7ndlan) r#dle,. 46 : ~19, 11~9
LYMPHANGIOMA
OF MESENTERY
IN A C H I L D *
A Case Report
HI~zuR ]~AIIMAIq,VJJAYKRISlDqA AOAI~WAL, SURgSH CHANDRAGUPTA AI4D ASHOK KUMAR SRIVASrAVA
,4ltahabad L y m p h a n g i o m a s of the mesentery are exceedingly rare. T h e Florentine anatomist Benevieni is generally credited to having described the first case in 1507. Parson (1936) found 500 cases of mesenteric cyzts in the literature, of which only 10 were possibly o f l y m p h a t i c origin. Beahrs c~ al. ,(1950) found only 9 cases of l y m p h a n g i o m a of the mesentery at Mayo Clinic from 191l to 1942. Collins and Berdes (1934) did not find e,aen a single case of chylous cyst o f the mesentery in 15000 necropsies at the University of Minnesota. Due to the infrequent occurrence of these cysts a n d the importat,ce of their recognition and m a n a g e m e n t it seemed desirable to report this case of l y m p h a n gioma of the mesentery. Report of a Case A . , a 3-year-old male child, was a d m i t t e d to the S.N. Children's Hospital, Altahabad, on4.11.77 with the history of pain a[Jd swelling in the a b d o m e n for ten days. T h e pain was severe in intensity, arising in the umbilical area, spreading all over the a b d o m e n and coming in bouts. 1 h e swelling noticed in the lett side oi the a b d o m e n *From the S.N. Children's Hospital, the Departments of Paediatrict and Pathology, MLN. bledical College, (lhurch Lane, Allahabad, 211002. Reeeived on June 27, 1978.
gradually increased in size over a period of ten days. H e had few bouts of v o m i t i n g during hospitalisatlon. Bowel movements were normal. Physical examination revealed a m o d e rately well nourished child, without any significant l y m p h a d e n o p a t h y , cyanosls, j a u n dice or o e d e m a . . Abdomlna] e x a m i n a t i o n revealed a, smooth, nontender, firm, intraabdominal lump occupying the left lower hypochondrium, l u m b a r and left side of the umbilical regions, not moving with respiration but slightly mobile from side to side. Its limits were welt defined. T h e renal angles were free. No fluid was present in the peritoneal cavity.
Investigations: Haematological investigations except showing mild a n a e m i a were within normal limits. A plain X-ray of the a b d o m e n showed a soft tissue shadow occupying the left side of the abdomen. Barium meal studies showed displacement of one loop of small bowel to the extreme left side and of the rest of the bowel loops to tl;e right. I.V.P. showed both kidneys functioning normally. Clinical diagnosis of a mesenteric l u m p was made. Exploratory laparotomy revealed a large multicystic swelling arising from the jejunal mesentery extending upto its root. T h e jejunal loop was stretched over the cyst and its mesenteTic border merged with the
220
INDIAN JOURNAL OP PgDIATIglOS
cyst wall. T h e lumen of the cyst did not communicate with that of the jejunum. T h e cyst along with 20 cm of a jejunal loop firmly attached with it was excised and end to end anastomosis was performed. T h e post-operative course was uneventful and the patient recovered completely, T h e specimen measured 12 cm • 8.5 cm. T h e outer surface was nodular and the cut surface showed multiple cystic spaces. On histopathological examination, large cavernous spaces filled with lymph and collection of lymphoid tissue were seen.
