Pitfalls in the surgical management of the incomplete duodenal diaphragm

Pitfalls

in the Surgical

Incomplete ByWILLIAM R.

Management

Duodenal

of the

Diaphragm

RICHARDSON ANDLESTER W. MARTIN

HE SIGNIFICANT IMPROVEMENT in results of surgical treatment of jejuno-ileal atresia and stenosis achieved in the past 15 years1m3 has not been matched in the intrinsic duodenal obstructions, where reported mortality rates are still 25-50 per cent. 4-8 Associated major anomalies and prematurity undoubtedly account for some fatalities in the duodenal atresias but it is our impression that factors of surgical judgment and technic play a major role when dealing with the rare incomplete diaphragmatic occlusion. The purpose of this report is to indicate the common pitfalls in management of this anomaly with therapeutic suggestions based on our experience with 21 cases.

T

CASE MATERIAL In the past 14 years, we have encountered 21 patients with a diaphragmatic type of duodenal stenosis. Their ages ranged from one day to 12 years. Six were operated upon in the first week of life, 10 in the first month, and 13 in the first year. Three patients had undergone previous unsuccessful laparotomies. Th ere were no fatalities. The preoperative clinical diagnosis specified duodenal diaphragm in 10 cases, stenosis in 7, malrotation in 3, and anastomotic dysfunction in one. The prolapsing type of diaphragm (Fig. 1) was predicted several times, Plain three-positional abdominal roentgenograms demonstrated a “double-bubble” in every instance, although it was not always considered diagnostic. The hugely dilated proximal duodenum in a 3-year-old child mimicked pneumoperitoneum and another child’s roentgenograms demonstrated gastric intramural gas without gangrene or perforation. Contrast studies indicated varying degrees of obstruction, usually in the second or third portion of the duodenum, and a smooth rounded bulb was the most constant clue (Fig. 2). Identification of the “plunging” or “windsock” diaphragmR-I1 was enhanced by cinkroentgenographic studies. Operative cholangiography with an aperture marker was done three times, but proved to be of only academic interest. Barium enemas helped to identify associated rotational anomalies. Seven of the 21 patients were mongoloid. Five had an anomalous rotation of the colon or duodenum. Other anomalies were hiatal hernia and congenital heart disease twice each, and situs inversus, Meckel’s diverticulum, and From the Division of Pediatric Surgery of The Children’s Hospital, Cincinnati, and the Depurtment of Surgery, College of Medicine, University of Cincinnati, Cincinnati, Ohio. WILLIAK~ R. RICHARDSON, M.D.: Assistant Clinical Professor of Surgery, College of &fedicine, University of Cincinnati. LESTER W. MARTIN, M.D.: Associate Professor of Surgery, College of Medicine, University of Cincinnati; Director of Pediatric Surgery, The Children’s Hospital, Cincinnati, Ohio. JOURNAL OF PEDIATRIC SURGERY,VOL. 4, No. 3 (JUNE),

1969

303

304

RICHARDSON AND MARTIN

Fig. l.-Sectional diagram of an incomplete “windsock” diaphragm prolapsing distalIy from its circumferential origin at ampullary level. The aperture was 6-7

mm. in diameter, eccentric and about 2.0 cm. from the junction of septum and duodenal wall. The common bile duct orifice was on the upper surface, separated from the aperture by a shallow groove. The duodenal wall distal to the attachment of the diaphragm was dilated and hypertrophied over the full extent of the windsock. nonobstructive annular pancreas once each. Two patients proved to have two separate duodenal diaphragms and a sibling of one had succumbed to a similar double lesion 8 years before (Fig. 3). 0 ne infant was born prematurely, one required exchange transfusion for jaundice, and one presented with significant alimentary bleeding. The diaphragms possessed most of the classic anatomic features described they were almost all attached peby previous authors. lo-13 Characteristically ripherally at the level of the ampulla of Vater, although some descriptions specified only the second portion of the duodenum. The site was commonly but not constantly indicated externally by a slight constriction in the duodenal wall. The excised segments all had proximal and distal mucosal surfaces with no muscle fibers except an occasional incomplete muscularis mucosa and rarely a few Brunner’s glands. In 14 patients, the aperture size was specified, ranging from “pin-point” up to larger than 1.0 cm., averaging 5 to 6 mm. and having only a rough correlation with age. In only 7 descriptions was the eccentric site of the hole specified, although recalled impressions suggest others. Three were specified as “central” or “apical.” Operative descriptions localized the bile duct orifices in 8 patients. The duct opened in the aperture rim or on the superior or inferior surface 3 times, less than 2 cm. above or below the diaphragm 3 times, and between the double septa found in 2 patients.

