Solitary cyst of the falciform ligament of the liver

Solitary Cyst of the Falciform Ligament of the Liver Report of a Case and Review of the Literature WILLIAM H. GONBRING, M.D., Oklahoma City, Oklahoma

From the Department of Surgery, The Univers#y of Oklahoma Medical Center, Oklahoma City, Oklahoma.

OLITARY CYSTS of the falciform ligament of the" liver are rare. T h e r e h a v e been only eight cases recorded in the English literature; the earliest in 1909 by Henderson from the M a y o Clinic [1 ]. T h e falciform ligament contains the ligam e n t u m teres hepatis, the obliterated fetal left umbilical vein. This lies in the free edge of the faleiform ligament and extends from tile umbilicus to the h e p a t a portis where it attaches to the ligamentum venosum between the lobes of the liver. T h e falciform ligament represents a portion of the persistent ventral mesentery and therefore consists of two opposed mesothelial layers containing the round ligament, paraumbilical veins, adipose tissue, and a small collection of both smooth and striated muscle fibers [21. Wakeley and M a c M y n [3] were the earliest to consider the cause of the cyst. h i 1931 t h e y pointed out t h a t a cyst could arise in the faleiform ligament if the ligamentum teres was obliterated partially instead of completely. A retained endothelimn can continue to secrete. Furthermore, cysts are known to be formed from such fetal remains elsewhere as in the round ligmnent of the uterus or the funicular portion.of the processus vaginalis. Lightwood and Campbell [4] in 1939 reported a cyst of the round ligament, discovered at a u t o p s y in a four m o n t h old infant. Karabin [5] in 1981 coneluded t h a t the etiologic factor of a cyst in the faleiform ligament is the failure of the left fetal umbilical vein to obliterate.

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F)26

More recently, Brown [6] has classified tlmse cysts as p r i m a r y and secondary. P r i m a r y cysts are those of congenital developmental origin, as a failure of the left umbilical vein either to obliterate or to retain peritoneal inclusions. Secondary cysts include t h o s e of: infectious origin, such as an echinococcus cyst or one resulting from extravasation of bile; neoplastic origin with secondary degeneration of the t u m o r ; t r a u m a t i c origin with liquefaction of a resulting h e m a t o m a . CASE REPORT

A twenty-seven year old white mother of five was first seen in the University of Oklahoma Hospitals emergency room on December 4, t963, because of a sharp colicky epigastric pain that penetrated through to the back. It had become increasingly sex'ere within the last four weeks. Seven years prior to this admission, the first sharp attack of colicky epigastric pain developed and was associated with icterie sclerae. Intermittently since then, this pain often occurred in the absence of jaundice. The pain was increased by food and associated with eructation and flatulence. Four weeks prior to adnfission, it became much more constant. Eight days prior to admission, aeholie stools, icterie sclerae, and bilirubimlria occurred. This icterus had progressively deepened, although the pain had decreased in frequency and intensity. There was nausea but no vomiting. Malaise, anorexia, and a 0..5 pound weight loss had occurred during the preceding month. There was no history of blood diseases, bleeding diatheses, or chills and fever. Past history was pertinent in that an appendectomy had been performed at the age of twelve years. On admission, her genc*tal appearance was that of an obese, ieteric woman with chronic abdominal distress. The vital signs were: blood pressure 90/,50 A merlcan Journal of Surgery

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1 2 FiG. 1. Sagittul section demonstrating cyst within falciform ligament and its attachtnent to the posterior rectus sheath.

