226
Proc. roy. Soc. Med. Volume 69 March 1976
were present in large amounts. Serum thyroxine 70 nmol/l. TSH 12.5 mu/I. There was an excess of /3 and pre-/3 lipoproteins. Triglycerides 220 mg/ 100 ml. Four-hour radioiodine uptake was 17% of the ingested dose; after TSH injection of 2.5 units it was only 21 %, and after a further injection 18 %; this indicates no thyroid reserve. Discussion These two patients who presented with ischemic heart disease have the syndrome of premyxoedema. Patients with premyxcedema: (1) are clinically euthyroid; (2) have normal serum levels of thyroid hormones; (3) show evidence of thyroid disease, usually autoimmune thyroiditis (AIT), with thyroid antibodies or having had partial thyroid ablation by irradiation or surgery; (4) have a raised serum TSH; (5) usually have raised serum lipids and commonly present with coronary artery disease. It has been argued that the association of hypercholesterolhmia with AIT must be fortuitous if current serum thyroid hormone levels are normal (Lewis & Krikler 1971). This assumption is incorrect because the hypercholesterolmmia could be related, for example, to the raised serum TSH level. Since the original descriptions of premyxoedema (Fowler & Swale 1967, Fowler et al. 1970) 100 patients with the diagnosis of premyxcedema have been assessed (Ikram et al. 1973) and many more subsequently diagnosed; the two patients described here are typical examples. The analysis of 100 patients included measurement of the thyroid reserve by noting the response of the gland to TSH stimulation as described by Hobbs et al. (1963). The thyroid reserve is an indirect measurement of the subject's serum TSH level. Results of thyroid reserve tests and TSH levels usually agree. Occasionally the thyroid reserve is reduced when the serum TSH is normal. The thyroid reserve is therefore either more sensitive or less discriminatory than the direct measurement of the serum TSH. Subsequent studies measuring the rise of TSH after thyrotrophin releasing hormone (TRH) correlate well with Hobbs's test of the thyroid reserve. Some patients with premyxoedema have a normal TSH but exaggerated response to TRH. Clofibrate in premyxcedema invariably reduces the serum cholesterol to normal and has been used in these patients until they have progressed to myxcedema, when L-thyroxine has been started. Follow up will show whether coronary artery disease can be prevented in premyxcedema by keeping the serum lipids normal with clofibrate and in myxoedema by giving sufficient L-thyroxine to reduce the serum lipids to normal. This is in contrast to the adjustments of L-thyroxine dosage based on symptomatic relief or relying on the reduction of TSH levels alone.
10
.~ ~ .:,~ . '":S
REFERENCES Fowler P B S & Swale J (1967) Lancet i, 1077 Fowler P B S, Swale J & Andrews H (1970) Lancet ii, 488 Hobbs J R, Bayliss R I S & Maclagan N F (1963) Lancet i, 8 lkram H, Banim S & Fowler P B S (1973) Lancet ii, 1405 Lewis B & Krikler n M (1971) Lancet i, 1295
Diverticular Fistula between Sigmoid Colon and Sacral Cleft R A Hurlow' FRCS and B P Bliss MS FRCS (Department of Suirgery, Charing Cross Hospital, London W6 8RF)
Mrs I T, aged 67 History: Presented in June 1974 with pain over lower back and left sacroiliac area. No abnormality was visible and only degenerative changes were present on X-ray of lumbar spine. A steel reinforced corset was supplied but was abandoned after one wearing because of pain. Over the next two months pain continued and a tender lump appeared over the sacrum, associated with onset of watery diarrhea, with no blood or slime. On 21.8.74 the lump discharged foul-smelling pus, and the overlying skin ulcerated.
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~............. ,,,~~~~~~~~~~~~~.,.-''....s.,'
~~~~~~~~~~...............
I.:::
.. -. !*..;3!. |j.
.. .. ~ . . ~....~ ..! ~ESB
Fig 1 Ulcer beginning to heal 1Present address: Department of Surgery, Queen Elizabeth Hospital, Birmingham
j..
