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Intestinal lymphangiectasia and colonic polyps: Surgical intervention
Intestinal Lymphangiectasia and Colonic Polyps: Surgical Intervention By H. G. Parsons and P. B. Pencharz
Montreal, Quebec, Canada 9 A 36-too-old boy with Miiroy's Disease, intestinal lymphangiectasia, and an exudative enteropathy (EE), was shown to have four colonic polyps. A large adenomatous polyp was excised from the transverse colon in an effort to control his EE and hypoalbuminemia (1.95 g/dl). His clinical status then stabilized until age 50 mo w h e n there was a marked exacerbation of his EE. Medical management resulted in a t e m p o r a r y stabilization of his condition. A partial resection (40 era) of the visually worse affected jejunum was performed. There was no improvement in the EE as measured by S~Cr-tagged albumin study; however, his clinical response was dramatic, In the 10 mo since surgery, he has been well and has shown catchup in linear growth. INDEX W O R D S : Intestinal lymphangiectasia; colonic polyps; i i l r o y ' s disease; protein-lowing enteropatby.
INTESTINAL lymphangiectap RIMARY sia represents a congenital hypoplasia of the mesenteric lymphatics resulting in dilated small bowel lymph vessels and an exudative enteropathy. j The hypoplasia may occur at a number of different sites in the bowel wall including the lamina propria, submucosa, serosa, and the mesentery. The clinical manifestations vary widely and often include lymphatic anomalies outside the gastrointestinal tract, 2 lymphopenia, hypoalbuminemia and hypoproteinemic edema. 3 Management of patients with an exudative enteropathy has been, until recently, medical. This involved restriction of the dietary intake of long chain fatty acids, supplementation of the diet with medium chain triglycerides and a high protein intakes. 4'5 Surgery was reserved for the rare patient with only a localized segment of his small bowel involvedY Recently, partial resecFrom the Department of Paediatrics, Montreal Children's Hospital, Montreal. Quebec, Canada. Presented at the llth Annual Meeting of the Canadian Association of Paediatric Surgeons, Montreal, February 5-9, 1979. Address reprint requests to Dr. H. G. Parsons, c/o Dr. P. B. Peneharz, The Hospital for Sick Children, 555 University Avenue. Toronto, Ontario, Canada M5G IX8. 9 1979 by Grune & Stratton, Inc. 0022-3468/79/1405-0006501.00/0 530
tion of the worst affected areas of bowel has been reported with modest success) We present a child with colonic polyps and intestinal lymphangectasia whose condition was improved by polypectomy and a partial resection of the jejunum. CASE REPORT K.R., a boy, born in July, 1972, was diagnosed as suffering from Milroy's Disease within the first few days of life. Lymphedema involved the whole of his right arm and leg, left hand and forearm, and scrotum and penis. A summary of the patient's laboratory findings, clinical course and treatment is shown in Table 1. At 18 mo of age, he was extensively reevaluated because of recurrent viral illnesses, hypogammaglobulinemia, and poor-feeding habits. A diagnosis of intestinal lymphangectasia with an exudative enteropathy was established by a S~Cr-tagged albumin study and a barium contrast study of the small bowel. A right-sided pedal lymphangiogram showed a reduced number of lymph vessels and four inguinal nodes, one of which was of normal size. Whereas on the left, 9 inguinal nodes were demonstrated, 2 of which were of normal size. The pelvic lymph channels and the thoracic duct were poorly visualized. He was discharged on a high protein, fat-free diet with supplemental MCT* oil, and monthly IM injections of gamma-globulin. On this regime he remained essentially stable, although his linear growth was retarded (Fig. 1). At 38 mo of age, he developed bloody diarrhea and his clinical status deteriorated. This was evidenced by an acute weight gain, increasing peripheral edema and marked lethargy. His serum albumin fell from 2,25 g to 1.39 g/dl and a double contrast barium enema revealed one large polyp in the transverse colon plus three smaller colonic polyps. Medical management proved unsuccessful and a 4.5 cm adenomatous polyp was resected from his transverse colon. After the polypectomy, his clinical state rapidly improved and stabilized for about 1 yr, at which time (50 mo of age) there was a recurrence of his symptoms. At 54 mo of age, he developed intermittent abdominal cramps, anorexia and vomiting, which prevented an adequate oral intake of calories and protein. His condition remained unstable and over the next 7 mo he required 5 lengthy hospital admissions. On the first of these admissions, he posed a very difficult fluid and electrolyte problem due to hypoalbuminemia and secondary byperaldosteronism that was eventually resolved by the use of daily spironolactone, furosemide, and total parenteral nutrition. His medical management, while not in the hospital, consisted of the above
*MCT o i l - - M e d i u m chain triglycerides oil. Mead Johnson Laboratories.