t:tG. 2. Diagranmlatie illustration of anteri~,r al~pcaranee of tile cyst eaveloped in the falcifortn ligament with its attachmetlt into the porta hepatis. ram. IIg, pulse S0 and rcgmlar, t e m p e r a t u r e 100°F. ¢~rally, a n d respirations 20 a n d regular. P e r t i n e n t physical findings were limited to the following: skin a n d c o n j u n c t i v a were mildly icteric. C a r i o u s teeth were present. Rhonchi were present t h r o u g h o u t the bases of b o t h lungs t h a t cleared upon coughing. T h e a b d o m e n was obese and relaxed with p r o m i n e n t striae. A well healed .McBurney's incision was present. Bowel sounds were n o r m a l . T h e r e was a b d o m inal t e n d e r n e s s o n l y to deep p a l p a t i o n in the right upl)er q u a d r a n t . A definite m a s s in the right Ul)t)er q u a d r a n t was p a l p a t e d a n d e x t e n d e d from the midline of the a b d o m e n laterally 8 cm. and 6 era. below the right costal margin. I t did not extend below the ttmbilicus a n d was diffusely lender. Although this spherical cystic t u m o r did not m o v e with r e s p i r a t o r y excursions, the clinical eoncensus was an inflamed gallbladder. Mild peripheral e d e m a was present. L a b o r a t o r y d a t a on admission were as follows: h e m o g l o b i n l l.(i gill. per cent, h e m a t o c r i t 37 per cent, a n d white blood cell c o u n t 6,400 per cu. ram. T h e tlrille was alnl)er ill a p p e a r a n c e with 1 + bilirubinuria. Bilirubin on admission was 13.7 rag./100 co., direct fraction 4.~ tng. per 100 co. Alkaline phosp h a t a s e was 18 Bessie-Lnwrv units, t o t a l s e r u m proteins t;.l gin./100 ce., cephalin flocculation in twenty-four, h o u r s was .'3+ a n d in f o r t y - e i g h t hours ,~+. Serttm electrolytes were within n o r m a l limits on admi½sion, a n d b l o o d . u r e a nitrogen was 22 m g . / 100 ce. Serologic test for syphilis was nonreactive. P o s t e r n a n t e r i o r a n d left lateral chest roentgenog r a m s d e m o n s t r a t e d an old healed i n t l a m m a t o r y disease, m o s t likely tuberculosis. R o c n t g e n o g r a m s of the a b d o m e n were n o n c o n t r i l m t o r y . (In Deceml)er tl, l:)f;3, with tile p a t i e n t u n d e r onI'ol. i0~. A p,'il IOt~5

0"27

d o t r a c h e a l p e n t o t h a l - c n r a r a e - n i t r o u s oxide anesthesia, tile a b d o m e n was explored through a right sttbcostal ( K o c h e r ) incision. A thick-walled, cystic, p e a r - , h a p e d mass lying entirely in and c o n t i n u o u s with the falciform l i g a m e n t was observed. I t m e a s u r e d 7 era. in d i a m e t e r a n d e x t e n d e d i 1 cm. inferiorly f r o m the h c p a t a portis. Superiorly. its neck, eoutiguuusly with the o b l i t e r a t e d l i g a m e n t u m teres hepatis, was inserted in a funnel-shaped m a n n e r into the p o r t a het)atis in the fissure of tile round ligament. (Fig. 1 and 2.) T h e c y s t wall was fibrous, thick, a n d s m o o t h , except where the p e r i t o n e u m was reflected a n t e r i o r l y and a t t a c h e d to the t r a n s v e r s u s fascia. T h e c y s t wall was stained a glistening reddish .green. Tile c o n n n o n bile d u c t a p p e a r e d dilated. As the falciform m a s s was reflected front its peritoneal a t t a c h m e n t s in the midline to the posterior rectus sheath, it was i n a d v e r t e n t l y opened. Bilecolored fluid issued forth. Within the c y s t s o m e m o d e r a t e l y recent blood clots were also present. T h e neck was t h e n incised from t h e posteroinferior surface ~ff the liver in the fissure t h a t lodged the r o u n d ligmnent. This a r e a was quite lirm a n d not a t all cystic, seemingly confluent with the liver, a l t h o u g h cystic distally. Brisk bleeding was e n c o u n t e r e d from the parauntbilleal veins. T h e s e were closed with a r u n n i n g t h r o u g h a n d through s u t u r e of No. 4 - 0 arterial silk. T h e neck of the t u m o r was t h e n c o m pletely t r a n s e c t e d and the m a s s r e m o v e d . Choleeystectolny, c o n u n o n d u c t exploration, a n d o p e r a t i v e c h o l a n g i o g r a m were p e r f o r m e d w i t h o u t difficulty. A single mixed gallstone nleasnring 0.1 cm. in d i a m e t e r was r e m o v e d f m t n the c o m m o n bile d u c t a t the a m p u l l a of Valet. A No. 10 French T t u b e drain was inserted into the c m n m o n bile duct, anti the duct

