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Ptosis of cecum and ascending colon
PTOSIS OF CECUM AND ASCENDING ARVID
COLON
C. SILVERBERG, M.D. SEATTLE
DEFINITION
P
TOSIS may be defined as a prolapse downward of one or more of the abdomina1 viscera to such an extent as to change materiahy the normaI reIationship to one another and to the adjacent structures, especiahy when the subject is in the erect posture. ETIOLOGY
Predisposing causes : (a) Arrested deveIopment (b) MaIdeveIopment (c) SkeIetaI posture, shape of abdomina1 cavity, strength of abdomina1 waI1 2. Exciting causes: (a) Intra-abdomina1 pressure (b) Occupation To understand maIdeveIopments it is essentia1 to have at Ieast a rudimentary knowIedge of norma deveIopment. The aIimentary tract is deveIoped from the entodermic vesicIe of the zygote.2 This vesicIe becomes divided into an intraembryonic and an extra-embryonic portion. The intra-embryonic portion becomes the aIimentary tract and appendages. About the hfth week the aIimentary tract can be differentiated into three main subdivisions, the foregut, the midgut and the hindgut. The divisions are made on form, bIood suppIy, and function. Each part has a separate bIood supply. The foregut forms the gastrointestina1 tract beIow the ampuha of Vater to the dista1 portion of the transverse coIon with its circuIation supphed by the superior mesenteric artery. The hindgut is cauda1 to the transverse coIon and is suppIied by the inferior mesenteric artery. The function of the foregut is digestive; of the midgut absorptive; and of the hindgut excretory. DeveIopmentaI error is common in the I.
midgut whiIe in the foregut and hindgut maIformation is of rare occurrence. The viteIIine duct ordinariIy becomes detached about the fourth week of Iife. The rapid increase of the size of the Iiver of the midgut is out of proportion with that of the rest of the abdomen. The peritonea1 cavity cannot hoId a11 the structures, and the midgut is therefore pushed out through the umbiIicus, producing a physioIogic umbiIica1 hernia, The apex of the herniated Ioop is at the former attachment of the viteIIo-intestina1 duct and the termination of the superior mesenteric artery, which originaIIy was the right omphaIomesenteric artery. The artery Iies in the mesentery and sends branches to the anterior and posterior segments. The mesentery of the herniated Ioop Dott divides into a pre-arteria1 and a postarteria1 portion. The gut in this stage occupies a sagitta1 pIane. The rotation of the midgut occurs between the fifth and tenth week and may be divided into three stages. In the first stage the prearteria1 segment is on the right side and the post-arteria1 on the Ieft. The rotation is necessitated by the emargement and the descent of the liver and pressure of the umbiIica1 artery. The pre-arteria1 portion and the mesentery become disproportionateIy Iong. An enIargement in the postarteria1 segment can be seen in an embryo from twenty-seven to thirty days oId. This is the beginning of the deveIopment of the cecum and appendix. The second stage of rotation begins about the beginning of the tenth week when the gut returns to the abdomen. Ma11 suggests that the increase in the Iength of the Ioops and their rotation in the abdomina1 cavity produce enough traction to repIace the extra-abdomina1 intestina1 Ioops within the abdomen. The
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interna orifice of the umbiIica1 cana is comparativeIy smaI1, making it impossibIe for the entire contents of the hernia to return to the abdomina1 cavity en masse. The greater size of the cecum may offer sufficient resistance to retain it in the hernia1 sac, thus permitting the pre-arteria1 segment to return first. In doing so, it passes behind the superior mesenteric artery which extends from the aorta to the umbiIicus. The smaI1 intestine as it enters the abdomen pushes the intra-abdomina1 smaI1 gut and mesentery, which has been occupying the midIine, to the Ieft and posterior portion of the abdomina1 cavity. The cecum is the Iast to be reduced into the abdomina1 cavity. It occupies a position in the region of the umbilicus, anterior to the smaI1 intestines and superior mesenteric artery and beneath the Iiver. The cecum continues to descend to Iie in the norma aduIt position in the iIeoceca1 fossa. In the tweIfth week the superior mesenteric artery, which is brought to the iIiac region by the migration of the cecum, Iies in intimate contact with the posterior parietal peritoneum and becomes adherent from above downwards to the posterior abdomina1 waI1. AIong the right side of the artery the ascending coIon and cecum become fixed. The post-arteria1 mesenteric segment persists as the mesocoIon. The pre-arteria1 segment remains free as the mesentery of the smaI1 intestine. The mesentery of the hindgut becomes obIiterated by fusion aIong the midIine with the posterior parieta1 peritoneum. DEVELOPMENTAL
ANOMALIES
MaIrotation and non-rotation depend upon the sequence with which the intestina Ioops return to the abdomen. As a resuIt of non-rotation the smaI1 intestine lies chiefly to the right. The duodenum passes to the right of the superior mesentery artery. The colon is Iocated entireIy on the Ieft side and does not cross the superior mesenteric artery. Norma1 fixation or absence of fixation, is frequentIy encountered, producing an abnormaIIy
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mobiIe sigmoid or cecum. Non-rotation is the resuIt of a return of the cecum and the post-arteria1 segment to the abdominal cavity before return of the pre-arterial segment. Reverse rotation may take pIace but it is of IittIe significance cIinicaIIy, because the various parts of the intestina1 tract have normaI reIationships, except that they are in reverse. In maIrotation of the midgut, the pre-arteria1 segment of the midgut passes in front of the superior mesenteric artery and the posterior segment incIuding the cecum aIso passes in front of the origin of the artery. The mesentery does not become adherent to the posterior abdomina1 waI1. The duodenum and iIeum Iie side by side in front of the superior mesenteric vesseIs. The various congenita1 anomahes are due to the variation in the size of the embryonic umbiIica1 orifice. Most cIinica1 anomalies, however, arise from fauIty third stage rotation. The third stage rotation consists IargeIy of fusion of the various mesenteries with the posterior parietal peritoneum, making the various parts of the gastrointestina1 tract Iess mobiIe. EarIy fixation or deficient fixation with the exception of the eIongation of the coIon produce definite anomaIous conditions, The most important of these, pathoIogicaIIy, are those resuIting from deficient fixation of the coIon. The position of the retroceca1 appendix can easiIy be expIained if one remembers the descent and fusion of the cecum and ascending colon to the parieta1 peritoneum. The appendix can be turned back behind the descending cecum, becoming enclosed in the mesocoIon with the parietal peritoneum. On th e other hand, if the, appendix takes a position anterior to the cecum in the descent of the coIon it will have a free position in the abdominal cavity. EXCITING
CAUSES
If the occupations of ptotic people require that they be on their feet and
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especiaIIy if they have to do Iifting, the condition progresses from bad to worse. Through tke I&s of weight and of the fat
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supporting the abdomina1 viscera is aImost a negligible one. - Long continued intrathoracic conditions,
FIG. I.