Journal of Pediatric Surgery, Vol. 14, No. 5 (October), 1979
LYMPHANGIECTASIA: SURGICAL INTERVENTION
531
Table 1, A Summary of the Patient's Laboratory Findings, Clinical Course, and Treatment Lymphocytes
Hb
Yotat Protein
Albumin
Age
(ram 3)
01/74 18/09/75
18 mo 38 mo
1166 1260
11.3 11.0
5.6 3.4
2.6 1.39
09/10/75 29/10/75 24/11/75 17/12/75 13/01/77 13/04/77 28/09/77
39 mo 391/z mo 401/2 mo 41 mo 54 mo 57 mo 62 mo
1200 1340
12.6 8.6 10.0
1120
12.4
4.5 3.5 5.5 4.9 3.7 3.8 4.9
2,36 1.95 2.66 3.00 1.79 1.82 3.00
12/10/77 25/01/78 07/06/78 09/08/78
63 66 71 73
4.8 4.1 3.5 3.5
2.90 2.70 2.20 2.10
Date
mo mo mo mo
990
(g/dl)
13.4
daily combined diuretic therapy, a high protein fat-free diet with M C T and a supplement of 600 mt of Vivonext per day. Radiographic examinations over this period of time were: ( 1) a repeat double contrast barium enema that showed no change in the size of the three remaining colonic polyps: (2) an upper GI and follow through showed the presence of thickened and edematous folds throughout the small intestine but no obstruction: (3) a repeat bipedal lymphangiogram that proved possible only to identify lymphatics in the left foot, which drained into a single large palpable superficial inguinal lymph node with no further upward passage of the dye. Visualization of two opacified external iliac lymph nodes, one on either side, from a previous examination at 38 too, suggested severe obstruction of the lymphatic drainage in the inguinal area (Fig. 2L Excretion in the stool of S~Cr-tagged albumin over a 4 day period was 4.2% (normal <2%). Although his clinical state had periods of stability, his medical management was not possible without recurrent hospitalization and hence, a faparotomy was performed at the age of 62 too. There was marked chylous acites, the entire small bowel was thickened and edematous and the mesentery was shortened, grossly thickened, and had multiple enlarged lymph nodes. There was relative sparing af the terminal ileum and the colon was unaffected. A 40-cm segment of the visually worst affected jejunum was rescctcd and an end to end anastomosis was performed. Pathological examination of the excised bowel and adjacent lymph nodes revealed dilation of lymphatics in the lamina propria, submucosa, and subserosa with atrophy and fatty replacement of the regional lymph nodes. Postoperatively his recovery was uneventful and he was discharged on a high protein, fat-free diet, M C T oil, Vivonex, calcium, and multiple vitamin supplements. His subsequent progress has been satisfactory, he has shown catchup growth (Fig. 1), is full of energy and is now attending regular school.
~Vivonex--an elemental fat-free formula. Eaton Laboratories.