INCOMPLETE

DUODEKAL

305

DIAPHRAGM

Fig. 2.-Vivid roentgenographic evidence of incomplete diaphragmatic occlusion in two children. A, Barium swallowed by a 3-year-old girl lingers in an enlarged stomach and huge duodenum, the smooth bulb of which predicts the presence of an intrinsic obstruction in addition to the malrotation and peritoneal bands which were found at laparotomy. B, Characteristic pedunculated third bubble of barium in a 12-year-old girl with a “windsock” type diaphragm originating at ampullary level but with the smooth double outline and thin halo of its pouch reaching the fourth part of the duodenum. Eleven

of our 93 lesions

and indirect

evidence

up to 6-7 cm. in length tugging

effect

longest

pouches,

ment

others.

but appeared

of duodenal

peristalsis

as of the “windsock”

At laparotomy,

by cineradiography

on a pouch

partly

curve near the proximal

by the confusing

ease

configuration

the pouches

even longer

usually found in the older children,

on the inner duodenal evidenced

were confirmed

suggested

of digital

measured

all had eccentric

attachment. inversion

with the

filled with food. Their

mobility

and proximal

The

apertures was

displace-

at operation.

Although bypass procedures were used 9 times, we have come to favor a direct approach in most situations. In 2 patients, the septum was only incised to the aperture tomy drainage

and the longitudinal

duodenotomy

was used in most patients

closed

transversely.

Gastros-

in the past 8 years.

PITFALLS IN PREOPERATIVE DIAGNOSIS The major pediatric surgical texts do not furnish adequate details of the pathology of this particular anomaly probably because they reflect experience based

on an indirect

bypass

treatment

policy

which

does

not permit

precise

observation. Typical prolapsing diaphragms have been referred to frequently in radiologic literature as “intraluminal duodenal diverticuli.“14~z6 An occa-

306

RICHARDSON AND MARTIN

Fig. 3.-Diagrams depicting some of features of 2 patients encountered with double diaphragms. a, Proximal septum was prolapsing and had an eccentric aperture but the external constriction shown here was not present. Distal septum was at the sudden caliber change in the fourth portion of the duodenum after complete mobilization of the duodenum and division of the ligament of Treitz. b, Longitudinal duodenotomy at the taper allowed the septum to be divided through its central aperture. Transverse closure allowed proximal duodenal content to be milked through easily but continued clinical and radiologic obstruction led to re-exploration and successful excision of the elongated upper septum. c and d, Indicate the nature of the other double diaphragm in the series. The cross section shows two septa with small central perforations, separated by about 2 cm. of the distal second part of the duodenum into which the common bile duct was found to drain. After both were subtotally excised through the single duodenotomy, transverse closure provided an ample lumen. sional case of “chronic may be explained

duodenal

by an obscure

ileus” or “superior intrinsic

mesenteric

artery syndrome”

web.lj

Some points of clinical confusion deserve comment. Our experience suggests that there may be a higher incidence of incomplete duodenal diaphragm, especially of the prolapsing or “windsock” type, than has been appreciated.

307

INCOMPLETE

DUODENAL DIAPHRAGM

Conclusions

as to the level of obstruction

the vomitus can be misleading.

based

Bile-stained

on the presence of bile in

vomiting

has occurred

when

the duct or orifice lay below the obstruction, Bile ducts have bifurcated to empty both above and below and our series included 2 examples of double diaphragms with a bile duct orifice identified between the two levels.10.‘3 Rowe recently reported that the presence of situs inversus in 3 of 6 newborn patients with perforate duodenal webs had twice contributed to improper abdominal incisions. Plain roentgenograms failed to suggest the duodenal obstruction in 3 patients with coexistent low intestinal obstruction due to anal atresia or Hirschsprung’s disease.l” PITFALLS IN DIAGNOSISAT OPERATION External examination of the duodenum may mislead, suggesting that obstruction is not present or is extrinsic. Visible and palpable intraluminal anatomy may remain confusing if the duodenotomy is inadequate or improperly placed or the surgeon is unfamiliar with this lesion. External indentation of the duodenum may be minimal or absent. The usual patency tests utilizing air, fluid, finger, slender instrument, or catheter may fail to detect a perforate diaphragm. The thin prolapsed membrane may be difficult to palpate through the frequently dilated thick-walled distal duodenum. The position of the pouch apex may vary over a distance of twice its length. A chronically obstructed proximal segment in a growing infant tends to elongate as well as dilate, suggesting that the obstruction is further distal than it actually is (Fig. 2). The secondary elongation and tortuosity of the duodenum may accentuate the overlying peritoneal folds which can masquerade as the extrinsic obstructing bands associated with maIrotation.17 Dilation and hypertrophy of the duodenum below the “windsock” attachment can easily mislead the surgeon into improper placement of the duodenotomy and even into construction of a useless duodenojejunostomy below the obstruction (Figs. 1 and 4).