Cyst

of Faleiform

Ligament

FIG. 3. Microscopic section of cyst wall demonstrating: A, peritoneal reflection about cyst; B, recent coagulmn within cyst cavity; C, acute and chronic inflammatory infiltrate with bile precipitate (magnification X 20 before reduction; hematoxylin and eosin stain). closed a b o u t the T tube, A T t u b e c h o l a n g i o g r a m s u b s e q u e n t l y p e r f o r m e d showed good filling of the c o m m o n bile d u c t a n d its radicles. A Penrose drain was placed within the bed of the gallbladder a n d leading up to the point where the t u m o r h a d been t r a n s e c t e d a n d then b r o u g h t out t h r o u g h a s t a b wound. T h e a b d o m e n was closed. Considerable clear bile d r a i n a g e f r o m the T t u b e occurred for the first t w e n t y - f o u r hours a n d subsequently decreased. T h e r e m a i n d e r of the p o s t o p erative course was entirely u n e v e n t f u l . T h e T t u b e was r e m o v e d a f t e r a n o r m a l T t u b e c h o l a n g i o g r a m on the ninth p o s t o p e r a t i v e d a y and the p a t i e n t discharged. She has not returned for follow-up visits. Pathologic r e p o r t of the specimen consisted "grossly of a 9 b y 11 b v 7 cm. p e a r - s h a p e d m a s s c o m p o s e d for t h e m o s t p a r t of a thick i n d u r a t e d material t h a t was a n m t t l c d reddish broxxm to reddish green and contained local areas of a t t a c h e d adipose tissue." " T h e specimen was opened a n d c o n t a i n e d a cyst-like c a v i t y t h a t h a d some greenish discoloration of the wall a n d s c a t t e r e d reddish brown blood clot within its l u m e n . " T h e m a j o r i t y of the m a s s was c o m p o s e d of a m o r e solid s t r u c t u r e t h a t a p p e a r e d to be green discolored adipose tissue with various string~,, slightly s p o n g y a r e a s of more m u c o i d tissue and firmer green areas. S c a t t e r e d lobulations were noted. Microscopically, m u l t i p l e sections d e m o n s t r a t e d a thin peritoneal reflection b e n e a t h which were lai-ge areas of granulation tissue and fat which c o n t a i n e d cystic spaces t h a t were filled with an a n m r p h o u s m a t e r i a l t h a t a p p e a r e d to be bile. (Fig. 3 a n d 4.) A c u t e a n d chronic i n f l a m m a t o r y reaction was s c a t t e r e d t h r o u g h o u t the specimen. O t h e r areas showed fibroeollagenous disposition. T h e lesion a p p e a r e d multiloculated, T h e gallbladder m e a s u r e d 7 cm. in length a n d had

