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Congenital atresia of the small intestine
Congenital
Atresia
of the Small Intestine
EDWARD G. STANLEY-BROWN, M.D., New York, New York From-tbe Department of Surgery (Pediatric), Hospital, New York, New York.
diate neonatal period is quite common and may be caused by birth injury, infection, Iesions of the centra1 nervous system, improper feeding technics or smaI1 intestina1 atresia. The cardina1 distinguishing feature of the vomitus of the newborn infant with intestinal atresia is the green or yelIow color imparted by contained biIe. As Potts has so emphaticaIIy stressed, “if the baby ‘vomits green’ he has intestinal obstruction unti1 proven innocent.” It is important to urge that the coIor of vomitus, as we11 as stooIs, be recorded in the nurses’ notes in the newborn nursery. In our experience, the diagnosis of intestinal obstruction in the newborn infant is invariabIy suggested by an aIert nurse in the newborn nursery. Soon after birth the newborn infant starts to swaIIow air. If atresia is present in the duodenum only the stomach and hence the epigastrium wiI1 become distended. Impressive generalized abdomina1 distention wiI1 graduaIIy deveIop in the patient with atresia of the dista1 iIeum. WhiIe distention of the abdomen shouId aIways suggest the possibiIity of obstruction, its absence does not guarantee that the gastrointestina1 tract is normaI. This is of key importance to the physician attending the newborn infant. On two occasions in our HospitaI the responsibIe resident expressed astonishment at the diagnosis of duodenal atresia for, as he noted, “There was no abdomina1 distention I” PeristaItic waves are commonIy observed with high-lying atresia, and intestina1 patterning is often visibIe on the abdomina1 waI1 especiaIIy in premature infants. The newborn infant with smaI1 intestinal atresia wiI1 commonIy pass a Iight green or grayish stoo1, smaI1 in amount and frequentIy having the consistency of putty. AI1 too often the observation of this materia1 is recorded in the patient’s chart as the first meconium stoo1.
St. Luke’s
R. Thomas Bryant, Senior Surgeon to Guy’s HospitaI, London, writing in the Britisb Medical ,Journal of December 6, 1884, adduces :
M
“A simple obstruction may destroy Iife either by bringing about exhaustion due to the inability of the patient to take or retain food, the consequence of vomiting; or by peritonitis, the result of back pressure upon the bowe1 above the seat of obstruction; if not more directIy occasioned by sloughing, rupture or uIceration of the boweI, the consequence of over distension [I].” These observations are appIicabIe today despite the fact that they were written over seventy-eight years ago. EIsewhere in this issue it is noted that the mortaIity attendant to intestina1 obstruction in the newborn infant continues to be discouragingly high. The dire events described by Bryant [I] are almost entireIy preventabIe through earIy diagnosis, exact surgery, and meticuIous attention to the numerous detaiIs of optima1 pre- and postoperative care. This articIe reIates the management of one cause of intestina1 obstruction in the newborn infant, congenita1 atresia of the smal1 intestine. WhiIe rare (estimated to occur once in every 20,000 births) [2], atresia of the small intestine represents complete obstruction which, if untreated, wiI1 destroy Iife in four to ten days. DIAGNOSIS
The newborn infant with atresia of the smaI1 intestine wiIl commonIy vomit biIe-stained materia1 during the first tweIve to twenty-four hours of Iife despite the fact that no feeding has been ingested. Vomiting during the immeAmerican
Journal
of Surgery,
Volume
rod,
September
1961
448
Congenital
FIG. I, Supine
duodenal
Atresia
and erect abdominal atresia.
of SmaII Intestine
roentgcnograms.