Course and Treatment
Preliminary investigation Recurrent URI, diarrhea, hospitalized with increasing edema and hypoproteinemia Discharged following 20 days TPN Hospitalized for polypectomy Discharged post-polypectomy Stabilized on high protein, low fat, MCT diet Anorexia, emesis, diarrhea, abdominal cramps 5 days post 26 days TPN Fifth hospitalization in 7 mo; post-TPN, prior to intestinal resection 11 days after resection of 40 cm of jejunum 4 mo outpatient follow-up 81/2 mo post intestinal resection, clinical well 10 mo post-intestinal resection, clinical well
DISCUSSION
We are unable t o find any previous reference to colonic polyps occurring in a patient with small bowel intestinal lymphangiectasia. The excised polyp was a benign adenomatous lesion not associated with any m a l f o r m a t i o n of
135 130 125-
/ //"
120height, 115 cm
110 t 100
~ ee
f
8~t
Intestff, al resection
75,
70-
~olypectomy
I'
I
|
2 3
4
I
I
5 6
i
i
78
age, years Fig. 1.
Height growth record of the patient.
i
910
i
532
PARSONS AND PENCHARZ
Fig. 2. Left pedal lymphangiogram August, 1977 (note the dye remaining from the bi-pedal study done in August, 1975).
lymphatic tissue. The polyp was removed more because it was impossible to maintain the serum albumin than because of blood loss. We were unable to prove that there was a significant additional loss of protein from the polyp(s) in the face of the background of protein loss occurring in the small bowel. However, excision of the polyp did result in a dramatic resolution of his clinical status, which then remained stable for 1 yr. His second episode could be attributed to an increased loss of albumin in his small bowel which we hypothesize resulted in a vicious cycle. The reduction in serum albumin reduced serum oncotic pressure--one consequence of which was to further increase the edema of the bowel wall. If this edema was sufficient it would result in a
functional intestinal obstruction with vomiting. The failure of protein and energy intake would reduce liver albumin synthesis thus perpetuating the cycle. Certainly during two of his admissions for abdominal cramps, vomiting and diarrhea, thickened loops of bowel were readily palpated, although the barium studies showed no anatomical obstruction. Another consequence of his further reduced serum albumin was secondary hyperaldosteronism. His condition was then stabilized only by the use of continuous diuretics, salt poor albumin, and total parenteral nutrition. It was recognized that this was a precarious and unsatisfactory solution which led us to seek an alternative. Although it was firmly established that the lymphangectasia involved the whole of the small intestine, we were impressed by the favourable results of limited resection achieved by Kinmonth and Cox. s Therefore, we proposed a limited resection of the worst affected bowel, hoping that the resulting loss in absorptive surface would be insignificant while the reduction in the exudative enteropathy would be significant. In actuality, this did not prove to be the case, for 1 yr after surgery, intravenous chromium tagged albumen showed a loss of 15% of the administered dose over 96 hr, compared to 4% prior to operation. However, what it did do was relieve any further attacks of nausea, vomiting, emesis, and abdominal cramps and thus permit a high consistent dietary intake of protein. He no longer requires diuretics, his linear growth rate has increased and his general feeling of well being and activity is greater than at any previous time in his life. From our review of the literature this boy has the worst reported case of intestinal lymphangiectasia. His future is still guarded but his response to elective limited resection of the worst affected area of bowel has been dramatic.
REFERENCES
1. Gordon RS Jr: Exudative enteropathy. Lancet 1:325326, 1959 2. Pomerantz M, Waldmann TA: Systemic lymphatic abnormalities associated with gastrointestinal protein loss secondary to intestinal lymphangectasia. Gastroenterology 45:703-711, 1963 3. Waldmann TA: Protein-losing enteropathy. Gastroenterology 50:422-443, 1966 4. Holt PR: Dietary treatment of protein loss in intestinal lymphangectasia. The effect of eliminating dietary long chain triglycerides on albumin metabolismin this condition. Paediatrics 34:629-635, 1964
5. Tift WL, Lloyd JK: Intestinal lymphangectasia longterm results with MCT diet. Arch Dis Child 50:269-275, 1975 6. Muzaffar K, Block MA, Fox TA, Jr.: Primary intestinal lymphangectasia. Surgical implication. Am Surg 38:288-291, 1972 7. Vardy PA, Lebenthal E, Schwachman H: Intestinal lymphangectasia. A repraisal. Paediatrics 55:842-851, 1975 8. Kinmonth JB, Cox SJ: Protein-losing enteropathy in primary lymphoedema: mesenteric lymphography and gut resection. Br J Surg 61:589-593, 1974
Montreal, Quebec, Canada 9 A 36-too-old boy with Miiroy's Disease, intestinal lymphangiectasia, and an exudative enteropathy (EE), was shown to have four colonic polyps. A large adenomatous polyp was excised from the transverse colon in an effort to control his EE and hypoalbuminemia (1.95 g/dl). His clinical status then stabilized until age 50 mo w h e n there was a marked exacerbation of his EE. Medical management resulted in a t e m p o r a r y stabilization of his condition. A partial resection (40 era) of the visually worse affected jejunum was performed. There was no improvement in the EE as measured by S~Cr-tagged albumin study; however, his clinical response was dramatic, In the 10 mo since surgery, he has been well and has shown catchup in linear growth. INDEX W O R D S : Intestinal lymphangiectasia; colonic polyps; i i l r o y ' s disease; protein-lowing enteropatby.