11
On examination: The patient was anaemic (haemoglobin 9.4 g/100 mnl), with an iron deficient picture, and slightly pyrexial (temperature 37.5°C). Abdominal and rectal.examination normal. Over the sacrum an ulcer of 2.5 cm diameter and an adjacent smaller ulcer were visible, with infected granulating bases. The surrounding skin was indurated, red and hot. Couirse: Local dressings failed to initiate improvement. 2.9.74: examined under anasthetic, ulcers debrided and connecting skin bridge removed; no deep track seen, vaginal and rectal examinations normal; sinography demonstrated only a subcutaneous cavity. Histology of removed skin: chronic granulation tissue with no evidence of tuberculosis or Crohn's disease. Subsequently the ulcer became cleaner and slow healing occurred, as shown in Fig 1. 21.10.74: particles of peas and carrots, from a recently consumed meal, appeared on the dressings. Second sinogram was performed and showed dye entering a subcutaneous cavity and passing through a fistula to rectum and descending colon (Fig 2). Barium enema (Fig 3) demonstrated a localized narrowing in sigmoid colon suggestive of a carcinoma, with sigmoid diverticula present. An IVP excluded left ureteric involvement. Operation (5.11.74): An inflammatory mass in the sigmoid colon was adherent to the left side of the
Fig 2 Sinogram showing dye entering suibcuitaneous cavity (A), passing througlhfistula to recti'm (B), and descending colon (c)
Clinical Section
227
Fig 3 Barium enema shows localized narrowing in sigmoid colon, with sigmoid diverticula
sacrum and pelvis. The left ureter passed through it but was intact. Diverticular disease involved the sigmoid and descending colon, but no carcinoma was found. A track of granulation tissue passed from the mass through the left greater sciatic notch. The sigmoid and descending colon were excised and a transverse colorectal anastomosis with right transverse colostomy was performed. A piece of omentum was interposed between the fistulous track and anastomosis. Progress: The colostomy was closed on 6.1.75. The sacral ulcer healed slowly and healing was complete by March 1975. Discussion There has been only one previous sigmoidsacral diverticular fistula reported (Goldfarb et al. 1964). In their case the fistulous opening was on the left buttock. Among reports of 1555 operated cases of diverticulitis reviewed by Colcok & Stahmann (1972) there were 296 fistule (19%). Of these, 77 were colocutaneous but only 10 occurred spontaneously, the remainder presenting postoperatively, although in some cases only after a considerable delay. In Colcok & Stahmann's own series of 64 fistulae of all types, 61 % followed surgical procedures, in particular simple drainage of an abscess, with or without colostomy but with no resection of the affected segment. They recommend one-stage resection of fistule in ideal circumstances, i.e. a well prepared colon with little or no acute inflammation or obstruction. However, in most cases a two- or threestage resection will be preferred.
228
12
Proc. roy. Soc. Med. Volume 69 March 1976
REFERENCES Colcok BP & Stahmann F D (1972) Annals of Surgery 175, 838-846 Goldfarb W B, Monafo W & McAlister W H (1964) American Journal oJfSurgery 108, 902
Adult Hypertrophic Pyloric Stenosis and Crohn's Disease R E Taor MB FRCS and A G Johnson Mchir FRCS (Professorial Unit of Suirgery, Charing Cross Hospital, London W6 8RF) Mrs H H, aged 57 This patient first presented at the hospital in 1960 with thyrotoxicosis, for which she underwent a subtotal thyroidectomy. At that time she had no gastrointestinal symptoms, and she has remained clinically and biochemically euthyroid since. In 1973 she presented again with a five-year history of epigastric pain, vomiting, episodic diarrhoea and weight loss. Barium meal investigation showed pyloric canal narrowing, but the stomach and duodenum were normal. Gastroscopy revealed a pin-hole pylorus and a normal stomach, it being impossible to enter the duodenum. Operation (14.1.73): Assuming a pyloric ulcer, selective vagotomy and pyloroplasty (HeinekeMikulicz) were performed but, on opening the pylorus, hypertrophic pyloric stenosis with no evidence of ulceration was found. Biopsy, important in excluding pyloric carcinoma, confirmed the diagnosis. The small and large intestine were noted to be normal. However a mucocele of the gallbladder with a gallstone impacted in Hartmann's pouch was found, leading to cholecystectomy following operative cholangiography. In other series of adult hypertrophic pyloric stenosis (Christiansen & Grantham 1962) 17 % of patients have had co-existing gallbladder disease or hiatus hernia. Following operation and during the next nine months, our patient continued to have symptoms of vomiting, unrelieved by metoclopramide; and diarrheea thought to be due mainly to the vagotomy. Reinvestigation by gastroscopy showed restenosis of the pylorus. After consultation of the available literature on adult hypertrophic pyloric stenosis (Keynes 1965) it was decided to excise the whole pyloric area and construct a gastroduodenal anastomosis - a Billroth 1 type of procedure. Again full histopathology of the whole pylorus confirmed the original diagnosis. Second operation (September 1975): The terminal ileum and mesentery were found to be red, thickened and cedematous; a right hemicolectomy was performed. Histopathology confirmed the
diagnosis of Crohn's disease and led to a careful reexamination of the pylorus, but revealed no evidence of Crohn's disease to explain the pyloric narrowing. Since her second operation this patient has remained fit and well, with no symptoms of obstruction and requiring diphenoxylate only occasionally to control a tendency to loose and frequent motions.