ActuaIly, the small inspissated mass of epithelia1 celIs, lanugo hair and other debris is almost diagnostic of intestinal obstruction. Again, the alert nurse will readiIy distinguish such a stool as compared with the sticky, greenblack normal meconium, of which approximately I20 gm. wit1 be passed by a norma neonate during the first twenty-four hours of life. Bile-stained vomitus, abdomina1 distention and deIay or faiIure in passage of meconium comprise the triad of signs and symptoms of intestina1 atresia in the newborn infant. Any one of this triad or any combination thereof suff&es to suggest strongly the possibiIity of intestina1 atresia. Without delays for further observation, administration of an enema or any other temporizing measures, the patient should be taken to the x-ray department for an erect and supine plain x-ray film of the abdomen. Barium must never be administered by mouth to the newborn infant suspected of having intestinal atresia. Subsequent vomiting and aspiration of such barium may easily cause death [3]. Swenson [4] has recommended routine administration of a barium enema to rule out Hirschsprung’s disease and to insure patency of the coIon. We have not folIowed this recommendation routinely but reserved the barium enema for patients suspected of having terminal ileal or large bower obstruction. The plain abdominal roentgenogram usuaIIy confirms the diagnosis. (Fig. I.) Once the diagnosis of intestinal atresia is assured arrangements are made for early definitive surgery. If need be, the patient shouId be transferred to an institution equipped
“Double
bubble”
sign of
for optima1 pediatric surgical care. The need for warmth during transport of the newborn infant with intestinal obstruction and for frequent gastric aspiration through a smaI1 plastic or rubber catheter has been stressed and cIearIy outlined by Bishop [T]. PREOPERATIVE
PREPARATION
WhiIe surgery for the newborn infant with smaII intestinaI atresia is urgent and must not be postponed, an hour or two is usualIy required for the necessary preparation. A caref1u p h ysica1 examination is essentia1. Stigmas of mongoIism should be Iooked for particuIarIy in the infant with duodena1 atresia (incidence of about 30 per cent). Evidence of cardiac, central nervous system or other anomalies should be gently explained during the preoperative discusslon with the patient’s father and grandparents. The patient is pIaced in a warm IsoIette@* in an atmosphere of high humidity. A blood sample is obtained for bIood type and Rh factor and IOO to 200 m1. of compatible whole bIood readied for transfusion. As the newborn infant is reIativeIy hypervoIemic, the initial blood repIacement may be 25 to 50 ml. of single donor fresh frozen plasma, especiaIIy if the hemoglobin IeveI is very high 161. (The normal variation is 14.5 to 22.5 gm.) A smaI1 pIastic nasogastric tube is carefuIIy passed into the stomach and attached to constant intermittent suction. The Comco thermotic drainage pump (Unit No. 763-N) works admirabIy we11 for this purpose. (Fig. 2.) With * Air-Shields
449
Corp., Hatboro,
Pennsylvania.
StanIey-Brown There is wide variation in the amount of Auid per drop depending on the type of infusion apparatus used. Vitamin K, (phytonadione) should be administered in a singIe parentera dose of 0.5 to 1.0 mg. [IO]. A dose of 0.1 mg. atropine suIfate is administered subcutaneously to a11patients unless IocaI anesthesia aIone is to be used. OPERATION
The patient is transported to the operating room in the IsoIette, which is pIugged in and kept warm during surgery. To maintain norma body temperature during operation we have found the Aquamatic-K Pad* most usefu1. (Fig. 3.) The patient is placed directly upon this pIastic, warm water mattress and the extremities restrained with fIanne1. For premature infants the extremities may be wrapped in cotton sheet-wadding or fIanne1 strips to further conserve body heat. (Fig. 4.) A thermocoupIe electrode is pIaced in the patient’s rectum and the Tele-thermometer? empIoyed for constant monitoring of the patient’s body temperature. (Fig. 5.) The nasogastric tube is Ieft open for gravity drainage. It may be necessary to aspirate the stomach from time to time during surgery especiaIIy if this organ becomes distended with air or anesthetic gases. It is not within the scope of this presentation to discuss the anesthetic management of patients with atresia of the smaI1 bowe1; however, for premature infants, in particuIar those weighing Iess than 4 pounds, we have used IocaI procaine (0.5 or I.0 per cent) infiItration of the abdomina1 waI1 and sedation in the form of brandy injected through the nasogastric tube into the stomach (3 to 4 cc. of a mixture of one part brandy to three parts IO per cent glucose in water [II].) When IocaI anesthesia is empIoyed oxygen may be bIown over the patient’s face. The newborn infant with a body weight of 4 pounds or more may usuaIIy be managed successfuIIy with endotrachea1 intubation and cycIopropane or nitrous oxide as the anesthetic agent combined with a generous supply of oxygen. FolIowing appropriate preparation of the skin, with specia1 attention to the umbiIicus and stump of the umbiIica1 cord, the operative site is draped with specia1 singIe Iayer, light
FIG. 2. Gomco Thermotic Aspirator No. 763-N. Unit produces intermittent mild suction at 70 or IOO mm. of mercury and automaticaIIy operates at 25 second intervaIs.