FIG.
FIG. 3. FIGS. I TO 4. Stages of deveIopment
in the meshes of the mesentery greater eIongation occurs, causing a sagging of the abdomina1 viscera. A reIaxed abdomina1 waII after Iong wasting disease may be foIIowed by a visceroptosis. The reIaxation of the IateraI muscIe groups, that is, the transversaIis and the oblique, than that of the recti abdominis, which determines the condition. The part pIayed by the peIvic Aoor in
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and rotation
2.
FIG. 4. of the midgut.
new growths and, at as Iarge exudates, times, pneumothorax may cause ptosis of the abdomina1 viscera. In this condition the diaphragm is fixed in the inspiratory position and thus renders its muscuIar opponents, the muscles of the abdomina1 waI1, useIess. Dr. Arthur Keith has made an embryoIogic and anatomic study of this condition and states that the abdominal muscIes in thoracic respiration act as
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opponents to the diaphragm. In a sheIflike manner they support the abdomina1 viscera during inspiration and aid the diaphragm in the eIevation of the ribs and thorax. In reIaxation of the IateraI muscIes of the abdomina1 waI1 this norma reIationship is Iost. The tendency to ptosis often runs in families, various members showing a phthisica1 chest and’giving a history of ancestra1 tubercuIosis. PATHOLOGIC
ANATOMY
AND
PHYSIOLOGY
In the norma position of the cecum and ascending coIon in the iIeoceca1 fossa, where proper fusion of the post-arteria1 segment has taken pIace, they get support from what Coffey caIIs the iIiopsoas sheIf. The center of gravity passes through the ascending coIon and cecum, we11 posterior to the anterior border of the psoas muscIe. In deficient fusion with a Iong mesocoIon the weight of the intestine forces it into the peIvis, causing a gradua1 eIongation of the mesentery. Nature attempts to correct the defect by Iaying down a membrane which graduaIIy becomes more dense, and often forming bands which become painfu1 when puIIed upon by the distended cecum. These bands produce stagnation by kinking the ascending coIon in the region of the hepatic ffexure. A child born with a defective prenata1 fixation of the abdomina1 viscera wiI1 have gastrointestina1 upsets from birth on. After adoIescence, with.the greater activity of the chiId, a gradua1 deveIopment of the typica ptotic figure is the ruIe. The colon, dropping from its norma Iocation, sIides off the psoas sheIf into the peIvis. The kidney fossa becomes shaIIower. The kidney often sIides down. The drag on the bottom of the stomach causes dispIacement of the pyIoric end of the stomach, retarding the gastric contents. This causes indigestion with subsequent Ioss of ffesh and further reduces the viscera1 support by decreasing the intra-abdomina1 pressure. Gas and ffuid in the gastrointestina1 tract are increased. The stretching of the
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gastro-intestina1 muscIes causes a gradua1 reduction of peristaIsis. The efficiency of digestion is generaIIy Iowered. The abdomina organs tend to gravitate more and more to the Iower portion of the abdomen and peIvis. The drag on the diaphragm and the fascia attached to the ribs causes the Iower portion of the chest to become narrow and the angIe of the ribs with the vertebrae to become sharper. The Iower end of the spine and the peIvis are tiIted backwards in an effort to hoId the Ioad by means of the thoracic fascia. The norma curvature of the spine disappears, and the spine becomes reIativeIy straight with the Iower end tiIted reIativeIy forward and upward and the upper part backward. The drag on the viscera1 nerves and sympathetic pIexus materiaIIy affects the nervous stabiIity of the patient, and these individuaIs are often cIass&ed as neurasthenics. The intestina1 stasis and resuIting autointoxication extending over years causes a depletion of the adrenaIs with a gradua1 Iowering of the bIood pressure, increasing weakness and making for easy exhaustion on physica and menta1 exertion. SYMPTOMATOLOGY
The discussion of symptoms is here Iimited to those of right sided abdomina1 ptosis onIy, the ptosis of the cecum and ascending coIon. This condition is, practicaIIy without exception, due to a congenita1 defect, the cecum and ascending coIong having faiIed to fuse properIy with the posterior parieta1 peritoneum. The symptoms generaIIy appear at the time of adoIescence and are often diagnosed as intestina1 colic. There are attacks of right sided abdomina1 pain which may Iast onIy a short time or may Iast for days, with rest in bed necessary before they subside. As time goes on the cecum and ascending coIon become Iarger and their contents are Iess easiIy expeIIed. Nature meanwhiIe tries to come to the rescue by Iaying down supporting mem-
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branes aIong the Iine of stress from the right Iateral waI1 of the abdomina1 cavity to the cecum and ascending coIon. These
CUffEn...... 75% FIG. j.