Discussion L y m p h a n g i o m a o f the mesentery is a rare entity. Common sites are neck and axilla but these have also been reported in arm, mediastinum, m o u t h region and abdomen, other rare sites being kidney, spleen, and pancreas (Willis 1967). Most of the cervical and axillary growths are noticed at birth but some do not appear until later. In the report of Burnett a at, (1950) o f 200 cases of mesenteric cysts of all types, 25% of the cases were children under the age of 10 years. Gross (1953) reported the discovery of congenital cysts of the omentum, mesentery and mesocolon in 19 children, all I0 years or younger. Sex is nearly equally affected, females slightly predominating (Moore 1957, Willis 1967). Burnett a al. 1950) also reported a slight female preponderance (1.6:1). T h e r e is considerable doubt as to whether these lesions are true angiomatous malformations. Goetsch t938) believes that these lymphatic tumours are true infiltrating neoplasms and not merely an enlarging cyst or malformation. Ewing (1940) considered these as iymphangiomas arising from
VoL. 46, No. 37Z embryonic lymphoid centres due to congenB tal or acquired obstruction of the lacteal~j In the opinion of Willis (1967) fluid accumulation, progressive formation df collaterals and in some cases supervenin~ thrombosis and organisation suffice t a account for growth of these tumoursr Anderson (1967) thought that probably these tumours arose from seqttestrations of lymphatic tissue. Gross (1953) regarded them to he arising from congenitally dis-, placed lymphatics which tailed to commtt~ nicate with the normal channels. Lympha.,, tic obstruction as a possible cause has also] been suggested (Handelsman and Ravitc~ 1954, Amos 1959). These cysts are usually single. In some cases a number of cysts m a y be present: T h e y may be present in any part of the mesentery but usually in the root (Andersor~ 1967). These cysts may attain a great size. ! n the report of 4 cases by Moore (1957), all cysts were of a large size, two being dumb-bell shaped. These cysts were unilocular. Clinically, the most suggestive symptorfis in many cases are repeated attackr of abdominal pain, abdominal enlargement, vomiting and nausea (Gross 1953, Moore 1957). 80% o f cases of Burnett etal. (1950) had abdominal pain as the most frequent symptom and 50~/o had a palpable mass, vomiting and nausea. Acute symptoms are usually due to torsion and haemorrhage into the cyst. A c u t e intestinal obstruction may be produced due to stretching of the gut thus obliterating the lumen of the bowel (McNab and Menzies 1949) or due to volvulus of the bowel (Rohatgi et al. 1974). Major symptoms in 13 cases reported by Gross 0953) and 4cases by
221
qtAIIMAN ET AL.~LYMPHANOIOMA OF MESENTIgRy I[N'A ChiLD
]r (1957) were abdominal enlarge.~ent, pain and nausea and vomiting. Fever, diarrhoea and constipation were aTso present in some cases. Miller (195't) reports the case of a 3-week-old infant with fever and melaena. Major symptoms ~n our case were pain, an abdominal lump and occasional vomiting. Solitary tumours can easily be enu~leated without jeopardising the blood supply of the adjacent bowel whereas the tumours that ~eem to invade the adjacent tissue, need wide resection. Out of 13 cases, Gross 0 9 5 3 ) had to perform resection with anastomosis in 4 cases and simple enucleafloe. in 7. T w o cases had associated V0[~'ulus of the small bowel which was corrected. T w o cases of Moore (1957) and 4 of Handelsman and Ravitch (1954) required resection. In our case removal of the turnout with resection and end to end anastomosis was required. Summary and Conclusion A case of lymphangioma of the mesentery in z 3-year-old boy is presented. Regarding the aetiopathogenesis, consensus of opinion is that these are hamartomatous malformations. Most of the cases present with pain and swelling in the abdomen, nausea and vomiting, intravenous pyelograms and barium meal studies may ilelp in establishing the diagnosis. Simple enucleation if possible should be done. Resection with anastomosis may be required in some ~.ases,
References
Amos, .J.A.C. (1959). Mu|tip|e lymphatic cyst.#of the mesentery. Brit. J , Surg. 46, 588.
Anderson, W. (1967}. Boyd's Pathology for Surgeons. 8th Ed., W.B. ,C;aundersCo.,Philadelphla, P. 147. Beahr% O.H., Judd, E.S., Dockerty, M.B. (1950), Chylous cyst of abdomen, Surg, Clin. North Am. 30,
1081, Benevieni. (1957). Ann, Surg., 145,428.
Quoted from Moore, T.G.
Burners. W.E., Roscmond, G,P., Buhcher, R.M. (1950). Mesenterlc cyst, Arch. Surg. 60, 699. Collins, A.N., Berdes, G.L. (1934). Chyle cysts of the mesentery. Arch. Surg. 28, $35. gwing, S. (1940). Neoplastic Diteases.i 4th Ed.~ W.B. $aundu: Co., Philadelphia. Goettch, E. (1938). Hygtoma colli cysticum and hygroma axillare. Arch. Surg. 36, 394.
Gross, R.E. (1953). Surgery of Infancy and Childhood. W.B. Saunders Co., Philadelphi% P. 336. Handelsman, J.C., Ravitch, M.M. (1954). Chylous cyst of rr~esentery in children. Ann. Surg. 140 185.
McNab, I., Menzles, T. (1959). Lymphamgioma Qf the mesentery. Brit..7. Surg. 37, 294. Miller, D.B. (1954). Chyle cyst of the mesentory. Arch, Surf. Chitago, 68, 359.
Moore, T.C., (1957). Congenital cyst of mesentery. Ann. Surg. 145, 428. Parson, E.O. (1936). True proliferating cystic iymphangioma of the mesentery. Ann. Surg. 103, 595.
Rohatgi, M., Berry, M., Des, P.K. (19"/4). Lymphangioma of mesentery, a rare tumour of infancy and childhood. Indian Pcd;a'r. 11, 231. Willis, R.A., ~1967). Pathology of Tumours. 4th Ed. London Butterworth.