tubes of such smaI1 caIiber frequent cIearing or irrigation with air or smaIl amounts of saIine soIution is vitaI. A venous cutdown is performed using IocaI anesthesia and if possibIe the Iargest size poIyethyIene catheter (Iumen accepts a No. 20 needIe) is inserted into the saphenous vein at the ankIe. DetaiIs of this frequentIy vexing and often time-consuming procedure have been documented by RandoIph and others [7,8]. A properIy functioning venous cutdown is absoIuteIy essential to the safe management of the patient and surgery must not be started unti1 the catheter is securely in pIace and functioning weI1. Five per cent gIucase in one-third or one-quarter norma saIine soIution is infused at the start. (Input is caIcuIated on a basis of 2,000 cc. per square meter of body surface area per twenty-four hours [g].) It is wise to order the rate of ffow as so many cubic centimeters per hour, rather than so many drops per minute as is commonIy done.
* Gorman-RuppCo., BeIIeviIIe, Ohio. t Yellow Ohio.
450
Springs
Instrument
Co.,
Yellow
Springs,
Congenital
Atresia
of Small Intestine
FIG. 3. The Aquamatic-K Pad. The control unit worms distilled water to desired temperature and continuously circulates fluid through plastic pad. There are no electric wires in the mattress. which is available in several sizes.
FIG. 4. A premature infant, weighing 41 ounces, with duodenal atresia who survived. Note absence of abdominal distention. Patient lies on Aquamatic-K Pad with Telethermometer probe in rectum.
weight, steriIe linen. Large and heavy linen drapes must not be used as the weight itself may suffice to seriously impair respiration. Also, such drapes will certainly contribute to heat retention, a complication to be avoided since febrile convulsions may occur. In generaI, we have employed generous paramedian incisions (3 or 4 inches long), retracting the rectus muscle Iaterally. Once the anterior rectus sheath has been divided, its media1 edge is grasped with mosquito forceps and elevated by an assistant. The dehcate rectus muscIe must be freed from the sheath by sharp dissection. MeticuIous hemostasis with No. 4-o or 5-o plain catgut is empIoyed. If the upper pole of the incision lies over the
liver, the posterior sheath and peritoneum may convenientIy be entered at this point without fear of injury to underlying distended small intestine. Once the initia1 opening through the peritoneum is accomphshed the remainder of this layer is divided with scissors aIong the entire length of the incision. Care is exercised at the extremities of the incision where the extraperitoneal umbilical vessek, still patent, are located and may easily be transected. A small quantity of peritoneal fluid usually emerges (if cIoudy or odoriferous perforation is suspected), immediately followed by coiIs of distended small intestine. Rather than trying to replace these Ioops of bowel, time may be saved by withdrawing all of the small bowel. 45’
Stanley-Brown
FIG. 5. The TeIethermometer. Numerous probes are avaiIabIe for rectaI, skin or air recording. EIectricaI source is from AashIight batteries.
This maneuver facihtates early Iocation of the most proxima1 site of atresia and does not unduly disturb the infant. As the surgeon traces gentIy aIong the distended bowel (which is easiIy ruptured) he wiI1 soon Iocate the first or most proximal atresic area. An assistant may retain this area with a moist sponge whiIe the
distal collapsed and deffated bowe1 is carefuhy examined for possibIe second or third atresic areas. Although duodena1 atresia is commonIy isolated, multipIe atresias are frequent in the distal smaI1 intestine. An aid to examination of the intestine beyond the first site of atresia is injection of saline soIution or minera 0iI aIong with some air dehvered through a No. 23 or 24 size needle. The liquid and bubbIes may then be milked along and successfu1 distal intraIuminaI passage observed if the atresia is isolated. (Fig. 6.) If a barium enema has been performed inspection of the coIon is unnecessary . Having examined the entire bowel, the surgeon is now ready to perform the anastomosis at one or more sites. The enormous size of the proxima1 segment, balIooned with trapped gas and meconium Auid, may suggest to the inexperienced surgeon that side to side anastomosis represents the onIy conceivable soIution. Such an anastomosis is undesirabIe [la] and shouId be avoided if possible. An end to end or end to side anastomosis is the ideal means of re-estabIishing intestinaf continuity. As Swenson has recommended, the dilated proxima1 bowel is resected taking care to divide the mesentery cIose to the bowel wall [a]. The outer Iayer of the anastomosis is perbIack formed with No. 4-o or 5-o atraumatic siIk suture whiIe No. 5-o to 6-o chromic catgut with swaged on needIe is used for the inner Iayer. Despite resection of the bIind proxima1
FIG. 6. Liquid and air bubbIes distend coIIapsed segment distal to atresia of the smaI1 bowe1. Note proximal diIated segment above site of atresia.
