Lymphoedema in hereditary recurrent cholestasis from birth

402

ABsTRAcrs

over an 11 year period and a review of the literature, summarizes the clinical features of chylous ascites as it occurs in infants and children. Symptoms are those of increased intra-abdominal pressure. Diagnostic paracentesis usually yields a milky

a 3 hour period. was then inserted

fluid containing approximately 3% protein. When the etiology is undetermined, chylous

The sump-Penrose drain or (triple lumen drain) consists of a sump tube threaded inside a regular Penrose drain. The Penrose

ascites

is

usually

a

self-limited

disease

requiring only symptomatic and supportive therapy. Parenteral alimentation, intravenous infusion of as&c fluid, and internal drainage by venoperitoneal shunts have been used with indifferent results. Dietary regulation (particularly short and medium-chain triglyceride as fat source) reduces chyle formation and helps to control ascites. Surgical treatment is indicated for surgically correctable lesions, for persistent ascites not responding to low fat diet, and when progressive deterioration occurs with chylous ascites of undetermined etiology. Trauma, mechanical intestinal obstruction, mesenteric defects, umbilical venous remnants with mesenteric compression, chylous cysts, diffuse lymphangioma, and mesenteric adenitis are lesions associated with chylous ascites that had been diagnosed and treated surgically with subsequent relief of the chylous ascites. The high incidence of gastroenteritis preceding

the

onset

of chylous

ascites

(three

The drain under study into four groups of dogs

(five dogs in each group). A Penrose drain, a rubber catheter (No. 20 French), a No. 20 French sump tube, and a sump-Penrose drain combination were used in this study.

drain is perforated on both sides and its distal end is tied around the shaft of the sump tube with a ligature proximal to the air vent. Intermittent suction was applied to all but the Penrose drain The tubes were not irrigated, Collection of the drained fluid was continued for 3 additional hours after termination of the intraperitoneal infusion. At the conclusion, the peritoneal cavity was reopened and the abdominal viscera were inspected for the degree and extent of the staining by the methylene blue. Although the Penrose drain has been the mostly commonly used drain for the peritoneal cavity, it was found to be the least efficient. The Penrose drain was found to drain an average of 40% of the intra-peritoneal fluid, the rubber catheter drained 39% the sump tube drained 58% and the new sump-Penrose drain ,removed 72% of the fluid. The author believes the latter method is more efficient. During the past 4 years

of seven patients) and the occurrence of mental aberrations (three of seven patients) has not been previously reported.-W. K.

he has used this method is 157 patients following various abdominal operations. He finds that the abdominal incision remains

%ber.

dry and frequent dressing nated--G. Holcomb, Jr.

EFFICIENCYOF PERITONEALDRAINAGE. E. A. Hanna. Surg. Gynec. Obstet. I31:983-985

( November ) 1970. The author measured the efficiency of four methods of intraperitoneal drainage in the laboratory under controlled experimental conditions, and reports on the use of one of these in I57 patients. Twenty mongrel dogs were selected. Each dog underwent a laparotomy and a No. 15 plastic cannula was introduced into the peritoneal cavity through a stab wound in the left subcostal region, Its distal end was positioned in the left subhepatic space, anterior to the gastrohepatic ligaments, with the tip at the anterior border of the gallbladder. Through this cannula, 250 cc of normal saline plus 5 cc of methylene blue were introduced at a constant rate during

changes

are elimi-

ADRENAL INJURY DURING REPAIR OF DIAPHRACMATICHERNIA IN INFANTS. R. C. hf. Cook and J. B. Beckwith. Surgery 69:251255 (February) 1971. Fifteen of seventeen cases of neonatal diaphragmatic hernia who came to autopsy after operation had extensive adrenal hemorrhage. The authors suggest that such damage may have contributed to the death of these infants and urge great care while working in the region of the adrenal glands in the newborn.-W. K. Sieber. LYMPHOEDEMA IN HEREDITARY RECURRENT CHOLESTASISFROMBIRTH.0. Aegenaes and H. Sigstad. Arch. Dis. Child. 45:690, 1970. This