LYMPHANGIOMA
OF MESENTERY
IN A C H I L D *
A Case Report
HI~zuR ]~AIIMAIq,VJJAYKRISlDqA AOAI~WAL, SURgSH CHANDRAGUPTA AI4D ASHOK KUMAR SRIVASrAVA
,4ltahabad L y m p h a n g i o m a s of the mesentery are exceedingly rare. T h e Florentine anatomist Benevieni is generally credited to having described the first case in 1507. Parson (1936) found 500 cases of mesenteric cyzts in the literature, of which only 10 were possibly o f l y m p h a t i c origin. Beahrs c~ al. ,(1950) found only 9 cases of l y m p h a n g i o m a of the mesentery at Mayo Clinic from 191l to 1942. Collins and Berdes (1934) did not find e,aen a single case of chylous cyst o f the mesentery in 15000 necropsies at the University of Minnesota. Due to the infrequent occurrence of these cysts a n d the importat,ce of their recognition and m a n a g e m e n t it seemed desirable to report this case of l y m p h a n gioma of the mesentery. Report of a Case A . , a 3-year-old male child, was a d m i t t e d to the S.N. Children's Hospital, Altahabad, on4.11.77 with the history of pain a[Jd swelling in the a b d o m e n for ten days. T h e pain was severe in intensity, arising in the umbilical area, spreading all over the a b d o m e n and coming in bouts. 1 h e swelling noticed in the lett side oi the a b d o m e n *From the S.N. Children's Hospital, the Departments of Paediatrict and Pathology, MLN. bledical College, (lhurch Lane, Allahabad, 211002. Reeeived on June 27, 1978.
gradually increased in size over a period of ten days. H e had few bouts of v o m i t i n g during hospitalisatlon. Bowel movements were normal. Physical examination revealed a m o d e rately well nourished child, without any significant l y m p h a d e n o p a t h y , cyanosls, j a u n dice or o e d e m a . . Abdomlna] e x a m i n a t i o n revealed a, smooth, nontender, firm, intraabdominal lump occupying the left lower hypochondrium, l u m b a r and left side of the umbilical regions, not moving with respiration but slightly mobile from side to side. Its limits were welt defined. T h e renal angles were free. No fluid was present in the peritoneal cavity.
Investigations: Haematological investigations except showing mild a n a e m i a were within normal limits. A plain X-ray of the a b d o m e n showed a soft tissue shadow occupying the left side of the abdomen. Barium meal studies showed displacement of one loop of small bowel to the extreme left side and of the rest of the bowel loops to tl;e right. I.V.P. showed both kidneys functioning normally. Clinical diagnosis of a mesenteric l u m p was made. Exploratory laparotomy revealed a large multicystic swelling arising from the jejunal mesentery extending upto its root. T h e jejunal loop was stretched over the cyst and its mesenteTic border merged with the
220
INDIAN JOURNAL OP PgDIATIglOS
cyst wall. T h e lumen of the cyst did not communicate with that of the jejunum. T h e cyst along with 20 cm of a jejunal loop firmly attached with it was excised and end to end anastomosis was performed. T h e post-operative course was uneventful and the patient recovered completely, T h e specimen measured 12 cm • 8.5 cm. T h e outer surface was nodular and the cut surface showed multiple cystic spaces. On histopathological examination, large cavernous spaces filled with lymph and collection of lymphoid tissue were seen.