Coexistence with Extrinsic Obstructive

Lesions

Several recent reports show that over 10 per cent of patients with malrotation plus obstruction proved to have also an intrinsic duodenal block. A still higher incidence of intrinsic lesions is found in obstructed infants with an annular pancreas~xJ8-oo One of our 6 patients with coexisting malrotation required reexploration for an overlooked diaphragm. We have found a large bore Foley catheter to be reliable in ruling out a significant intrinsic lesion (Fig. 5).

Occurrence of Multiple Intrinsic Obstructions Two patients with double duodenal diaphragms have been encountered in the past 8 years. One required reoperation (Fig. 3). Boyd’s first reported case of congenital duodenal diaphragm in 1845 was a similar double lesion.llj”l Difficulties in management of patients whose second intrinsic obstruction was in the stomach, anus, and distal colon have also been described.10,“’

308

RICHARDSON AND MARTIN

/NDENTA

NON-BYPA

T/ON

AT

Si/ffG

DUODENOJ-f .Tlov0s1aw

Fig. 4.-Diagram of useless nonbypassing duodenojejunostomy found in a one year old with persistent intermittent obstruction since her first exploration at age one month. Duodenotomy just above the external caliber change had revealed no obstruction to proximal or distal exploration so the opening was used for anastomosis. lntimate Anatomic Relationship of Bile and Pancreatic Ducts to the Diaphragm Intrinsic

obstructive

lesions

are

known

to occur

most

frequently

at the

but the closeness and constancy of the relationship and the occasional actual intraseptal course of the common bile duct have not been sufficiently appreciated or publicized. Madden collected 70 cases of stenosis and suggested a close relationship of the bile duct orifice m about 80 per cent, dating back to Buchanan’s description in 1861.23-25 Eleven of 12 autopsy reports and specimens described by Boyden, Cope, and Bill in their recent study of the local anatomy and embryology showed the stenosis or atresia to be in the immediate vicinity of the major papilla.13 In four of their 6 stenoses, the duct opened at the exact site of obstruction, in one it opened just above and in another, 1.0 cm. below the obstruction. Our own cases have shown a similar distribution. Several authors have indicated that accessory or duplicated bile ducts may drain above the web or rarely into stomach or colon. Three case reports have been found in which an unsuspected terminal bile duct was injured during a local excision of the web or duodenum.6a26 general

level of the major Vaterian

Frequency

of Anastomotic

papilla

Dysfunction

Delayed function of side-to-side duodenal anastomoses is not uncommon and

INCOMPLETE DUODENAL DIAPHRAGM

Fig. 5.-Diagram to indicate use of the Foley catheter bag technic for demonstrating absence of significant intrinsic duodenal obstruction at the completion of a Ladd procedure for mafrotation. Gentle passage of the large bore catheter distally is facilitated by partial bag inflation after it has passed the pylorus. A very small or eccentric septal aperture may block full insertion in which case the true level of origin of the septum will be indicated by accentuation of the indentation.

a review

of the literature

indicates

a significant

number

of serious

early post-

and fatalities considered to be related to the duodenojejunostomy. Occasional cases have had late alimentary symptoms corrected by excision of the diaphragm and closure of the anastomosis.5,27 Gastrojejunostomy still appears in reported series, but is not recommended for this lesion because of its well-known incidence of dysfunction.