/nrno VZD
2s
%
M087UTrL. 0%
Tabulation of cases with prominent toms, treatment and resuIt.
symp-
membranes stand out as dense white bands, often kinking the bowe1 in the region of the hepatic ffexure, stiI1 further impeding the movements of the intestina contents. The appendix in these cases is apt to become infected because of the stagnation in the coIon. RemovaI of the appendix gives IittIe or no relief. There is vomiting after the pain has started, not at the beginning as in an attack of acute appendicitis. There may be fever of a degree or so if the stasis is considerabIe. On physica examination we often find a paIpabIe cecum and an ascending coIon which contains gas and ffuid. There is tenderness aIong the attachment of the membranous bands. In severe cases we find a ptotic figure and signs of neurasthenia. A barium meal shows a Iow lying cecum and often a retention of barium for days. A barium enema wiII show the same and, if there are tight bands, deIay of the barium enema at the region of the hepatic ffexure can be seen. DIAGNOSIS
In the differentia1 diagnosis we may consider a11 right sided abdomina1 conditions which give rise to pain, vomiting and
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toxemia, as we11 as right sided kidney Iesions and, in women, right peIvic Iesions. The succussion spIash in the cecum is said to be pathognomonic of a redundant cecum with stasis. GastrointestinaI x-ray studies are concIusive, but a diagnosis can generaIIy be made without the aid of the x-ray. The history of right sided abdomina1 pain from the time of adoIescence which is apt to get worse on physica exertion especiaIIy when the patient has to be on his feet for any Iength of time is characteristic. This pain may become worse with each attack. Pain occurring during or after an evening of dancing is very suggestive. The Iocation of the pain is higher than the average appendix. It is described as a dragging or a heavy pain in the right side or, if partia1 obstruction has occurred, as coIicky. PROGNOSIS
Due to the stasis and irritation produced by decomposition a coIitis is set up which in advanced cases spreads from the ptotic portion of the coIon. The symptoms are thus far in excess of what one wouId expect from the origina pathoIogy. After the correction of the pathoIogic anatomy the coIitis must be treated. Otherwise, the prognosis is good. TREATMENT
MedicaI management consists in reIieving the intestina1 stasis and attempting to shorten the mesentery by deposition of fat in the mesenteric meshes. The patient shouId be kept in bed with the foot of the bed raised and put on forced feeding, sometimes for months. A gain in weight may reIieve a11 symptoms. The shortened mesentery supports the intestines we11 enough to prevent kinking. If the weight can be maintained after a return to usua1 activities, good heaIth may continue, but generaIIy a gradua1 Ioss of weight causes return of the symptom compIex. BeIts and abdomina1 supports may be vaIuabIe in gastroptosis and genera1 ptosis
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but I beIieve they are worse than useIess in right sided ptosis, except for their psychic effect. SurgicaI correction shouId be carried out before a marked coIitis has set in. SurgicaI treatment is in most cases successful Preoperative management requires emptying of the coIon with a miId Iaxative and enemata. HospitaIization for some days prior to the operation is advisabIe. IntraperitoneaI vaccination is aIso vaIuabIe, especiaIIy if there is concomitant coIitis. In debiIitated patients, a buiId up program before operation shouId be carried out. Transfusion is heIpfu1. SpinaI anesthesia gives exceIIent reIaxation and shouId alIow suficient time. The operation can be performed in Iess than an hour. Ether anesthesia, where there is no contraindication, is satisfactory. A right pararectus incision is made, of sufhcient Iength to enabIe one to expose the hepatictflexure and the cecum. The bands and adhesions are reIeased and the appendix is removed. An incision is made IateraI to the cecum and ascending coIon as high as the hepatic ffexure, this being the IateraI Ieaf of the ascending mesocoIon. The peritoneum is reffected IateraIIy, exposing the perirena1 fat, practicaIIy a bIoodIess dissection. Care is taken not to injure the ureter. Sutures of chromic catgut or duIax are passed through the edge of the reflected peritonea1 foId, the first through the Iower end of the reflected foId and the region of the appendicea1 stump. The foIIowing sutures are inserted $6 inch to I inch apart through the anterior IongitudinaI band of the cecum and ascending coIon. I have found it advantageous to Ieave the sutures untied unti1 they are a11 inserted. When the sutures are tied the penduIous cecum and ascending colon roI1 into the denuded space where they soon wiI1 become firmIy attached. As there generaIIy is a coexisting ptosis of the right kidney, the kidney wiI1 become fixed in its proper position. At times the bands and membranes which have been formed in nature’s attempt to reIieve the oathoIogic condition can be
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used to strengthen the anchoring of the ascending coIon. If there stiI1 remains a sharp kinking at the hepatic ffexure the incision can be curved around and the condition corrected by suitabIy pIaced sutures. In cases where there is a Iong transverse coIon which faIIs down into the peIvis, dragging on the hepatic fIexure, I anchor it to the anterior abdomina1 waI1 media1 to the hepatic Aexure. Sutures are inserted through the root of the omentum and parieta1 peritoneum of the anterior abdomina waI1. This is what Coffey describes as his hammock operation. It can be extended right across the abdomina1 cavity if necessary and wiI1 give support to a ptotic stomach. These patients have remarkabIe IittIe postoperative disturbance. The reIief of the partia1 obstruction and the absence of intestina1 toxemia give them a feeIing of we11 being. They can Ieave the hospita1 when the wounds are heaIed and are permitted to resume active Iife as after any other simiIar abdomina1 operation. CoIitis, if present, is treated by diet and other measures. The patient often expects to be compIeteIy cured and does not coiiperate for a Iong enough time to have the coIon return to normaI. In cases where a portion of the coIon is much invoIved it might be wise to do a partia1 coIectomy. CASE
REPORTS
I have operated on tweIve patients and have seen a number of other cases in which no operation has been done, but in which has been estabIished by the diagnosis radiographic study. The tweIve operations have produced highIy satisfactory resuIts, with ten compIete cIinica1 cures and two much improved. The coIitis which existed in the Iatter two cases necessitates a more or Iess strict dietary regime. They are, however, reIieved of the toxemia and the symptoms associated with the partia1 TypicaI case histories are obstruction. appended.