of Liver

F1G. 4. Microscopic section from blocked area in Figure 3 demonstrating bile precipitate (magnification X 450 before reduction). an a v e r a g e d i a m e t e r of 3 cm. I t c o n t a i n e d t w e n t y calculi t h a t were m u l t i f a c e t t e d yellowish green in color. T h e m u c o s a a p p e a r e d a t r o p h i c a n d yellow in color. Section of the gallbladder showed chronic i n f l a m m a t o r y reaction t h r o u g h o u t its wall with dev e l o p m e n t of R o k i t a n s k y - A s c h o f f sinuses. CLINICAL FEATURES AND DIAGNOSIS OF FALCIFORM CYSTS I n t h e m a j o r i t y o f c a s e s , t h e s y m p t o m s will vary individually and present no specific sympt o m c o m p l e x . O f t e n a m a s s is n o t i c e d a n d t h i s may be the only complaint. Indigestion, flatulence, and a feeling of fullness after meals are often complaints. The pain, when present, is o f t e n r e l a t e d t o p o s i t i o n a n d v a r i e s f r o m a dull aching intermittent pain to a sharp colicky p a i n w h i c h is p o o r l y l o c a l i z e d . There are often no physical signs on examinat i o n . T h e t m n o r , w h e n p r e s e n t , is t e n d e r , g e n e r a l l y lies t o t h e r i g h t o f t h e m i d l i n e , a n d d o e s n o t e x t e n d b e l o w t h e u m b i l i c u s . T h e r e is n o motion with respiration. T h e s t r i k i n g s i m i l a r i t y of t h e s e n i n e c a s e s has been the uniform confusion and assumption t h a t t h i s t u m o r in t h e r i g h t u p p e r q u a d r a n t is a renal or hepatic tumor or gallbladder. Derm o l d s , h e m a t o m a s , h y p e r n e p h r o m a s , t u m o r s of the omentum, mesenteric cysts, pseudocysts, a n d d i l a t e d g a l l b l a d d e r s h a v e all b e e n c o n s i d e r e d in t h e d i f f e r e n t i a l d i a g n o s i s . A n a m e b i c abscess or echinococeus cysts have been suspected, but pertinent history, laboratory and antigenic tests have ruled these out. Pertinent x - r a y s t u d i e s a r e of v a l u e in r u l i n g o u t r e n a l and gastrointestinal involvement.

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America. Journal of Surgery

Gondring "FAm.E I CASF. R~VI~W Age (yr.) and ;;ex

Author

Presenting Symptoms

Henderson, 1909 [1]

41, Male

lg,ight year history of abdominal mass

Chifoliau, 1926 17]

49, Male

Four year history of abdominal mass

~,Vakeley anti ~;1acMyn, 1937 13] Iterrou. 1937 [8]

5,t, Female

tterrou, 19:t7 [8]

31, Female

Dyspeps..~ for many years a n d a four month history of abdominal mass Two year history of abdominal mass and dyspct),'-! a liight yem t~istory of right lumbar pain

Lightwood and Campbell,

.tmo..Male

Abdomiual mass from birth

Brown. 19-18 [6]

26, Male

Acute condition within the abdomen with at¢dominal umss

Karabin. 1951 [5]

24, Female

Gondring, 1961

27, Female

Dull abdominal pain for six weeks after b h m t t r a u m a to abdomen; repair of partial eventration of the intestine through the umbilicus at birth Six year history of progressive colicky epigastric pain, abdominal mass of recent duration

1939 [41

32, Male

TREATMENT

W h e n suspected, l a p a r o t o n l y is indicated. T h e correct t r e a t m e n t is to relnove the c y s t to obtain the correct pathologic diagnosis as well as to avoid subsequent conaplications from the c y s t p e r se. H e m o r r h a g e into t h e cyst can produce a s u d d e n onset of acute s y m p t o m s . In cases in which the c y s t is a t t a c h e d b y a pedicle or a fibrous band is present, torsion and strangulation can result [6]. In all cases reported t h u s far in the American and British literature, the surgical t r e a t m e n t has been excision witho u t a n y operative or p o s t o p e r a t i v e colnplieations. (Table r.)