452
Congenital
Atresia
of Small Intestine
FIG. 7. A, the dilated proxima1 bowe1 which is to be resected. B, This shows distention of the dista1 seement and detaiIs of the end to side anastomosis in C, D and E.
pouch there is apt to he considerabIe disparity in the diameter of the bowel to be reunited. As an aid to placing the first layer of silk sutures a Pott,‘s cIamp may be used to occlude the most proximal segment of the distal collapsed bowel which is then intIated with air by injection. (Fig. 7.j Each catgut and silk suture must be preciseIy pIaced 2 to 3 mm. apart with no ever&on of the mucosa. The outer layer of sutures must not incIude more than the seromuscular coat because penetration of the mucosa wit1 permit Ieakage and peritonitis. Special care in placing sutures into the dista1 segment is necessary to avoid compromise of the aIready miniscule lumen. Once the anastomosis has been compIeted the surgeon should demonstrate patency by milking air through the new connection. The mesenteric defect is carefully closed with interrupted fine chromicized catgut sutures. I n patients having more than one atresia muItipIe anastomoses wiI1 be required. When atresic areas are closely situated resection of a short segment of normal intervening bowe1 is preferable thus making it possible to reestablish continuity with a single anastomosis.
When several sausage shaped bits of atresic bowel are left in situ they may grow and cause cvsts, abdominal pain or obstruction. (Fig. 8.) With more than one anastomosis the possibiIity for leakage is greater and when several anastomoses are required the chances of a successful outcome are diminished. In other words, the fewer anastomoses the better, but it is vital to preserve as much Iength of the smaII intestine as possibIe. Blood loss must constantly be appraised by weighing soaked sponges and measuring blood in suction bottIes. AlI blood lost should be replaced during operation and insofar as possible when it is lost. A three-way stopcock is incorporated into the tubing Ieading to the intravenous catheter. Ideally an additional member of the house staff is assigned the sole responsibility of blood repIacement. Pre- and postoperative body weight of the patient (adjusted for organs removed and addition of catheters, bandages and the like) serves as a useful guide to the exactness of blood replacement. Once the anastomosis or anastomoses are completed the surgeon must decide whether 453
StanIey-Brown
FIG. 8. Cystic masses containing cheesy materia1 resected in an older child who initially underwent side to side iIeocoIostomy bypassing segmental atresic areas in the terminal ileum. (Patient of Dr. HaroId A. ZinteI.)
sheath is closed with interrupted sutures of the same materia1 placed 3 to 4 mm. apart. The skin edges are approximated with interrupted sutures of No. 4-o or 5-o bIack siIk. The ques-
or not to construct a temporary gastrostomy. With premature infants and those with highlying obstruction, we have found gastrostomy imme?seIy useful. The details of this operation are we11documented by HoIder and Gross [13]. It is important to suture the stomach securely to the peritoneum and posterior fascia1 layers to prevent Ieakage and peritonitis. CarefuI cIosure of the abdomina1 waI1 is undertaken with No. 4-0 atraumatic chromic catgut as a running suture for the peritoneum and posterior rectus sheath. The anterior rectus
tion of using retention
sutures
depends
some-
what on the Iocation of the incision. In generaI, a high paramedian incision so pIaced that the Iiver wiI1 under-lie and buttress the upper two thirds of the incision may be expected to hea safeIy without retention sutures. If the paramedian incision is IargeIy beIow the Ever or so pIaced that the umbiIicus is at the IeveI 454
Congenital
Atresia
of SmaII Intestine studies obtained. The antibiotic to which the organisms are sensitive shouId be administered intravenousIy thus disturbing the patient as IittIe as possibIe during a period when rest is of the essence. GentIe nasopharyngeal suction is instituted as required and vital signs (pulse and respiration) are observed at regular frequent intervals. We have given a 3 per cent gIucose in one quarter normal saIine soIution intravenousIy, changing to poIyionic preparations containing potassium and other eIectroIytes after twentyfour hours. Hemoglobin and microhematocrit are determined six to eight hours postoperativeIy and, if needed, blood may be transfused. SmaII pIasma and bIood transfusions are