INTESTINAL lymphangiectap RIMARY sia represents a congenital hypoplasia of the mesenteric lymphatics resulting in dilated small bowel lymph vessels and an exudative enteropathy. j The hypoplasia may occur at a number of different sites in the bowel wall including the lamina propria, submucosa, serosa, and the mesentery. The clinical manifestations vary widely and often include lymphatic anomalies outside the gastrointestinal tract, 2 lymphopenia, hypoalbuminemia and hypoproteinemic edema. 3 Management of patients with an exudative enteropathy has been, until recently, medical. This involved restriction of the dietary intake of long chain fatty acids, supplementation of the diet with medium chain triglycerides and a high protein intakes. 4'5 Surgery was reserved for the rare patient with only a localized segment of his small bowel involvedY Recently, partial resecFrom the Department of Paediatrics, Montreal Children's Hospital, Montreal. Quebec, Canada. Presented at the llth Annual Meeting of the Canadian Association of Paediatric Surgeons, Montreal, February 5-9, 1979. Address reprint requests to Dr. H. G. Parsons, c/o Dr. P. B. Peneharz, The Hospital for Sick Children, 555 University Avenue. Toronto, Ontario, Canada M5G IX8. 9 1979 by Grune & Stratton, Inc. 0022-3468/79/1405-0006501.00/0 530
tion of the worst affected areas of bowel has been reported with modest success) We present a child with colonic polyps and intestinal lymphangectasia whose condition was improved by polypectomy and a partial resection of the jejunum. CASE REPORT K.R., a boy, born in July, 1972, was diagnosed as suffering from Milroy's Disease within the first few days of life. Lymphedema involved the whole of his right arm and leg, left hand and forearm, and scrotum and penis. A summary of the patient's laboratory findings, clinical course and treatment is shown in Table 1. At 18 mo of age, he was extensively reevaluated because of recurrent viral illnesses, hypogammaglobulinemia, and poor-feeding habits. A diagnosis of intestinal lymphangectasia with an exudative enteropathy was established by a S~Cr-tagged albumin study and a barium contrast study of the small bowel. A right-sided pedal lymphangiogram showed a reduced number of lymph vessels and four inguinal nodes, one of which was of normal size. Whereas on the left, 9 inguinal nodes were demonstrated, 2 of which were of normal size. The pelvic lymph channels and the thoracic duct were poorly visualized. He was discharged on a high protein, fat-free diet with supplemental MCT* oil, and monthly IM injections of gamma-globulin. On this regime he remained essentially stable, although his linear growth was retarded (Fig. 1). At 38 mo of age, he developed bloody diarrhea and his clinical status deteriorated. This was evidenced by an acute weight gain, increasing peripheral edema and marked lethargy. His serum albumin fell from 2,25 g to 1.39 g/dl and a double contrast barium enema revealed one large polyp in the transverse colon plus three smaller colonic polyps. Medical management proved unsuccessful and a 4.5 cm adenomatous polyp was resected from his transverse colon. After the polypectomy, his clinical state rapidly improved and stabilized for about 1 yr, at which time (50 mo of age) there was a recurrence of his symptoms. At 54 mo of age, he developed intermittent abdominal cramps, anorexia and vomiting, which prevented an adequate oral intake of calories and protein. His condition remained unstable and over the next 7 mo he required 5 lengthy hospital admissions. On the first of these admissions, he posed a very difficult fluid and electrolyte problem due to hypoalbuminemia and secondary byperaldosteronism that was eventually resolved by the use of daily spironolactone, furosemide, and total parenteral nutrition. His medical management, while not in the hospital, consisted of the above
*MCT o i l - - M e d i u m chain triglycerides oil. Mead Johnson Laboratories.
