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VOLUME 12 JULY 1953 NUMBER 1
ORIGINAL
ARTICLES
CONGENITAL
MEGACOLON
By ORVAR SWENSON, M.D.
Boston
T
years ago I had the privilege of presenting to this Academy our concepts of the pathology, the related dysfunction of the colon, and the surgical treatment of con-genital megacolon.1 I should now like to relate our interim clinical experience with thesepatients and particularly to stress diagnostic criteria of this disease.
Children with constipation should, from a practical standpoint, be divided into two groups: those with chronic constipation on a habit or psychologic basis who should be
treated medically; and those with congenital megacolon who should be treated surgically. The differentiation of the two groups is usually not difficult from the information gained in taking the history and performing the physical examination. The large majority of our patients with congenital megacolon have a history of constipation from birth. Often
obstipation, vomiting and abdominal distention have been of such prominence during the first few days of life that a diagnosis of intestinal obstruction has been entertained and
exploration occasionally performed. A considerable number of infants die of congenital
megacolon in the first weeks of life. The surviving infants continue to have varying amounts of constipation with intermittent abdominal distention and require frequent
enemas or some other aid in evacuation. In contrast to this the children with chronic constipation on a habit basis have histories of no symptoms until they are 2 or 3 years of
age, and abdominal distention is rarely present. Furthermore, there is usually a history of parental overanxiety concerning intestinal function, and this emotional attitude toward the child’s problem accounts for their tendency to exaggerate symptoms.
Further differentiation of the two groups can be made on physical examination, for the child with congenital megacolon will have abdominal distention and increased antero-posterior diameter of the chest with superior and lateral flaring of the rib margins. Fre-quently there is a large fecal impaction in the lower abdomen, which cannot be palpated
E. Mead Johnson Award Address, read at the Annual Meeting, American Academy of Pediatrics,
Chicago, Oct. 22, 1952.
From The Boston Floating Hospital (for Infants and Children) and the Departn)ent of Surgery, Tufts College Medical School, Boston.
FI(.. 1. #{149}Iypl(dl lcsion of congenital Iegacolon. Note nonLliStcndcd rectulTi and rcctosiuiuid with dllatdtlon of ‘,IgflU)id.
on redal examination, indicating that the impaction is above the congenital lesion.
Chil-dren with chronic constipation usually have no abdominal distention or deformity of the chest cage, and when a fecal impaction is present it can always be palpated Ofl rectal examInation indicatIng the absence of a congenital lesion in the lower colon.
All absolute diagnosis of congenital megacolon can only be made by roentgen
CXdIflIIlt-tion, and there is considerable misconception of tile criteria necessary to establish the
diag-110515. The origtnal radiograpllic examinations were performed by Dr. E. B. D. Neuhauser.
Enlargement of tile colon per in is of secondary importance diagnostically ; for while it invariably occurs ill congenital megacolon it frequently is due to other causes,
par-ticularly chronic colistipatlon. To establish the diagnosis of congenital megacolon there must he definite evidence of a segment of nondistended colon extending from the anus to
the rectosigmoid. Proximal to this the sigmoid is dilated, and there is an accumulation of gas and fecal material (Fig. 1) . Narrow areas 2 to 3 cm. in length in the sigmoid and
rectosigmold with some dilatation of the colon above and below are usually artifacts and
Insufilcient to make a diagnosis of congenital megacolon.
The technic of tile roentgeil examination was devised by Dr. E. B. D. Neuhauser. A straight catileter is inserted through the anal canal, and a small ailloullt of barium is iiljected
as the patient is observed on tile fluoroscopic screen in an oblique or lateral position. Tile
contrast media is allowed to flow in slowly, and as the sigmoid begins to fill the flow of barium is stopped. Should the sigmoid he completely filled it may overlap and obscure
the lesion which illut be identified to estabiish tile diagnosis of congenital mcgacolon. Wilen tile diagnosis has been confirmed radiographically the patient should have re section of tile agangilollic portion of the colon. Tile operation of resection of the rectum
A
* The author operated on 65 patients at Children’s Hospital, Boston.
Ftc,. 2. A. Preoperative RG of patient with congenital megacolon. This postevacuation film dem-onstrates inability of colon to empty properly. B. RG 14 mo. after operation. Note ability of colon to enlptv normally.
Is experienced and familiar with this operative procedure. Recently State has advocated
tilat a segmental resection be performed for this disease leaving the aganglionic rectum in
place. He advocated an incomplete resection of the involved bowel because he feared resection of the rectum and rectosigmoid might cause impotence in the male patients. Three of our patients have been adult males, and following resections including the rectum
and rectosigmoid ejaculation is normal. Thus, State’s hypothetic objection to complete excision of the lesion has not been borne out in our experience.
Furthermore, if the resection is limited to the lesion extending above the pelvic pen-tonc-um about half the patients would not be helped at all, because in our experience 47% of the patients have lesions which only involve the colon below the pelvic peritoneum. Most important of all, a partial resection of the lesion is not reasonable from a theoretic standpoint because we see patients with congenital megacolon with lesions no longer than
would he left in place with an anterior resection. In the past such incomplete resection, while helping some patients, has not completely relieved them; and there has been a recurrence of the disease in some. It seems reasonable that if surgery is to be used in the treatment of this disease a complete removal of the pathology should be performed.
The postoperative results of resection of the entire aganglionic segment have been
extremely gratifying. There has been one recurrence in 108* patients, and in that instance there was incomplete resection of the aganglionic bowel. The lesion extended to the splenic
flexure, and only the distal half of it was resected. In order to guard against such
recur-rence frozen sections are made at operation to determine that ganglion cells are present at
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IIRVARSWENSONone to two I#{232}1 ifibvements a day and require no special diet, laxatives or enemas. The
abdominal distention gradually disappears, and the abdominal contour is normal one year after operation. Barium enema examinations have demonstrated the dilated bowel returns to essentially normal size. Most important is the ability of the colon to completely
empty (Fig. 2).
REFERENCES
1. Swenson, 0., Congenital megacolon (Hirschsprung’s disease): Follow-up on 82 patients treated surgically, PEDIATRICS 8:542, 1951.
- 2. Swenson, 0., Rheinlander, H. F., and Diamond, I., Hirschsprung’s disease: New concept of
eti-ology; operative results in thirty-four patients, New England J. Med. 241:551, 1949.
3. State, D., Surgical treatment for idiopathic congenital megacolon (Hirschsprung’s disease), Surg., Gynec. & Obst. 95:201. 1952.
4. Hiatt, R. B., Surgical treatment of congenital megacolon, Ann. Sung. 133:321, 1951.