helpful over the postoperative period. After forty-eight hours, nasogastric suction is discontinued and the first ora or gastrostomy feeding started with 3 to IO m1. of gIucose and water repeated every two to three hours. If such feedings are absorbed in two to three hours, as proved by gastric aspiration, dilute formula of evaporated milk and water (one part to ten) is cautiousIy begun, IO to 15 cc. at a time. If vomiting occurs the stomach should be Iavaged with warm water, aspirated until empty and feedings restarted after a rest of three or four hours, beginning again with gIucose and water. In some patients the anastomosis may be exasperatingIy SIOW to function and intermittent nasogastric suction will have to be continued for several days. The first stools passed are usually composed of meconium and oId bIood; however, once bile appears in the stool, the surgeon may reIax in the knowledge that the gastrointestina1 tract is now patent. In the absence of vomiting, the formula may be increased in strength and amount but the suction machine and Iaryngoscope are kept near at hand, because of the ever-present possibihty of vomiting and aspiration. At our Hospital aI1 nurses have been instructed in the use of the Iaryngoscope by members of the Anesthesia Staff. Since inauguration of this teaching program, many instances of successfu1 resuscitation by the nurse have been recorded. For the patient who has undergone surgery for atresia of the smaII bowel the postoperative road to recovery is long, bordered by many pitfaIls and open trenches. A wound infection may Iead to dehiscence, which is apt to prove
of the middle pole of the incision, retention sutures of No. 2-o or 3-o bIack silk may be pIaced through al1 Iayers except the peritoneum. These may be secured with buttons as described by Potts [14] or tied over a smaI1 gauze rol1. The dressing should be smaI1 since buIky dressings obscure the abdomen and do not allow auscuItation or adequate inspection. A narrow gauze strip may be secured with one or two 35 inch widths of adhesive tape and then held in pIace by three or four singIe turns of gauze rolIer bandage compIeteIy encircIing the patient’s abdomen. It is convenient and advisable to dispense with the dressing aItogether after twenty-four hours. An alternative is to cIose the skin with subcuticuIar sutures of continuous or interrupted fine siIk or catgut and thereafter to coat the skin surface with pIastic spray or coIIodion. If gastrostomy has been performed it is vita1 that the tube be securely anchored in place, for even premature infants have demonstrated incredibIe ski11 in pulling out the gastrostomy tube. POSTOPERATIVE
CARE
Postoperative care commences the instant the skin closure is compIeted. The patient is returned to his Isolette which has been kept warm during operation. The nasogastric or gastrostomy tube is re-attached to intermittent suction with the Gomco Thermotic Drainage pump.* With the miniature nasogastric tubes necessary for the premature or normal newborn infant, a tiny Aeck of mucus or a bit of secretion may compIeteIy occlude the lumen; thus, the tube should be irrigated at Ieast once every hour with 5 to IO m1. of norma saIine soIution. The efficacy of these tubes is aImost entireIy dependent upon carefu1 supervision. The experienced surgeon will check and aspirate the tube himseIf at least once every twenty-four hours. An atmosphere of high humidity is heIpfu1 and the Vapojette Supersaturation Attachment@t (Fig. g) produces an exceIIent fog. We have not used antibiotics routineIy except for those infants with a moist “soupyappearing” umbilica1 cord stump. Such an obvious site of contamination shouId be cuItured and appropriate antibiotic sensitivity * Gomco Surgical Manufacturing Corp., New York. t Air-Shields Corp., Hatboro, PennsyIvania.
Buffalo,
455
StanIey-Brown
FIG. g. The IsoIette photographed with the Vapojette Supersaturation Attachment in pIace. This device may be activated by a flow of oxygen or through use of a specia1 pump avaiIabIe from Air-Shields Corporation.
accIimatization is particuIarIy important for a mother with her first baby. It is wise to reassure the mother that the infant wiI1 not “come apart” and that he may be handIed and fondIed without fear. The premature infant may be discharged once body weight has reached 555 pounds. In the absence of postoperative compIications patients with normal birth weights usuaIIy remain in the nursery an average of ten to fifteen days after operation.