Commenit This patient has presented with four clinical conditions: thyrotoxicosis, adult hypertrophic pyloric stenosis, gallstones, and Crohn's disease. Three of these conditions could explain the diarrheea, although her present mild and improving symptom may well be due to the operation of vagotomy; it is hoped it will completely abate in time. A number of operations have been used in the treatment of adult hypertrophic pyloric stenosis, including simple dilatation, a Rammstedt procedure, gastroenterostomy, pyloroplasty and a limited distal partial gastrectomy. Certainly a surgical procedure is necessary, positively to exclude complicating disease (especially carcinoma), and more importantly to relieve the obstructing symptoms. Most fall short by failing to relieve fully the obstruction. The limited gastrectomy is now favoured (Keynes 1965), because it excises the whole affected area; but a pyloroplasty of the Finney type has had some success. In this patient's case, a HeinekeMikulicz type, of pyloroplasty was performed initially and was inadequate. In our patient the adult hypertrophic pyloric stenosis must on the evidence be regarded as primary and in treatment excision of the whole pylorus is stressed. REFERENCES Christiansen K H & Grantham A (1962) Archi ves of Surgery 85, 207-214 Keynes W M (I 965) Gut 6, 240-251
The following cases were also presented: Arthritis and Liver Disease Dr D B Yates (for Dr J T Scott) Late Presentation of a Congenitally Malrotated Cmcum Mr R W Hoile (for Mr K W Reynolds) Late Infection in a Dacron Aortic Bifurcation Graft Mr D R Harvey (for Mr B P Bliss) Hodgkin's Disease Dr B Backhouse (for Dr D O'Connell) Carcinoma of Breast, Postherpetic Neuralgia, Hiatus Hernia and CEsophageal Stricture Dr S Keeton (for Dr N Howard)
Proc. roy. Soc. Med. Volume 69 March 1976
were present in large amounts. Serum thyroxine 70 nmol/l. TSH 12.5 mu/I. There was an excess of /3 and pre-/3 lipoproteins. Triglycerides 220 mg/ 100 ml. Four-hour radioiodine uptake was 17% of the ingested dose; after TSH injection of 2.5 units it was only 21 %, and after a further injection 18 %; this indicates no thyroid reserve. Discussion These two patients who presented with ischemic heart disease have the syndrome of premyxoedema. Patients with premyxcedema: (1) are clinically euthyroid; (2) have normal serum levels of thyroid hormones; (3) show evidence of thyroid disease, usually autoimmune thyroiditis (AIT), with thyroid antibodies or having had partial thyroid ablation by irradiation or surgery; (4) have a raised serum TSH; (5) usually have raised serum lipids and commonly present with coronary artery disease. It has been argued that the association of hypercholesterolhmia with AIT must be fortuitous if current serum thyroid hormone levels are normal (Lewis & Krikler 1971). This assumption is incorrect because the hypercholesterolmmia could be related, for example, to the raised serum TSH level. Since the original descriptions of premyxoedema (Fowler & Swale 1967, Fowler et al. 1970) 100 patients with the diagnosis of premyxcedema have been assessed (Ikram et al. 1973) and many more subsequently diagnosed; the two patients described here are typical examples. The analysis of 100 patients included measurement of the thyroid reserve by noting the response of the gland to TSH stimulation as described by Hobbs et al. (1963). The thyroid reserve is an indirect measurement of the subject's serum TSH level. Results of thyroid reserve tests and TSH levels usually agree. Occasionally the thyroid reserve is reduced when the serum TSH is normal. The thyroid reserve is therefore either more sensitive or less discriminatory than the direct measurement of the serum TSH. Subsequent studies measuring the rise of TSH after thyrotrophin releasing hormone (TRH) correlate well with Hobbs's test of the thyroid reserve. Some patients with premyxoedema have a normal TSH but exaggerated response to TRH. Clofibrate in premyxcedema invariably reduces the serum cholesterol to normal and has been used in these patients until they have progressed to myxcedema, when L-thyroxine has been started. Follow up will show whether coronary artery disease can be prevented in premyxcedema by keeping the serum lipids normal with clofibrate and in myxoedema by giving sufficient L-thyroxine to reduce the serum lipids to normal. This is in contrast to the adjustments of L-thyroxine dosage based on symptomatic relief or relying on the reduction of TSH levels alone.