Journal of Pediatric Surgery, Vol. 14, No. 5 (October), 1979
LYMPHANGIECTASIA: SURGICAL INTERVENTION
531
Table 1, A Summary of the Patient's Laboratory Findings, Clinical Course, and Treatment Lymphocytes
Hb
Yotat Protein
Albumin
Age
(ram 3)
01/74 18/09/75
18 mo 38 mo
1166 1260
11.3 11.0
5.6 3.4
2.6 1.39
09/10/75 29/10/75 24/11/75 17/12/75 13/01/77 13/04/77 28/09/77
39 mo 391/z mo 401/2 mo 41 mo 54 mo 57 mo 62 mo
1200 1340
12.6 8.6 10.0
1120
12.4
4.5 3.5 5.5 4.9 3.7 3.8 4.9
2,36 1.95 2.66 3.00 1.79 1.82 3.00
12/10/77 25/01/78 07/06/78 09/08/78
63 66 71 73
4.8 4.1 3.5 3.5
2.90 2.70 2.20 2.10
Date
mo mo mo mo
990
(g/dl)
13.4
daily combined diuretic therapy, a high protein fat-free diet with M C T and a supplement of 600 mt of Vivonext per day. Radiographic examinations over this period of time were: ( 1) a repeat double contrast barium enema that showed no change in the size of the three remaining colonic polyps: (2) an upper GI and follow through showed the presence of thickened and edematous folds throughout the small intestine but no obstruction: (3) a repeat bipedal lymphangiogram that proved possible only to identify lymphatics in the left foot, which drained into a single large palpable superficial inguinal lymph node with no further upward passage of the dye. Visualization of two opacified external iliac lymph nodes, one on either side, from a previous examination at 38 too, suggested severe obstruction of the lymphatic drainage in the inguinal area (Fig. 2L Excretion in the stool of S~Cr-tagged albumin over a 4 day period was 4.2% (normal <2%). Although his clinical state had periods of stability, his medical management was not possible without recurrent hospitalization and hence, a faparotomy was performed at the age of 62 too. There was marked chylous acites, the entire small bowel was thickened and edematous and the mesentery was shortened, grossly thickened, and had multiple enlarged lymph nodes. There was relative sparing af the terminal ileum and the colon was unaffected. A 40-cm segment of the visually worst affected jejunum was rescctcd and an end to end anastomosis was performed. Pathological examination of the excised bowel and adjacent lymph nodes revealed dilation of lymphatics in the lamina propria, submucosa, and subserosa with atrophy and fatty replacement of the regional lymph nodes. Postoperatively his recovery was uneventful and he was discharged on a high protein, fat-free diet, M C T oil, Vivonex, calcium, and multiple vitamin supplements. His subsequent progress has been satisfactory, he has shown catchup growth (Fig. 1), is full of energy and is now attending regular school.
~Vivonex--an elemental fat-free formula. Eaton Laboratories.
Course and Treatment
Preliminary investigation Recurrent URI, diarrhea, hospitalized with increasing edema and hypoproteinemia Discharged following 20 days TPN Hospitalized for polypectomy Discharged post-polypectomy Stabilized on high protein, low fat, MCT diet Anorexia, emesis, diarrhea, abdominal cramps 5 days post 26 days TPN Fifth hospitalization in 7 mo; post-TPN, prior to intestinal resection 11 days after resection of 40 cm of jejunum 4 mo outpatient follow-up 81/2 mo post intestinal resection, clinical well 10 mo post-intestinal resection, clinical well
DISCUSSION
We are unable t o find any previous reference to colonic polyps occurring in a patient with small bowel intestinal lymphangiectasia. The excised polyp was a benign adenomatous lesion not associated with any m a l f o r m a t i o n of
135 130 125-
/ //"
120height, 115 cm
110 t 100
~ ee
f
8~t
Intestff, al resection
75,
70-
~olypectomy
I'
I
|
2 3
4
I
I
5 6
i
i
78
age, years Fig. 1.