fataI. An anastomotic Ieak with peritonitis is rareIy saIvabIe and emphasizes the absoIute importance of precise and exact technic in constructing the anastomosis. IntestinaI obstruction caused by adhesions may require reoperation. Constant nursing observation is essentia1, known in some hospitals as direct reIief. SimpIy stated, the nurse may not Ieave her patient unattended for so much as a singIe second. The greatest possible credit goes to our nurses who with skiI1, Iove and devoted interest have safeIy guided the majority of our patients out of the VaIIey of the Shadow of Birth [IT], a journey made a11 the more precarious by surgical trauma. When the patient progresses to fuI1 formuIa and gains weight daiIy it is important to have the mother feed her new baby and “get acquainted” in the hospita1 nursery. Such
SUMMARY
CongenitaI atresia of the smaI1 intestine is rare, uniformIy fatal if untreated and demands earIy diagnosis and careful preoperative preparation. Corrective surgery must be precise, designed to remedy the Iesion in a singIe procedure and conducted with carefu1 attention to bIood repIacement and maintenance of body warmth. SkiIIed anesthetic management is of 456
CongenitaI the essence and the postoperative demanding in the extreme.
Atresia care
of SmaII -.
is
2. 3.
4.
5.
6.
RANDOLPH, J. Technique for insertion of plastic catheter into saphenous vein. Pediaks, 24: 631,
1959. 8. STAXEY-BKOWU, E. G. The venous cutdown. Arch. Pediat., 75: 480, 1958. g. TALROT, N. B., CRAWFORD, J. D. and Br TLU, A. 51. Homeostatic limits to safe parenteral fluid therapy. New England J. A/ied., 248: I loo, 1953. IO. Report of Committee on Nutrition, American Academy of Pediatrics, hlarch I 961. Pediatrics, 28: 501, 1961. r I. SMITH, R. Cl. Anesthesia for Infants and Children, p. 78. St. Louis, 1959, C. V. hlosby Co. 12. HURWITZ, A. Reappraisal of side-to-side and endto-side intestinal anastomoses. Surgeyt-, 43: 864,
REFERENCES I.
Intestine
T. Harveian lectures on the mode of death from acute intestinal strangulation and chronic intestinal obstruction. Ann. Surg., I: 177, 1885. WANGENSTEEN, 0. H. Intestinal Obstructions, Ed. 3. Springfield, III., 1955. CharIes C Thomas. STANL.EY-BROWN,E. C., ZINTEL, H. A. and EAGLE, J. F. An anaIysis of operative deaths in infants and children. Surg. Gynec, @ Obst., I 14: 137, 1962. SWENSON, 0. and FISHER, J. H. SmaIl bowel atrcsia: treatment by resection and primary aseptic anastamosis. Surgery, 47: 823, 1960. BISHOP, H. C. Safe transportation of newborn infants for emergency surgery. J. A. M. A., 165: 123o, 1957. BISHOP, H. C. Factors affecting success and faiIure in neonatal surgery. Pediat. C&n. North America, 6: 945, ‘959. BRYANT,
1958. 13. HOLDER, T. ,\I. and GROSS, R. E. Temporarv gastrostomy in pediatric surgthry. Pediatrics. 26’: 36, rg6o. 14. POTTS, W. J. The Surgeon and the Child, p. 4;. Philadelphia, 1959. W. B. Saunders Co. 15. SMUITH,C. A. The vaIIey of the shadow of birth. Am. J. Dis. Cbild., 82: 171, 1951.
457
Atresia
of the Small Intestine
EDWARD G. STANLEY-BROWN, M.D., New York, New York From-tbe Department of Surgery (Pediatric), Hospital, New York, New York.
diate neonatal period is quite common and may be caused by birth injury, infection, Iesions of the centra1 nervous system, improper feeding technics or smaI1 intestina1 atresia. The cardina1 distinguishing feature of the vomitus of the newborn infant with intestinal atresia is the green or yelIow color imparted by contained biIe. As Potts has so emphaticaIIy stressed, “if the baby ‘vomits green’ he has intestinal obstruction unti1 proven innocent.” It is important to urge that the coIor of vomitus, as we11 as stooIs, be recorded in the nurses’ notes in the newborn nursery. In our experience, the diagnosis of intestinal obstruction in the newborn infant is invariabIy suggested by an aIert nurse in the newborn nursery. Soon after birth the newborn infant starts to swaIIow air. If atresia is present in the duodenum only the stomach and hence the epigastrium wiI1 become distended. Impressive generalized abdomina1 distention wiI1 graduaIIy deveIop in the patient with atresia of the dista1 iIeum. WhiIe distention of the abdomen shouId aIways suggest the possibiIity of obstruction, its absence does not guarantee that the gastrointestina1 tract is normaI. This is of key importance to the physician attending the newborn infant. On two occasions in our HospitaI the responsibIe resident expressed astonishment at the diagnosis of duodenal atresia for, as he noted, “There was no abdomina1 distention I” PeristaItic waves are commonIy observed with high-lying atresia, and intestina1 patterning is often visibIe on the abdomina1 waI1 especiaIIy in premature infants. The newborn infant with smaI1 intestinal atresia wiI1 commonIy pass a Iight green or grayish stoo1, smaI1 in amount and frequentIy having the consistency of putty. AI1 too often the observation of this materia1 is recorded in the patient’s chart as the first meconium stoo1.