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CASE I. A. S., maIe, age 20, compIained of right sided abdomina1 pain occurring at intervals of a few weeks, generalIy foIIowing dietary indiscretion or excessive physica exertion. The symptoms first appeared at the age of 14 and had since graduaIIy increased in severity. Vomiting occurred with the attacks, but rareIy fever. The pain lasted for two or three days. A diagnosis of appendicitis had been made. There was a paIpabIe cecum containing gas and ffuid. The Ieucocyte count was 10,000, the red count 4,400,000. A barium mea1 reveaIed a Iow lying cecum with stasis and probabIe kinking at the hepatic Aexure. At operation the appendix was found to be normal, but there was a we11 developed Jackson’s membrane with tight bands at the hepatic flexure. The cecum and ascending coIon were distended and penduIous. The appendix was removed and a Waugh operation was performed. The postoperative course was uneventfu1 and compIete recovery foIIowed. CASE II. A. N., maIe, age 28, had pain in the abdomen especiaIIy in the right side, fever, vomiting, constipation, attacks of diarrhea, and a rash which he had been toId might be due to syphilis. There was a paIpabIe mass in the right side of the abdomen, movable from side to side, and containing fIuid and gas. The temperature was 103O, the Wassermann negative, Ieucocytes 12,000. The urine was normaI. A diagnosis of intestinal stasis with toxemia was made. The intestina1 stasis was relieved, the symptoms disappeared, and the patient returned to work. One month later the condition recurred, and the patient was operated on. A Iarge and pendulous cecum and ascending colon were found with bands between the ascending coIon and the IateraI waI1 of the abdomen. The appendix showed no gross changes. The appendix was removed and a Waugh operation performed. Smooth recovery ensued, with no return of symptoms. CASE III. N. A., femaIe, age 25, had had periodic right sided abdominal pain for the previous ten years. It was apt to come on after exertion, such as dancing, but disappeared overnight or in a day or two. She was easiIy fatigued, had frequent headaches and was physically below par. Gas and ffuid could be made out in the cecum and ascending colon. There was tender-
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ness aIong the ascending coIon. A barium mea1 revealed a pendulous cecum with deIayed emptying of the cecum and ascending colon. Tight bands at the hepatic ffexure were found at operation. The appendix was apparentIy normal. It was removed, the bands cut, and a Waugh operation performed. ConvaIescence was uneventfu1 and recovery compIete. CASE IV. H. P., femaIe, age 25, had abdomina1 pains from 12 years of age. An appendectomy was done at the age of 20, when she was told that the appendix was norma but there was some peIvic condition causing her troubIe (“fibroid uterus”). Two years Iater she was operated on for an ovarian cyst. The right sided abdomina1 pain with constipation and toxemia persisted, however, and sometimes confined her to bed for days at a time. Genera1 debiIity and neurasthenia were noted. Gastrointestinal radiographic study reveaIed stasis in the cecum and ascending coIon. A long penduIous cecum rested on the peIvic floor. There were probabIy adhesions from the previous operation. The transverse coIon was Iong and occupied the pelvis. The coIon was kinked at the hepatic ffexure. A Iong pararectus incision was made on the right side. There were adhesions between the cecum and the old appendectomy scar, as we11 as bands and membranes between the cecum and ascending colon and the right side of the abdomina1 waI1. The coIon was inflamed and edematous. The adhesions were freed and coIopexy performed with the incision curving around the hepatic ffexure. Sutures were pIaced in the root of the Iarge omentum and to the anterior abdomina1 wall media1 to the hepatic fIexure halfway across the abdomen. In this case there was marked coIitis extending practically half way across the transverse colon and very marked at the hepatic fIexure. The patient’s postoperative course was smooth, a diet had to be followed. However, the stasis was reIieved and if there is no dietary indiscretion the patient’s health is good. CASE v. W. G., male, age 34, had periodic abdomina1 pain for several years which at times forced him to bed. He felt better when he kept his boweIs moving with laxatives and enemata. He was brought to the hospita1 as an emergency in the middle of the night with a fever of 103, marked tenderness and rigidity over the right side of the abdomen. Tenderness was equally marked under the right costal
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margins and over the right lower quadrant. Leucocytes numbered I 8,000, with go per cent polymorphonuclears. A long right pararectus incision made. An acutely inflamed appendix with a distended cecum and ascending colon and tight bands at the hepatic ffexure causing almost complete obstruction were observed. The ascending coIon was inffamed. The appendix was removed, the bands cut and a modified right sided colopexy done. A stormy convalescence for the first few days was followed by complete recovery. CASE VI. M. C., female, age 20, had had pain since the age of 13 or 14 which had gradually grown worse. She was afraid to go to dances because of the pain which followed. The attacks were aIways associated with vomiting, rarely by fever. There had been considerable loss of weight. One year before an appendectomy had been performed, but a Iong convalescence had been followed by no improvement. There was a paIpabIe cecum and ascending coIon, tenderness over the right side of the ascending colon. BIood and urinary findings were normal. Radiographic study showed six hour retention in the stomach, a Iarge penduIous
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cecum and colon, and apparentIy bands at the hepatic flexure. Operation confirmed the findings of the radiographic study. Adhesions between the pylorus, duodenum and hepatic ffexure and Jackson’s membrane were noted. The adhesions were released and a colopexy performed. Smooth convalescence followed. REFERENCES I. OCHSNER, ALTON. NeIson Loose-Leaf Surgery, Vol. v. 2. Dorr, N. M. &it. J. Surg., I I : 251, 1923. 3. COFFEY, R. C. In Dean Lewis. Practice of Surgery, VOI. VI. 4. CALLANDER, C. L. SurgicaI Anatomy. PhiIa., 1933. Saunders. 5. ROBERTSON, W. In Sajous. AnaIytic CycIopedia of Practical Medicine. 6. PATTENGER, F. M. South. California Pratt., March, 1912. 7. KEITH, ARTHUR. In Sajous. AnaIytic
8. g. IO. I I. 12.
CycIopedia of Practical Medicine, VoI. v, p. 776. RANKIN, F. W. Surgery of the CoIon. New York, 1926. AppIeton & Co. ALVAREZ, W. C. The Mechanics of the Digestive Tract. Hoeber. SHARP, H. V. Am. J. Surg., 24: 94-99. 1934. SMITH, J. W. Med. Chronicle, May, 1913. MALL. Textbook of EmbryoIogy.