10
.~ ~ .:,~ . '":S
REFERENCES Fowler P B S & Swale J (1967) Lancet i, 1077 Fowler P B S, Swale J & Andrews H (1970) Lancet ii, 488 Hobbs J R, Bayliss R I S & Maclagan N F (1963) Lancet i, 8 lkram H, Banim S & Fowler P B S (1973) Lancet ii, 1405 Lewis B & Krikler n M (1971) Lancet i, 1295
Diverticular Fistula between Sigmoid Colon and Sacral Cleft R A Hurlow' FRCS and B P Bliss MS FRCS (Department of Suirgery, Charing Cross Hospital, London W6 8RF)
Mrs I T, aged 67 History: Presented in June 1974 with pain over lower back and left sacroiliac area. No abnormality was visible and only degenerative changes were present on X-ray of lumbar spine. A steel reinforced corset was supplied but was abandoned after one wearing because of pain. Over the next two months pain continued and a tender lump appeared over the sacrum, associated with onset of watery diarrhea, with no blood or slime. On 21.8.74 the lump discharged foul-smelling pus, and the overlying skin ulcerated.
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~............. ,,,~~~~~~~~~~~~~.,.-''....s.,'
~~~~~~~~~~...............
I.:::
.. -. !*..;3!. |j.
.. .. ~ . . ~....~ ..! ~ESB
Fig 1 Ulcer beginning to heal 1Present address: Department of Surgery, Queen Elizabeth Hospital, Birmingham
j..
11
On examination: The patient was anaemic (haemoglobin 9.4 g/100 mnl), with an iron deficient picture, and slightly pyrexial (temperature 37.5°C). Abdominal and rectal.examination normal. Over the sacrum an ulcer of 2.5 cm diameter and an adjacent smaller ulcer were visible, with infected granulating bases. The surrounding skin was indurated, red and hot. Couirse: Local dressings failed to initiate improvement. 2.9.74: examined under anasthetic, ulcers debrided and connecting skin bridge removed; no deep track seen, vaginal and rectal examinations normal; sinography demonstrated only a subcutaneous cavity. Histology of removed skin: chronic granulation tissue with no evidence of tuberculosis or Crohn's disease. Subsequently the ulcer became cleaner and slow healing occurred, as shown in Fig 1. 21.10.74: particles of peas and carrots, from a recently consumed meal, appeared on the dressings. Second sinogram was performed and showed dye entering a subcutaneous cavity and passing through a fistula to rectum and descending colon (Fig 2). Barium enema (Fig 3) demonstrated a localized narrowing in sigmoid colon suggestive of a carcinoma, with sigmoid diverticula present. An IVP excluded left ureteric involvement. Operation (5.11.74): An inflammatory mass in the sigmoid colon was adherent to the left side of the
Fig 2 Sinogram showing dye entering suibcuitaneous cavity (A), passing througlhfistula to recti'm (B), and descending colon (c)
Clinical Section
227
Fig 3 Barium enema shows localized narrowing in sigmoid colon, with sigmoid diverticula
sacrum and pelvis. The left ureter passed through it but was intact. Diverticular disease involved the sigmoid and descending colon, but no carcinoma was found. A track of granulation tissue passed from the mass through the left greater sciatic notch. The sigmoid and descending colon were excised and a transverse colorectal anastomosis with right transverse colostomy was performed. A piece of omentum was interposed between the fistulous track and anastomosis. Progress: The colostomy was closed on 6.1.75. The sacral ulcer healed slowly and healing was complete by March 1975. Discussion There has been only one previous sigmoidsacral diverticular fistula reported (Goldfarb et al. 1964). In their case the fistulous opening was on the left buttock. Among reports of 1555 operated cases of diverticulitis reviewed by Colcok & Stahmann (1972) there were 296 fistule (19%). Of these, 77 were colocutaneous but only 10 occurred spontaneously, the remainder presenting postoperatively, although in some cases only after a considerable delay. In Colcok & Stahmann's own series of 64 fistulae of all types, 61 % followed surgical procedures, in particular simple drainage of an abscess, with or without colostomy but with no resection of the affected segment. They recommend one-stage resection of fistule in ideal circumstances, i.e. a well prepared colon with little or no acute inflammation or obstruction. However, in most cases a two- or threestage resection will be preferred.