Height growth record of the patient.
i
910
i
532
PARSONS AND PENCHARZ
Fig. 2. Left pedal lymphangiogram August, 1977 (note the dye remaining from the bi-pedal study done in August, 1975).
lymphatic tissue. The polyp was removed more because it was impossible to maintain the serum albumin than because of blood loss. We were unable to prove that there was a significant additional loss of protein from the polyp(s) in the face of the background of protein loss occurring in the small bowel. However, excision of the polyp did result in a dramatic resolution of his clinical status, which then remained stable for 1 yr. His second episode could be attributed to an increased loss of albumin in his small bowel which we hypothesize resulted in a vicious cycle. The reduction in serum albumin reduced serum oncotic pressure--one consequence of which was to further increase the edema of the bowel wall. If this edema was sufficient it would result in a
functional intestinal obstruction with vomiting. The failure of protein and energy intake would reduce liver albumin synthesis thus perpetuating the cycle. Certainly during two of his admissions for abdominal cramps, vomiting and diarrhea, thickened loops of bowel were readily palpated, although the barium studies showed no anatomical obstruction. Another consequence of his further reduced serum albumin was secondary hyperaldosteronism. His condition was then stabilized only by the use of continuous diuretics, salt poor albumin, and total parenteral nutrition. It was recognized that this was a precarious and unsatisfactory solution which led us to seek an alternative. Although it was firmly established that the lymphangectasia involved the whole of the small intestine, we were impressed by the favourable results of limited resection achieved by Kinmonth and Cox. s Therefore, we proposed a limited resection of the worst affected bowel, hoping that the resulting loss in absorptive surface would be insignificant while the reduction in the exudative enteropathy would be significant. In actuality, this did not prove to be the case, for 1 yr after surgery, intravenous chromium tagged albumen showed a loss of 15% of the administered dose over 96 hr, compared to 4% prior to operation. However, what it did do was relieve any further attacks of nausea, vomiting, emesis, and abdominal cramps and thus permit a high consistent dietary intake of protein. He no longer requires diuretics, his linear growth rate has increased and his general feeling of well being and activity is greater than at any previous time in his life. From our review of the literature this boy has the worst reported case of intestinal lymphangiectasia. His future is still guarded but his response to elective limited resection of the worst affected area of bowel has been dramatic.
REFERENCES
1. Gordon RS Jr: Exudative enteropathy. Lancet 1:325326, 1959 2. Pomerantz M, Waldmann TA: Systemic lymphatic abnormalities associated with gastrointestinal protein loss secondary to intestinal lymphangectasia. Gastroenterology 45:703-711, 1963 3. Waldmann TA: Protein-losing enteropathy. Gastroenterology 50:422-443, 1966 4. Holt PR: Dietary treatment of protein loss in intestinal lymphangectasia. The effect of eliminating dietary long chain triglycerides on albumin metabolismin this condition. Paediatrics 34:629-635, 1964
5. Tift WL, Lloyd JK: Intestinal lymphangectasia longterm results with MCT diet. Arch Dis Child 50:269-275, 1975 6. Muzaffar K, Block MA, Fox TA, Jr.: Primary intestinal lymphangectasia. Surgical implication. Am Surg 38:288-291, 1972 7. Vardy PA, Lebenthal E, Schwachman H: Intestinal lymphangectasia. A repraisal. Paediatrics 55:842-851, 1975 8. Kinmonth JB, Cox SJ: Protein-losing enteropathy in primary lymphoedema: mesenteric lymphography and gut resection. Br J Surg 61:589-593, 1974