COLON
C. SILVERBERG, M.D. SEATTLE
DEFINITION
P
TOSIS may be defined as a prolapse downward of one or more of the abdomina1 viscera to such an extent as to change materiahy the normaI reIationship to one another and to the adjacent structures, especiahy when the subject is in the erect posture. ETIOLOGY
Predisposing causes : (a) Arrested deveIopment (b) MaIdeveIopment (c) SkeIetaI posture, shape of abdomina1 cavity, strength of abdomina1 waI1 2. Exciting causes: (a) Intra-abdomina1 pressure (b) Occupation To understand maIdeveIopments it is essentia1 to have at Ieast a rudimentary knowIedge of norma deveIopment. The aIimentary tract is deveIoped from the entodermic vesicIe of the zygote.2 This vesicIe becomes divided into an intraembryonic and an extra-embryonic portion. The intra-embryonic portion becomes the aIimentary tract and appendages. About the hfth week the aIimentary tract can be differentiated into three main subdivisions, the foregut, the midgut and the hindgut. The divisions are made on form, bIood suppIy, and function. Each part has a separate bIood supply. The foregut forms the gastrointestina1 tract beIow the ampuha of Vater to the dista1 portion of the transverse coIon with its circuIation supphed by the superior mesenteric artery. The hindgut is cauda1 to the transverse coIon and is suppIied by the inferior mesenteric artery. The function of the foregut is digestive; of the midgut absorptive; and of the hindgut excretory. DeveIopmentaI error is common in the I.
midgut whiIe in the foregut and hindgut maIformation is of rare occurrence. The viteIIine duct ordinariIy becomes detached about the fourth week of Iife. The rapid increase of the size of the Iiver of the midgut is out of proportion with that of the rest of the abdomen. The peritonea1 cavity cannot hoId a11 the structures, and the midgut is therefore pushed out through the umbiIicus, producing a physioIogic umbiIica1 hernia, The apex of the herniated Ioop is at the former attachment of the viteIIo-intestina1 duct and the termination of the superior mesenteric artery, which originaIIy was the right omphaIomesenteric artery. The artery Iies in the mesentery and sends branches to the anterior and posterior segments. The mesentery of the herniated Ioop Dott divides into a pre-arteria1 and a postarteria1 portion. The gut in this stage occupies a sagitta1 pIane. The rotation of the midgut occurs between the fifth and tenth week and may be divided into three stages. In the first stage the prearteria1 segment is on the right side and the post-arteria1 on the Ieft. The rotation is necessitated by the emargement and the descent of the liver and pressure of the umbiIica1 artery. The pre-arteria1 portion and the mesentery become disproportionateIy Iong. An enIargement in the postarteria1 segment can be seen in an embryo from twenty-seven to thirty days oId. This is the beginning of the deveIopment of the cecum and appendix. The second stage of rotation begins about the beginning of the tenth week when the gut returns to the abdomen. Ma11 suggests that the increase in the Iength of the Ioops and their rotation in the abdomina1 cavity produce enough traction to repIace the extra-abdomina1 intestina1 Ioops within the abdomen. The
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interna orifice of the umbiIica1 cana is comparativeIy smaI1, making it impossibIe for the entire contents of the hernia to return to the abdomina1 cavity en masse. The greater size of the cecum may offer sufficient resistance to retain it in the hernia1 sac, thus permitting the pre-arteria1 segment to return first. In doing so, it passes behind the superior mesenteric artery which extends from the aorta to the umbiIicus. The smaI1 intestine as it enters the abdomen pushes the intra-abdomina1 smaI1 gut and mesentery, which has been occupying the midIine, to the Ieft and posterior portion of the abdomina1 cavity. The cecum is the Iast to be reduced into the abdomina1 cavity. It occupies a position in the region of the umbilicus, anterior to the smaI1 intestines and superior mesenteric artery and beneath the Iiver. The cecum continues to descend to Iie in the norma aduIt position in the iIeoceca1 fossa. In the tweIfth week the superior mesenteric artery, which is brought to the iIiac region by the migration of the cecum, Iies in intimate contact with the posterior parietal peritoneum and becomes adherent from above downwards to the posterior abdomina1 waI1. AIong the right side of the artery the ascending coIon and cecum become fixed. The post-arteria1 mesenteric segment persists as the mesocoIon. The pre-arteria1 segment remains free as the mesentery of the smaI1 intestine. The mesentery of the hindgut becomes obIiterated by fusion aIong the midIine with the posterior parieta1 peritoneum. DEVELOPMENTAL
ANOMALIES
MaIrotation and non-rotation depend upon the sequence with which the intestina Ioops return to the abdomen. As a resuIt of non-rotation the smaI1 intestine lies chiefly to the right. The duodenum passes to the right of the superior mesentery artery. The colon is Iocated entireIy on the Ieft side and does not cross the superior mesenteric artery. Norma1 fixation or absence of fixation, is frequentIy encountered, producing an abnormaIIy
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mobiIe sigmoid or cecum. Non-rotation is the resuIt of a return of the cecum and the post-arteria1 segment to the abdominal cavity before return of the pre-arterial segment. Reverse rotation may take pIace but it is of IittIe significance cIinicaIIy, because the various parts of the intestina1 tract have normaI reIationships, except that they are in reverse. In maIrotation of the midgut, the pre-arteria1 segment of the midgut passes in front of the superior mesenteric artery and the posterior segment incIuding the cecum aIso passes in front of the origin of the artery. The mesentery does not become adherent to the posterior abdomina1 waI1. The duodenum and iIeum Iie side by side in front of the superior mesenteric vesseIs. The various congenita1 anomahes are due to the variation in the size of the embryonic umbiIica1 orifice. Most cIinica1 anomalies, however, arise from fauIty third stage rotation. The third stage rotation consists IargeIy of fusion of the various mesenteries with the posterior parietal peritoneum, making the various parts of the gastrointestina1 tract Iess mobiIe. EarIy fixation or deficient fixation with the exception of the eIongation of the coIon produce definite anomaIous conditions, The most important of these, pathoIogicaIIy, are those resuIting from deficient fixation of the coIon. The position of the retroceca1 appendix can easiIy be expIained if one remembers the descent and fusion of the cecum and ascending colon to the parieta1 peritoneum. The appendix can be turned back behind the descending cecum, becoming enclosed in the mesocoIon with the parietal peritoneum. On th e other hand, if the, appendix takes a position anterior to the cecum in the descent of the coIon it will have a free position in the abdominal cavity. EXCITING
CAUSES
If the occupations of ptotic people require that they be on their feet and
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especiaIIy if they have to do Iifting, the condition progresses from bad to worse. Through tke I&s of weight and of the fat
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supporting the abdomina1 viscera is aImost a negligible one. - Long continued intrathoracic conditions,
FIG. I.