Straw-colored mass and thin-walled cystic tumor the size of a child's head; unilocular cyst, 8 era. in length, containing clear liquid Fibrous-walled cyst, 2 inches in diameter Uuilocular cyst the size of an infant's head Mnltiloeular cyst the size of an infant's head, with pedicle to liver and umbilicus Mass similar in size to liver 10 by 12 era. cyst with partial torsion about a fibrous band from the anterior abdominal wall Fusiform cyst 6 inches in length, 2 inches in width, 7 inches in depth, filled with serosanguineous fluid Pear-shaped 9 era. iu width. 11 era. in length, and 7 era. in depth, reddish brown cyst containing blood clot and bile colored fluid

a n d American literature of a falciform c y s t associated with cholelithiasis and chronic cholecystit is. CONCLUSIONS

1. A c y s t of the falciform ligament is a n o t h e r e n t i t y to consider in the differential diagnosis of a mass in the r i g h t upper q u a d r a n t t h a t feels a t t a c h e d to the anterior a b d o m i n a l wall t o w a r d the nfidline a n d above the umbilicus. 2. A diagnosis of these rare cysts can be m a d e only by surgical exploration. 3. T h e t r e a t m e n t of choice is excision. RF, F E R E N C E S 1. HENDERSON, M . S. C y s t o f t h e r o u n d l i g a m e n t o f t h e l i v e r . Ann. Surg., 50: 550, 1909. 2. HOLLINSHEAD, W . H . A n a t o m y f o r S u r g e o n s , v o l . 2. T h e T h o r a x , A b d o m e n , a n d P e l v i s , p, 3 0 1 , 317, 318. N e w Y o r k , 1956. P a u l B. H o e b e r , I n c . 3. WAKELEV, C. P. G . a n d M A c M v N , D . J . N o n - p a r a s i t i c c y s t o f t h e l i v e r . A r e p o r t of t w o c a s e s t o g e t h e r w i t h a case of t h e l i g a m e n t u m teres h e p a t i s . Lancet, 2 : 675, 1931. 4. Lmt~TWOOD, R . a n d CAMPBm.L, L. I. S.. C o n g e n i t a l c y s t o f r o u n d l i g a m e n t of l i v e r . Lancet, 2: 1027, 1939. 5. I~.ARABIN, J . n . C y s t in t h e l i g a m e n t u m t e r e s of t h e l i v e r . A m . J . S u r g . , 8 2 : 531, 1951. 6. BROWN, J . S, C y s t s of t h e f a l e i f o r m l i g a m e n t . Southern Surgeon, 14: 278, 1948. 7. C m F O L m U , M . K y s t e s d r e u x d a n s le l i g a m e n t s u s p e n s e u r d u foie. Bull. et m~m. Soc. Nat. de Chir., 52: 1197, 1926. ( F r o m : B r o w n , J . S. [ 6 ] . ) 8. H e R R O U . H . C o n t r i b u t i o n fi l ' 6 t u d e d e s K y s t e s s ~ r e u x d e s l i g a m e n t s d u role. P a r i s T h e s i s , 1937. ( F r o m : B r o w n , J . S. [ 6 ] . )

COMMENTS

T h e r e is histologie evidence t h a t bile was present within the cystic c a v i t y in this patient. Serial sections of t h e neck of t h e faleiform c y s t d e m o n s t r a t e d no biliary radieljes. A l t h o u g h there was no evidence of bile c o n t a m i n a t i n g t h e peritoneal c a v i t y , it m u s t be concluded t h a t there was e x t r a v a s a t i o n of bile from some source producing a bile r e t e n t i o n cyst. No histologic evidence of epithelium lining the cystic c a v i t y was present. T h e a d d i t i o n a l presence of' b o t h a c u t e and chronic i n f l a l n m a t o r y infiltrates f u r t h e r rulds o u t a congenital cause. T h e presence of recent blood clots w i t h o u t histologic evidence of hemosiderin suggests an a c u t e hemorrhage, perhaps a t surgery, and tends to rule out a hemorrhagic cause. This is the first case recorded in the British Vol, 109, April 1965

l)escription of Falclfornt Cyst

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