operative

complications

310

FUCHARDSON ANDMARTIN DISCUSSION

Problems peculiar to the incomplete duodenal diaphragm continue to plague the surgeon. There is a recurrent pattern in individual case histories that emphasizes the need for a planned surgical approach. In patients with partial duodenal obstruction, the usual three-positional abdominal and thoracic roentgenograms should frequently be supplemented by contrast studies of the colon and upper gastrointestinal tract. Use of multiple positions and additional air instilled via nasogastric tube prior to an infant’s initial roentgenograms may help delineate the obstructive site without the use of radiopaque contrast. At laparotomy the external position and relations of the common bile duct and pancreatic parenchyma should be noted. The presence of an intrinsic lesion cannot be excluded by external examination of the duodenum even with the mobilization of the entire duodenal loop that we recommend. A firm transgastric catheter passed into the duodenum should be used freely to indicate obstruction and the site of attachment of the diaphragm to the duodenal wall. If there is any suspicion of an intrinsic obstruction, a generous duodenotomy should be performed anterior to the greater curvature of the duodenum, extending well above the suspected level of web attachment. This permits inspection of both surfaces of the diaphragm and adjacent duodenum for biliary and pancreatic duct orifices. If no obstruction is encountered, an inflated Foley catheter or instrument of similar caliber should be passed well up into the stomach and down into the jejunum. The appeal of a procedure that avoids an anastomosis and maintains a normal relationship has led us to employ the direct approach more aggressively for the past several years. As Dr. Ladd wrote in 1937: “It would seem that the operation reported by Morton would restore much more normal conditions than any of the other operations so far used. The only possible disadvantage to this operation is the difficulty of approach.“2s+) Alerted by knowledge of the relationship to bile and pancreatic ducts, we have found it safe to excise the diaphragm. Since the web aperture is usually eccentric and near the medial duodenal wall where most duct orifices have been found, we have usually left the shorter shelf undisturbed. A probe or small catheter will often indicate and protect the course of the duct. Subtotal excision of the diaphragm usually leaves an interrupted crescentic mucosal defect which can be repaired with fine chromic gut sutures (Fig. 6). Duodenotomy closure should be in a transverse direction if it has been carried through the obstruction level, especially if the diaphragm has been incised only. A temporary tube gastrostomy has been beneficial especially in the depleted and small patients. SUMMARY Twenty-one consecutive patients with incomplete duodenal diaphragms were treated without fatality. Observed and reported pitfalls for the surgeon include difficulty in diagnosis before and during laparotomy, coexistence with intestinal malrotation or intrinsic obstructions of the duodenum, stomach or

INCOMPLETE

311

DUODENAL DIAPHRAGM

Fig. 6.--Sequence of diagrams outlining major steps in the operation proposed and performed by Mortonzg a, Anterior longitudinal duodenotomy started above the attachment of the diaphragm. b, “Windsock” diaphragm has been everted and in this case, bivalved longitudinally from the eccentric aperture to permit examination of both surfaces of the septum and the duodenal wall immediately above and below the septum. A probe is shown in the bile duct orifice found in the aperture rim. c, Most of the diaphragm has been excised using the Bovie and the resulting interrupted crescentic mucosal defect closed by a continuous fine chromic gut suture. Without positive identification of the bile duct, it is safer to excise less aggressively with scissors, using interrupted mucosal sutures. d, Duodenotomy has been closed transversely, after the inflated Foley catheter excluded the presence of another intrinsic obstructing lesion from midgastric to midjejunal level,

anorectum, features

intimate

bypassing

lateral

Successful

management

intrinsic

with protection

type

relationship

with

diaphragm,

and a tendency

the bile

ducts,

the confusing

for malfunction

of

anastomoses.

fort, use of a Foley calibrate

anatomic

of the windsock

has featured

bag catheter obstructions,

increased

or appropriate and a preference

preoperative instrument for excision

diagnostic

ef-

to help locate

and

of the diaphragm

of the bile ducts. SUMMARIO

IN INTERLINGUA

In le curso de 14 annos, 21 patientes con incomplete diaphragma duodenal esseva tractate sin mortalitate. Problemas pro le chirurgo es inter alteres le difficultate de1 diagnose ante e durante laparotomia, le coexistentia con malrotation intestinal o con le intime relation anatomic de1 ductos duodenal, gastric, o anorectal lesiones intrinsec, biliari, le confundite aspectos de1 diaphragma de typo “aerophagic,” e le tendentia de1 anastomose de derivation de functionar mal. Successes therapeutic ha essite obtenite gratias a plus intense effortios diagnostic ante

312

RICHARDSON AND MARTIN

le operation, al use de1 catheter a sacco de Foley in le location e al preferentia date al excision de1 diaphragma con protection transverse de1 duodenotomia longitudinal.