FIG.
FIG. 3. FIGS. I TO 4. Stages of deveIopment
in the meshes of the mesentery greater eIongation occurs, causing a sagging of the abdomina1 viscera. A reIaxed abdomina1 waII after Iong wasting disease may be foIIowed by a visceroptosis. The reIaxation of the IateraI muscIe groups, that is, the transversaIis and the oblique, than that of the recti abdominis, which determines the condition. The part pIayed by the peIvic Aoor in
MARCH.
and rotation
2.
FIG. 4. of the midgut.
new growths and, at as Iarge exudates, times, pneumothorax may cause ptosis of the abdomina1 viscera. In this condition the diaphragm is fixed in the inspiratory position and thus renders its muscuIar opponents, the muscles of the abdomina1 waI1, useIess. Dr. Arthur Keith has made an embryoIogic and anatomic study of this condition and states that the abdominal muscIes in thoracic respiration act as
Silverberg-Ptosis
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opponents to the diaphragm. In a sheIflike manner they support the abdomina1 viscera during inspiration and aid the diaphragm in the eIevation of the ribs and thorax. In reIaxation of the IateraI muscIes of the abdomina1 waI1 this norma reIationship is Iost. The tendency to ptosis often runs in families, various members showing a phthisica1 chest and’giving a history of ancestra1 tubercuIosis. PATHOLOGIC
ANATOMY
AND
PHYSIOLOGY
In the norma position of the cecum and ascending coIon in the iIeoceca1 fossa, where proper fusion of the post-arteria1 segment has taken pIace, they get support from what Coffey caIIs the iIiopsoas sheIf. The center of gravity passes through the ascending coIon and cecum, we11 posterior to the anterior border of the psoas muscIe. In deficient fusion with a Iong mesocoIon the weight of the intestine forces it into the peIvis, causing a gradua1 eIongation of the mesentery. Nature attempts to correct the defect by Iaying down a membrane which graduaIIy becomes more dense, and often forming bands which become painfu1 when puIIed upon by the distended cecum. These bands produce stagnation by kinking the ascending coIon in the region of the hepatic ffexure. A child born with a defective prenata1 fixation of the abdomina1 viscera wiI1 have gastrointestina1 upsets from birth on. After adoIescence, with.the greater activity of the chiId, a gradua1 deveIopment of the typica ptotic figure is the ruIe. The colon, dropping from its norma Iocation, sIides off the psoas sheIf into the peIvis. The kidney fossa becomes shaIIower. The kidney often sIides down. The drag on the bottom of the stomach causes dispIacement of the pyIoric end of the stomach, retarding the gastric contents. This causes indigestion with subsequent Ioss of ffesh and further reduces the viscera1 support by decreasing the intra-abdomina1 pressure. Gas and ffuid in the gastrointestina1 tract are increased. The stretching of the
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gastro-intestina1 muscIes causes a gradua1 reduction of peristaIsis. The efficiency of digestion is generaIIy Iowered. The abdomina organs tend to gravitate more and more to the Iower portion of the abdomen and peIvis. The drag on the diaphragm and the fascia attached to the ribs causes the Iower portion of the chest to become narrow and the angIe of the ribs with the vertebrae to become sharper. The Iower end of the spine and the peIvis are tiIted backwards in an effort to hoId the Ioad by means of the thoracic fascia. The norma curvature of the spine disappears, and the spine becomes reIativeIy straight with the Iower end tiIted reIativeIy forward and upward and the upper part backward. The drag on the viscera1 nerves and sympathetic pIexus materiaIIy affects the nervous stabiIity of the patient, and these individuaIs are often cIass&ed as neurasthenics. The intestina1 stasis and resuIting autointoxication extending over years causes a depletion of the adrenaIs with a gradua1 Iowering of the bIood pressure, increasing weakness and making for easy exhaustion on physica and menta1 exertion. SYMPTOMATOLOGY
The discussion of symptoms is here Iimited to those of right sided abdomina1 ptosis onIy, the ptosis of the cecum and ascending coIon. This condition is, practicaIIy without exception, due to a congenita1 defect, the cecum and ascending coIong having faiIed to fuse properIy with the posterior parieta1 peritoneum. The symptoms generaIIy appear at the time of adoIescence and are often diagnosed as intestina1 colic. There are attacks of right sided abdomina1 pain which may Iast onIy a short time or may Iast for days, with rest in bed necessary before they subside. As time goes on the cecum and ascending coIon become Iarger and their contents are Iess easiIy expeIIed. Nature meanwhiIe tries to come to the rescue by Iaying down supporting mem-
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Am~ricm Journnl of Surgery
branes aIong the Iine of stress from the right Iateral waI1 of the abdomina1 cavity to the cecum and ascending coIon. These
CUffEn...... 75% FIG. j.