Discussion L y m p h a n g i o m a o f the mesentery is a rare entity. Common sites are neck and axilla but these have also been reported in arm, mediastinum, m o u t h region and abdomen, other rare sites being kidney, spleen, and pancreas (Willis 1967). Most of the cervical and axillary growths are noticed at birth but some do not appear until later. In the report of Burnett a at, (1950) o f 200 cases of mesenteric cysts of all types, 25% of the cases were children under the age of 10 years. Gross (1953) reported the discovery of congenital cysts of the omentum, mesentery and mesocolon in 19 children, all I0 years or younger. Sex is nearly equally affected, females slightly predominating (Moore 1957, Willis 1967). Burnett a al. 1950) also reported a slight female preponderance (1.6:1). T h e r e is considerable doubt as to whether these lesions are true angiomatous malformations. Goetsch t938) believes that these lymphatic tumours are true infiltrating neoplasms and not merely an enlarging cyst or malformation. Ewing (1940) considered these as iymphangiomas arising from
VoL. 46, No. 37Z embryonic lymphoid centres due to congenB tal or acquired obstruction of the lacteal~j In the opinion of Willis (1967) fluid accumulation, progressive formation df collaterals and in some cases supervenin~ thrombosis and organisation suffice t a account for growth of these tumoursr Anderson (1967) thought that probably these tumours arose from seqttestrations of lymphatic tissue. Gross (1953) regarded them to he arising from congenitally dis-, placed lymphatics which tailed to commtt~ nicate with the normal channels. Lympha.,, tic obstruction as a possible cause has also] been suggested (Handelsman and Ravitc~ 1954, Amos 1959). These cysts are usually single. In some cases a number of cysts m a y be present: T h e y may be present in any part of the mesentery but usually in the root (Andersor~ 1967). These cysts may attain a great size. ! n the report of 4 cases by Moore (1957), all cysts were of a large size, two being dumb-bell shaped. These cysts were unilocular. Clinically, the most suggestive symptorfis in many cases are repeated attackr of abdominal pain, abdominal enlargement, vomiting and nausea (Gross 1953, Moore 1957). 80% o f cases of Burnett etal. (1950) had abdominal pain as the most frequent symptom and 50~/o had a palpable mass, vomiting and nausea. Acute symptoms are usually due to torsion and haemorrhage into the cyst. A c u t e intestinal obstruction may be produced due to stretching of the gut thus obliterating the lumen of the bowel (McNab and Menzies 1949) or due to volvulus of the bowel (Rohatgi et al. 1974). Major symptoms in 13 cases reported by Gross 0953) and 4cases by
221
qtAIIMAN ET AL.~LYMPHANOIOMA OF MESENTIgRy I[N'A ChiLD
]r (1957) were abdominal enlarge.~ent, pain and nausea and vomiting. Fever, diarrhoea and constipation were aTso present in some cases. Miller (195't) reports the case of a 3-week-old infant with fever and melaena. Major symptoms ~n our case were pain, an abdominal lump and occasional vomiting. Solitary tumours can easily be enu~leated without jeopardising the blood supply of the adjacent bowel whereas the tumours that ~eem to invade the adjacent tissue, need wide resection. Out of 13 cases, Gross 0 9 5 3 ) had to perform resection with anastomosis in 4 cases and simple enucleafloe. in 7. T w o cases had associated V0[~'ulus of the small bowel which was corrected. T w o cases of Moore (1957) and 4 of Handelsman and Ravitch (1954) required resection. In our case removal of the turnout with resection and end to end anastomosis was required. Summary and Conclusion A case of lymphangioma of the mesentery in z 3-year-old boy is presented. Regarding the aetiopathogenesis, consensus of opinion is that these are hamartomatous malformations. Most of the cases present with pain and swelling in the abdomen, nausea and vomiting, intravenous pyelograms and barium meal studies may ilelp in establishing the diagnosis. Simple enucleation if possible should be done. Resection with anastomosis may be required in some ~.ases,
References
Amos, .J.A.C. (1959). Mu|tip|e lymphatic cyst.#of the mesentery. Brit. J , Surg. 46, 588.
Anderson, W. (1967}. Boyd's Pathology for Surgeons. 8th Ed., W.B. ,C;aundersCo.,Philadelphla, P. 147. Beahr% O.H., Judd, E.S., Dockerty, M.B. (1950), Chylous cyst of abdomen, Surg, Clin. North Am. 30,
1081, Benevieni. (1957). Ann, Surg., 145,428.
Quoted from Moore, T.G.
Burners. W.E., Roscmond, G,P., Buhcher, R.M. (1950). Mesenterlc cyst, Arch. Surg. 60, 699. Collins, A.N., Berdes, G.L. (1934). Chyle cysts of the mesentery. Arch. Surg. 28, $35. gwing, S. (1940). Neoplastic Diteases.i 4th Ed.~ W.B. $aundu: Co., Philadelphia. Goettch, E. (1938). Hygtoma colli cysticum and hygroma axillare. Arch. Surg. 36, 394.
Gross, R.E. (1953). Surgery of Infancy and Childhood. W.B. Saunders Co., Philadelphi% P. 336. Handelsman, J.C., Ravitch, M.M. (1954). Chylous cyst of rr~esentery in children. Ann. Surg. 140 185.
McNab, I., Menzles, T. (1959). Lymphamgioma Qf the mesentery. Brit..7. Surg. 37, 294. Miller, D.B. (1954). Chyle cyst of the mesentory. Arch, Surf. Chitago, 68, 359.
Moore, T.C., (1957). Congenital cyst of mesentery. Ann. Surg. 145, 428. Parson, E.O. (1936). True proliferating cystic iymphangioma of the mesentery. Ann. Surg. 103, 595.
Rohatgi, M., Berry, M., Des, P.K. (19"/4). Lymphangioma of mesentery, a rare tumour of infancy and childhood. Indian Pcd;a'r. 11, 231. Willis, R.A., ~1967). Pathology of Tumours. 4th Ed. London Butterworth.