de1 obstructiones intrinsec, de1 vias biliari e clausura

REFERENCES 1. Louw, J. H.: Jejunoileal atresia and stenosis. J. Pediat. Surg. 1:8-24, 1966. 2. Handelsman, J. C., Abrams, S., and Corry, R. J.: Improvement of therapy for congenital jejunoileal atresia. Surg. Gynec. Obstet. 117:691, 1963. 3. Richardson, W. R.: The role and management of enterostomy in intestinal obstruction in infants. Amer. J. Surg. 106: 581592, 1963. 4. Longo, M. F., and Lynn, H. B.: Congenital duodenal obstruction. Mayo Clin. Proc. 42:385399, 1967. 5. Young, D. G., and Wilkinson, A. ‘II’.: Mortality in neonatal duodenal obstruction. Lancet 2: 18-20, 1966. 6. Nelson, T. Y.: Duodenal obstruction in infants and children. Med. J, Aust. 2: 709-713, 1963. 7. Aitken, J.: Congenital intrinsic duodenal obstruction in infancy. J. Pediat. Surg. 1: 546-559, 1966. 8. Knutrud, O., and Eek, S.: Combined intrinsic duodenal obstruction and malrotation. Acta Chir. Stand. 119:506-517, 1960. 9. Silcock, A. Q.: Epithelioma of the ascending colon; enterocolitis; congenital duodenal septum with internal diverticulum. Trans. Path. Sot. Lond. 36:207, 1885. 10. Nelson, W. I.: Congenital diaphragm of the duodenum. Minn. Med. 30:745-753, 1947. 11. Bill, A. H., Jr., and Pope, W. M.: Congenital duodenal diaphragm. Surgery 35:486, 1954. diaphragm. 12. Krieg, E. G.: Duodenal Ann. Surg. 106:3342, 1937. 13. Boyden, E. A., Cope, J. G., and Bill, and embryology of A. H., Jr.: Anatomy congenital intrinsic obstruction of the duodenum. Amer. J. Surg. 114:190-203, 1967. 14. Nance, F. C.: Intraluminal duodenal diverticula. Surg. Gynec. Obstet. 125:613619, 1967. 15. Rothenberg, R. E.: Congenital duodenal obstruction. Arch. Surg. 76:617-631, 1958.

16. Rowe, M. I., Buckner, D., and Clatworthy, H. W., Jr.: Windsock web of the duodenum. Amer. J. Surg. 116:444-449, 1968. 17. Ferguson, A. F., and Rothfield, N. J.: Intrinsic obstruction by duodenal diaphragm. Great Ormond St. Journal 8:105-112, 195455. i8. Gross, R. E.: The Surgery of Infancy and Childhood. Philadelphia, W. B. Saunders Company, 1953. 19. Schultz, L. R., Lasher, E. P., and Bill, A. H., Jr.: Abnormalities of rotation of the bowel. Amer. J, Surg. 101:128-134, 1961. 20. Boggs, T. R., Jr., and Bishop, H. C.: Neonatal hyperbilirubinemia associated with high obstruction of the small bowel. J. Pediat. 66:349-357, 1965. 21. Boyd, R.: Description of a Malformation of the Duodenum. Lancet 1:648, 1845. 22. Hailer, J. A., Jr., and Cahill, J. L.: Combined congenital gastric and duodenal obstruction: Pitfalls in diagnosis and treatment. Surgery 63:503-506, 1968. 23. Seidlin, S. M: Congenital duodenal septum with obstruction. Bull. Johns Hopkins Hosp. 37:328-339, 1925. 24. Buchanan, G.: Malformation of duodenum in a child. Trans. Path. Sot. Lond. 12: 121-129, 1861. 25. Madden, J. L., and McCann, W. J.: Congenital diaphragm&c occlusion of the duodenum with a report of three cases. Surg. Gynec. Obstet. 103:1-15, 1956. 26. Wiot, J. F., and Spiro, E.: Intraluminal duodenal diverticulum: A form of duplication. Radiology 80:4649, 1963. 27. Threadgill, F. D., and Hagelstein, A.: Duodenal diaphragm in the adult. Arch. Surg. 83:878-882, 1961. 28. Ladd, W. E.: Congenital duodenal obstruction. Surgery 1:878-885, 1937. 29. Morton, J. J., and Jones, T. B.: Obstructions about the mesentery in infants. Ann. Surg. 104:864-891, 1936.