/nrno VZD
2s
%
M087UTrL. 0%
Tabulation of cases with prominent toms, treatment and resuIt.
symp-
membranes stand out as dense white bands, often kinking the bowe1 in the region of the hepatic ffexure, stiI1 further impeding the movements of the intestina contents. The appendix in these cases is apt to become infected because of the stagnation in the coIon. RemovaI of the appendix gives IittIe or no relief. There is vomiting after the pain has started, not at the beginning as in an attack of acute appendicitis. There may be fever of a degree or so if the stasis is considerabIe. On physica examination we often find a paIpabIe cecum and an ascending coIon which contains gas and ffuid. There is tenderness aIong the attachment of the membranous bands. In severe cases we find a ptotic figure and signs of neurasthenia. A barium meal shows a Iow lying cecum and often a retention of barium for days. A barium enema wiII show the same and, if there are tight bands, deIay of the barium enema at the region of the hepatic ffexure can be seen. DIAGNOSIS
In the differentia1 diagnosis we may consider a11 right sided abdomina1 conditions which give rise to pain, vomiting and
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toxemia, as we11 as right sided kidney Iesions and, in women, right peIvic Iesions. The succussion spIash in the cecum is said to be pathognomonic of a redundant cecum with stasis. GastrointestinaI x-ray studies are concIusive, but a diagnosis can generaIIy be made without the aid of the x-ray. The history of right sided abdomina1 pain from the time of adoIescence which is apt to get worse on physica exertion especiaIIy when the patient has to be on his feet for any Iength of time is characteristic. This pain may become worse with each attack. Pain occurring during or after an evening of dancing is very suggestive. The Iocation of the pain is higher than the average appendix. It is described as a dragging or a heavy pain in the right side or, if partia1 obstruction has occurred, as coIicky. PROGNOSIS
Due to the stasis and irritation produced by decomposition a coIitis is set up which in advanced cases spreads from the ptotic portion of the coIon. The symptoms are thus far in excess of what one wouId expect from the origina pathoIogy. After the correction of the pathoIogic anatomy the coIitis must be treated. Otherwise, the prognosis is good. TREATMENT
MedicaI management consists in reIieving the intestina1 stasis and attempting to shorten the mesentery by deposition of fat in the mesenteric meshes. The patient shouId be kept in bed with the foot of the bed raised and put on forced feeding, sometimes for months. A gain in weight may reIieve a11 symptoms. The shortened mesentery supports the intestines we11 enough to prevent kinking. If the weight can be maintained after a return to usua1 activities, good heaIth may continue, but generaIIy a gradua1 Ioss of weight causes return of the symptom compIex. BeIts and abdomina1 supports may be vaIuabIe in gastroptosis and genera1 ptosis
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but I beIieve they are worse than useIess in right sided ptosis, except for their psychic effect. SurgicaI correction shouId be carried out before a marked coIitis has set in. SurgicaI treatment is in most cases successful Preoperative management requires emptying of the coIon with a miId Iaxative and enemata. HospitaIization for some days prior to the operation is advisabIe. IntraperitoneaI vaccination is aIso vaIuabIe, especiaIIy if there is concomitant coIitis. In debiIitated patients, a buiId up program before operation shouId be carried out. Transfusion is heIpfu1. SpinaI anesthesia gives exceIIent reIaxation and shouId alIow suficient time. The operation can be performed in Iess than an hour. Ether anesthesia, where there is no contraindication, is satisfactory. A right pararectus incision is made, of sufhcient Iength to enabIe one to expose the hepatictflexure and the cecum. The bands and adhesions are reIeased and the appendix is removed. An incision is made IateraI to the cecum and ascending coIon as high as the hepatic ffexure, this being the IateraI Ieaf of the ascending mesocoIon. The peritoneum is reffected IateraIIy, exposing the perirena1 fat, practicaIIy a bIoodIess dissection. Care is taken not to injure the ureter. Sutures of chromic catgut or duIax are passed through the edge of the reflected peritonea1 foId, the first through the Iower end of the reflected foId and the region of the appendicea1 stump. The foIIowing sutures are inserted $6 inch to I inch apart through the anterior IongitudinaI band of the cecum and ascending coIon. I have found it advantageous to Ieave the sutures untied unti1 they are a11 inserted. When the sutures are tied the penduIous cecum and ascending colon roI1 into the denuded space where they soon wiI1 become firmIy attached. As there generaIIy is a coexisting ptosis of the right kidney, the kidney wiI1 become fixed in its proper position. At times the bands and membranes which have been formed in nature’s attempt to reIieve the oathoIogic condition can be
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used to strengthen the anchoring of the ascending coIon. If there stiI1 remains a sharp kinking at the hepatic ffexure the incision can be curved around and the condition corrected by suitabIy pIaced sutures. In cases where there is a Iong transverse coIon which faIIs down into the peIvis, dragging on the hepatic fIexure, I anchor it to the anterior abdomina1 waI1 media1 to the hepatic Aexure. Sutures are inserted through the root of the omentum and parieta1 peritoneum of the anterior abdomina waI1. This is what Coffey describes as his hammock operation. It can be extended right across the abdomina1 cavity if necessary and wiI1 give support to a ptotic stomach. These patients have remarkabIe IittIe postoperative disturbance. The reIief of the partia1 obstruction and the absence of intestina1 toxemia give them a feeIing of we11 being. They can Ieave the hospita1 when the wounds are heaIed and are permitted to resume active Iife as after any other simiIar abdomina1 operation. CoIitis, if present, is treated by diet and other measures. The patient often expects to be compIeteIy cured and does not coiiperate for a Iong enough time to have the coIon return to normaI. In cases where a portion of the coIon is much invoIved it might be wise to do a partia1 coIectomy. CASE
REPORTS
I have operated on tweIve patients and have seen a number of other cases in which no operation has been done, but in which has been estabIished by the diagnosis radiographic study. The tweIve operations have produced highIy satisfactory resuIts, with ten compIete cIinica1 cures and two much improved. The coIitis which existed in the Iatter two cases necessitates a more or Iess strict dietary regime. They are, however, reIieved of the toxemia and the symptoms associated with the partia1 TypicaI case histories are obstruction. appended.