228
12
Proc. roy. Soc. Med. Volume 69 March 1976
REFERENCES Colcok BP & Stahmann F D (1972) Annals of Surgery 175, 838-846 Goldfarb W B, Monafo W & McAlister W H (1964) American Journal oJfSurgery 108, 902
Adult Hypertrophic Pyloric Stenosis and Crohn's Disease R E Taor MB FRCS and A G Johnson Mchir FRCS (Professorial Unit of Suirgery, Charing Cross Hospital, London W6 8RF) Mrs H H, aged 57 This patient first presented at the hospital in 1960 with thyrotoxicosis, for which she underwent a subtotal thyroidectomy. At that time she had no gastrointestinal symptoms, and she has remained clinically and biochemically euthyroid since. In 1973 she presented again with a five-year history of epigastric pain, vomiting, episodic diarrhoea and weight loss. Barium meal investigation showed pyloric canal narrowing, but the stomach and duodenum were normal. Gastroscopy revealed a pin-hole pylorus and a normal stomach, it being impossible to enter the duodenum. Operation (14.1.73): Assuming a pyloric ulcer, selective vagotomy and pyloroplasty (HeinekeMikulicz) were performed but, on opening the pylorus, hypertrophic pyloric stenosis with no evidence of ulceration was found. Biopsy, important in excluding pyloric carcinoma, confirmed the diagnosis. The small and large intestine were noted to be normal. However a mucocele of the gallbladder with a gallstone impacted in Hartmann's pouch was found, leading to cholecystectomy following operative cholangiography. In other series of adult hypertrophic pyloric stenosis (Christiansen & Grantham 1962) 17 % of patients have had co-existing gallbladder disease or hiatus hernia. Following operation and during the next nine months, our patient continued to have symptoms of vomiting, unrelieved by metoclopramide; and diarrheea thought to be due mainly to the vagotomy. Reinvestigation by gastroscopy showed restenosis of the pylorus. After consultation of the available literature on adult hypertrophic pyloric stenosis (Keynes 1965) it was decided to excise the whole pyloric area and construct a gastroduodenal anastomosis - a Billroth 1 type of procedure. Again full histopathology of the whole pylorus confirmed the original diagnosis. Second operation (September 1975): The terminal ileum and mesentery were found to be red, thickened and cedematous; a right hemicolectomy was performed. Histopathology confirmed the
diagnosis of Crohn's disease and led to a careful reexamination of the pylorus, but revealed no evidence of Crohn's disease to explain the pyloric narrowing. Since her second operation this patient has remained fit and well, with no symptoms of obstruction and requiring diphenoxylate only occasionally to control a tendency to loose and frequent motions.
Commenit This patient has presented with four clinical conditions: thyrotoxicosis, adult hypertrophic pyloric stenosis, gallstones, and Crohn's disease. Three of these conditions could explain the diarrheea, although her present mild and improving symptom may well be due to the operation of vagotomy; it is hoped it will completely abate in time. A number of operations have been used in the treatment of adult hypertrophic pyloric stenosis, including simple dilatation, a Rammstedt procedure, gastroenterostomy, pyloroplasty and a limited distal partial gastrectomy. Certainly a surgical procedure is necessary, positively to exclude complicating disease (especially carcinoma), and more importantly to relieve the obstructing symptoms. Most fall short by failing to relieve fully the obstruction. The limited gastrectomy is now favoured (Keynes 1965), because it excises the whole affected area; but a pyloroplasty of the Finney type has had some success. In this patient's case, a HeinekeMikulicz type, of pyloroplasty was performed initially and was inadequate. In our patient the adult hypertrophic pyloric stenosis must on the evidence be regarded as primary and in treatment excision of the whole pylorus is stressed. REFERENCES Christiansen K H & Grantham A (1962) Archi ves of Surgery 85, 207-214 Keynes W M (I 965) Gut 6, 240-251
The following cases were also presented: Arthritis and Liver Disease Dr D B Yates (for Dr J T Scott) Late Presentation of a Congenitally Malrotated Cmcum Mr R W Hoile (for Mr K W Reynolds) Late Infection in a Dacron Aortic Bifurcation Graft Mr D R Harvey (for Mr B P Bliss) Hodgkin's Disease Dr B Backhouse (for Dr D O'Connell) Carcinoma of Breast, Postherpetic Neuralgia, Hiatus Hernia and CEsophageal Stricture Dr S Keeton (for Dr N Howard)