further

report

from

the

Norwegian

403

ABSTRACK4

authors gives the second report on the syndrome characterized by lymphoedema and recurrent attacks of cholestasis The detailed family tree is given. Three adults and three children are reported. Lymphangiography drome characterized by lymphoedema and abnormal Iymphatic drainage is discussed. The intra-abdominal lymphatics appear to be normal as is the thoracic duct. Liver biopsy has been performed in infancy but does not show a specific change in the liver. The predominant changes are of intrahepatic cholestasis and some giant cell formation. D. G. Young. ACUTE HYDROPS OF THE GALLBLADDER IN CHILVHOOD..l. W. Chamberlain and D. W. Might. Surgery 68:899-905 (November)

1970. This article describes acute noncalculous, noninflammatory distension of the gallbladder in child,ren, reporting four cases and reviewing 25 additional cases from the literature. The cause of the distension of the gallbladder is unknown but it is not due to intrinsic inflammatory process, calculi, or congenital anomalies. The four reported cases include an 11 month old, a 4 month old, a 3 year old, and a 10 year old. Three were boys and one a girl. Treatment included cholecystectomy in one and cholecystostomy in the other three patients. Those treated by cholecystostomy were free of symptoms and had normal gallbladder function 10 years, 4 years, and 4 years, following the operative procedure. Reviewing the literature, a correct diagnosis was only made three times, the most common preoperative diagnosis being intussusception and appendiceal abscess. Abdominal pain was present in all patients, vomiting in 27 of 29 (90%), right upper quadrant tenderness in 28 of 29 (93%), and a palpable right upper quadrant mass in 16 of 29 ( 55%). There were 20 boys and 8 girls in this group of patients (in one the sex of the patient was not recorded). Treatment consisted of simple aspiration, cholecystostomy, or cholecystectomy. Postoperative course in all 29 patients was uncomplicated with no evidence of recurrence in any. The authors conclude that acute hydrops of the gallbladder in children is a distinct entity and the simplest and safest procedure

for its correction Sieber.

is cholecystostomy.-W.

K.

PRIMARY EXCISION OF CHOLEDOCHALCYSTS. M. Ishida, Y. Tsuchidu, S. Saito, and T. Tori. Surgery 68:884-888 ( November)

1970. This report from the University of Tokyo presents data concerning 31 patients with choledochal cysts treated by surgical procedures during the period 1953 to 1969. The average age at surgery was 3 years (22 days to 13 years). In 19 patients the cyst was excised, the distal choledochus ligated and the extrahepatic biliary tract reconstructed by anastomosing the common hepatic duct to a Roux-en-Y placed segment of jejunum which averaged 30 cm in length. Sixteen patients are living. Three died, one early due to shock and two later due to stricture of the anastomosis in one and irreversible hepatic cirrhosis in the other. Fourteen of the 16 survivors are completely well and asymptomatic. Nine patients had Roux-en-Y cystojejunostomy. Five are now living. Four died, one early of shock and three later of cholangitis. Two patients had choledochocystoduodenostomy and both are living. They have occasional fever and abdominal pain. Surviving patients have been followed an average of 5 years and 7 months. Liver function tests were performed in 19 surviving patients and were abnormal in eight of these. The authors recommend primary excision as the operation of choice in the treatment of congenital choledochal cyst.-W. K. Sieber. SPONTANEOUSPERFORATIONS OF THE BILIARY TRACT IN INFANCY. J. Prevot and J. M. Babut. Prog. Pediat. Surg. 1:187, 208,

1970. Biliary peritonitis is a rare but serious condition which was first described in 1932. A new case presenting at 25 months of age and 50 cases found in the literature are analyzed. Bile may fill the whole peritoneal cavity or be loculated into pseudocysts. Perforation of the biliary tract was found in 41 cases. Localized stenosis or dilation of the bile duct was present in five cases and calculi were found in four. The etiological