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CASE I. A. S., maIe, age 20, compIained of right sided abdomina1 pain occurring at intervals of a few weeks, generalIy foIIowing dietary indiscretion or excessive physica exertion. The symptoms first appeared at the age of 14 and had since graduaIIy increased in severity. Vomiting occurred with the attacks, but rareIy fever. The pain lasted for two or three days. A diagnosis of appendicitis had been made. There was a paIpabIe cecum containing gas and ffuid. The Ieucocyte count was 10,000, the red count 4,400,000. A barium mea1 reveaIed a Iow lying cecum with stasis and probabIe kinking at the hepatic Aexure. At operation the appendix was found to be normal, but there was a we11 developed Jackson’s membrane with tight bands at the hepatic flexure. The cecum and ascending coIon were distended and penduIous. The appendix was removed and a Waugh operation was performed. The postoperative course was uneventfu1 and compIete recovery foIIowed. CASE II. A. N., maIe, age 28, had pain in the abdomen especiaIIy in the right side, fever, vomiting, constipation, attacks of diarrhea, and a rash which he had been toId might be due to syphilis. There was a paIpabIe mass in the right side of the abdomen, movable from side to side, and containing fIuid and gas. The temperature was 103O, the Wassermann negative, Ieucocytes 12,000. The urine was normaI. A diagnosis of intestinal stasis with toxemia was made. The intestina1 stasis was relieved, the symptoms disappeared, and the patient returned to work. One month later the condition recurred, and the patient was operated on. A Iarge and pendulous cecum and ascending colon were found with bands between the ascending coIon and the IateraI waI1 of the abdomen. The appendix showed no gross changes. The appendix was removed and a Waugh operation performed. Smooth recovery ensued, with no return of symptoms. CASE III. N. A., femaIe, age 25, had had periodic right sided abdominal pain for the previous ten years. It was apt to come on after exertion, such as dancing, but disappeared overnight or in a day or two. She was easiIy fatigued, had frequent headaches and was physically below par. Gas and ffuid could be made out in the cecum and ascending colon. There was tender-
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ness aIong the ascending coIon. A barium mea1 revealed a pendulous cecum with deIayed emptying of the cecum and ascending colon. Tight bands at the hepatic ffexure were found at operation. The appendix was apparentIy normal. It was removed, the bands cut, and a Waugh operation performed. ConvaIescence was uneventfu1 and recovery compIete. CASE IV. H. P., femaIe, age 25, had abdomina1 pains from 12 years of age. An appendectomy was done at the age of 20, when she was told that the appendix was norma but there was some peIvic condition causing her troubIe (“fibroid uterus”). Two years Iater she was operated on for an ovarian cyst. The right sided abdomina1 pain with constipation and toxemia persisted, however, and sometimes confined her to bed for days at a time. Genera1 debiIity and neurasthenia were noted. Gastrointestinal radiographic study reveaIed stasis in the cecum and ascending coIon. A long penduIous cecum rested on the peIvic floor. There were probabIy adhesions from the previous operation. The transverse coIon was Iong and occupied the pelvis. The coIon was kinked at the hepatic ffexure. A Iong pararectus incision was made on the right side. There were adhesions between the cecum and the old appendectomy scar, as we11 as bands and membranes between the cecum and ascending colon and the right side of the abdomina1 waI1. The coIon was inflamed and edematous. The adhesions were freed and coIopexy performed with the incision curving around the hepatic ffexure. Sutures were pIaced in the root of the Iarge omentum and to the anterior abdomina1 wall media1 to the hepatic fIexure halfway across the abdomen. In this case there was marked coIitis extending practically half way across the transverse colon and very marked at the hepatic fIexure. The patient’s postoperative course was smooth, a diet had to be followed. However, the stasis was reIieved and if there is no dietary indiscretion the patient’s health is good. CASE v. W. G., male, age 34, had periodic abdomina1 pain for several years which at times forced him to bed. He felt better when he kept his boweIs moving with laxatives and enemata. He was brought to the hospita1 as an emergency in the middle of the night with a fever of 103, marked tenderness and rigidity over the right side of the abdomen. Tenderness was equally marked under the right costal
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Siherberg-Ptosis
margins and over the right lower quadrant. Leucocytes numbered I 8,000, with go per cent polymorphonuclears. A long right pararectus incision made. An acutely inflamed appendix with a distended cecum and ascending colon and tight bands at the hepatic ffexure causing almost complete obstruction were observed. The ascending coIon was inffamed. The appendix was removed, the bands cut and a modified right sided colopexy done. A stormy convalescence for the first few days was followed by complete recovery. CASE VI. M. C., female, age 20, had had pain since the age of 13 or 14 which had gradually grown worse. She was afraid to go to dances because of the pain which followed. The attacks were aIways associated with vomiting, rarely by fever. There had been considerable loss of weight. One year before an appendectomy had been performed, but a Iong convalescence had been followed by no improvement. There was a paIpabIe cecum and ascending coIon, tenderness over the right side of the ascending colon. BIood and urinary findings were normal. Radiographic study showed six hour retention in the stomach, a Iarge penduIous
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cecum and colon, and apparentIy bands at the hepatic flexure. Operation confirmed the findings of the radiographic study. Adhesions between the pylorus, duodenum and hepatic ffexure and Jackson’s membrane were noted. The adhesions were released and a colopexy performed. Smooth convalescence followed. REFERENCES I. OCHSNER, ALTON. NeIson Loose-Leaf Surgery, Vol. v. 2. Dorr, N. M. &it. J. Surg., I I : 251, 1923. 3. COFFEY, R. C. In Dean Lewis. Practice of Surgery, VOI. VI. 4. CALLANDER, C. L. SurgicaI Anatomy. PhiIa., 1933. Saunders. 5. ROBERTSON, W. In Sajous. AnaIytic CycIopedia of Practical Medicine. 6. PATTENGER, F. M. South. California Pratt., March, 1912. 7. KEITH, ARTHUR. In Sajous. AnaIytic
8. g. IO. I I. 12.
CycIopedia of Practical Medicine, VoI. v, p. 776. RANKIN, F. W. Surgery of the CoIon. New York, 1926. AppIeton & Co. ALVAREZ, W. C. The Mechanics of the Digestive Tract. Hoeber. SHARP, H. V. Am. J. Surg., 24: 94-99. 1934. SMITH, J. W. Med. Chronicle, May, 1913. MALL. Textbook of EmbryoIogy.