emetic. On Sept. 23rd Immediately after the splenectomy was performed. operation and on the following days the hum had disappeared. He was discharged on Oct. 19th.

Day

CAPTAIN,

Calcutta, M.R.C.P. Lond.

INDIAN MEDICAL

SERVICE, BURMA

I HAVE not been able to find any published account of the condition described in this note, which I have named " lymphatic cyst of the ear." I believe however that it is not uncommon. Clinical features.-A painless, tense and cystic swelling, translucent on illumination, appears in the concha on the lateral aspect of one or both ears. Its gress is extremely slow, and it may not be noticed for a long time. When well developed it may completely fill the

pro-

concha (see Figure). The pinna is not thickened and the auricular glands are not enlarged. The six patients I have seen have all been adult males living in Burma. E’(o.—There is no evidence of leprosy or filariasis. On aspiration of the cyst a thick straw-coloured fluid is obtained. Hamilton Dr. N. Fairley,

enough to examine the nmd from one them, reported that it contained no micro-organisms or cells and expressed the opinion that it was from a simple cyst. Evidently there is lymphatic obstruction of some sort and it is noteworthy that 5 of the 6 patients wore spectacles with curved aural supports which pressed against the angle formed by the ear and the skull. The spectacles had been worn for periods varying from 2 to 27 years. The sixth patient had no spectacles but wore a Burmese headdress called gaungbaung which consists of a fine silk scarf worn tightly round the head along a line passing across the forehead, just above the ears and downwards and backwards over the occiput. All these patients had seborrhoea, and often the irritation of the aural supports produced crops of acne and boils behind the ear. It is possible that the pressure exerted by the aural supports and the gaungbaung interfered with the lymphatic drainage of the ear resulting in who was kind

of

°

1282

cyst-formation in the and the repeated subminimal infections may also have favoured the onset and progress of the cysts. The Wassermann reaction was done in 2 cases and found to be negative. Treatment.-The pressure behind the ear must be relieved and spectacles changed and fitted properly. The local treatment consists in evacuating the cyst through a medium-sized hypodermic needle and injecting a sclerosing solution through the same needle. The cyst is emptied by applying pressure from the periphery towards the needle. The presence of blood indicates that too much pressure has been applied. Quinine urethane solution (Parke, Davis and Co.) was used but other sclerosing fluids would Pressure is applied for a few minutes, and a serve. pressure bandage could be applied for a few hours if blood appeared during the evacuation. The condition may recur but can be treated in the same way again if need be. My six patients have had no recurrence in the 2-7 years that have elapsed since the last treatment. intermittent obstruction and area

drained,

side of the face limited below by the lip margin and a line from the angle of the mouth to the malar bone. Above the orbit, the nsevus extends to the hair margin of the forehead and on to the hair-bearing area of the temporal region. There is no extension across the middle line of the face or scalp and the cartilaginous portion of the nose is not involved. On the buccal aspect the

SUMMARY

cystic swelling developed in the concha of the of 5 persons who wore spectacles with curved aural supports. A similar condition developed in a patient who had pressure of a different kind applied round his head. The cyst is believed to be the result of lymphatic obstruction. Treatment by evacuation and injection of sclerosing fluid gives good cosmetic A

ears

results. A CASE OF NÆVOID AMENTIA BY RALPH BATES, F.R.C.S. MEDICAL

Eng., D.P.M.

OFFICER, STOKE PARK COLONY, STAPLETON, BRISTOL

N aeVOID amentia is rare enough to justify the short of a characteristic case. Tredgold1 summarises the syndrome as a combination of nsevoid growths of the skin and meninges, mental defect

description

(usually

idiocy

1m DeCIlll:iY),

or

nemi-

and epileptifits. The frequency of the condition is given as about 1 in 800 mental defectives, but the case described below is the only one discovered in an examination of more than 2000 defectives. Vascular naevi are discussed in greatest detail in the standard textbooks on skin diseases, but these do not usually mention the syndrome nor do they call to attention

plegia,

form

FIG. l.-Photograph showing extent of the nsevns.

ULLV v

WOOV’l,).LWU.LVLL

between vascular naevi of the skin and vascular abnormalities in the meninges. A feeble-minded woman, aged 36, has been under observation at Stoke Park Colony for the past five years. As Fig. 1 shows, there is an extensive nsevus of the right

1 Tredgold,

A. F.

(1929) Mental Deficiency,

London.

FIG.

2.-Radiogram of skull showing calcification in frontal region.

covers the right cheek and extends on to the but is again limited by the midline. palate, The lower limit of the nsevus corresponds with the lower limit of the embryological maxillary process. The area covered by the nsevus corresponds to that part of the face which is formed by the maxillary, lateral nasal, and median nasal processes, while the nasal part of the frontonasal process is normal. The exception to an otherwise exact correlation is the fact that the ala nasi (which is formed from the lateral nasal process) is covered with normal skin. The islands of nsevoid tissue below the main nsevus are good examples of Virchow’s " fissural angiomata" situated at the junction of maxillary and mandibular processes. The conjunctival vessels of the right eye are dilated and tortuous. Examination of the left fundus shows a normal disc with rather full veins. The right fundus shows a very deep physiological cup (-7D) with the fundus raised around it. The vessels at the cup edge can be focused with - 3D and those in the periphery with -1.5D, The veins are very tortuous and dilated but diminished in number compared to the left side and the arteries are small. Examination of the limbs shows a difference in development on the two sides. The left hand is smaller than the right and the left upper limb is shorter by 1 in. The left lower limb is an inch shorter than the right. Both upper and lower limbs show muscular weakness and increased tendon reflexes on the left side. There is slight rigidity of the left lower limb, but the plantar reflex gives a flexor response. There is no alteration in sensation. An X ray photograph of the skull (Fig. 2) shows irregular areas of calcification in the frontal region compatible with calcification in a plexiform angioma. na3vus

This patient has suffered from epileptic fits since coming under observation. Before the attack she complains of pins and needles " in the left upper limb, "

and sometimes that the left upper and lower limbs " feel stiff." On regaining consciousness she feels " as if she has no strength " and the left upper and lower limbs " feel stiff " for a short time. Under ordinary epileptic treatment major fits now occur about once in six months.

My thanks are due to Dr. R. J. A. Berry, director of medical services at the Stoke Park Colony, for permission to publish this case.