St. Luke’s
R. Thomas Bryant, Senior Surgeon to Guy’s HospitaI, London, writing in the Britisb Medical ,Journal of December 6, 1884, adduces :
M
“A simple obstruction may destroy Iife either by bringing about exhaustion due to the inability of the patient to take or retain food, the consequence of vomiting; or by peritonitis, the result of back pressure upon the bowe1 above the seat of obstruction; if not more directIy occasioned by sloughing, rupture or uIceration of the boweI, the consequence of over distension [I].” These observations are appIicabIe today despite the fact that they were written over seventy-eight years ago. EIsewhere in this issue it is noted that the mortaIity attendant to intestina1 obstruction in the newborn infant continues to be discouragingly high. The dire events described by Bryant [I] are almost entireIy preventabIe through earIy diagnosis, exact surgery, and meticuIous attention to the numerous detaiIs of optima1 pre- and postoperative care. This articIe reIates the management of one cause of intestina1 obstruction in the newborn infant, congenita1 atresia of the smal1 intestine. WhiIe rare (estimated to occur once in every 20,000 births) [2], atresia of the small intestine represents complete obstruction which, if untreated, wiI1 destroy Iife in four to ten days. DIAGNOSIS
The newborn infant with atresia of the smaI1 intestine wiIl commonIy vomit biIe-stained materia1 during the first tweIve to twenty-four hours of Iife despite the fact that no feeding has been ingested. Vomiting during the immeAmerican
Journal
of Surgery,
Volume
rod,
September
1961
448
Congenital
FIG. I, Supine
duodenal
Atresia
and erect abdominal atresia.
of SmaII Intestine
roentgcnograms.
ActuaIly, the small inspissated mass of epithelia1 celIs, lanugo hair and other debris is almost diagnostic of intestinal obstruction. Again, the alert nurse will readiIy distinguish such a stool as compared with the sticky, greenblack normal meconium, of which approximately I20 gm. wit1 be passed by a norma neonate during the first twenty-four hours of life. Bile-stained vomitus, abdomina1 distention and deIay or faiIure in passage of meconium comprise the triad of signs and symptoms of intestina1 atresia in the newborn infant. Any one of this triad or any combination thereof suff&es to suggest strongly the possibiIity of intestina1 atresia. Without delays for further observation, administration of an enema or any other temporizing measures, the patient should be taken to the x-ray department for an erect and supine plain x-ray film of the abdomen. Barium must never be administered by mouth to the newborn infant suspected of having intestinal atresia. Subsequent vomiting and aspiration of such barium may easily cause death [3]. Swenson [4] has recommended routine administration of a barium enema to rule out Hirschsprung’s disease and to insure patency of the coIon. We have not folIowed this recommendation routinely but reserved the barium enema for patients suspected of having terminal ileal or large bower obstruction. The plain abdominal roentgenogram usuaIIy confirms the diagnosis. (Fig. I.) Once the diagnosis of intestinal atresia is assured arrangements are made for early definitive surgery. If need be, the patient shouId be transferred to an institution equipped
“Double
bubble”
sign of
for optima1 pediatric surgical care. The need for warmth during transport of the newborn infant with intestinal obstruction and for frequent gastric aspiration through a smaI1 plastic or rubber catheter has been stressed and cIearIy outlined by Bishop [T]. PREOPERATIVE
PREPARATION
WhiIe surgery for the newborn infant with smaII intestinaI atresia is urgent and must not be postponed, an hour or two is usualIy required for the necessary preparation. A caref1u p h ysica1 examination is essentia1. Stigmas of mongoIism should be Iooked for particuIarIy in the infant with duodena1 atresia (incidence of about 30 per cent). Evidence of cardiac, central nervous system or other anomalies should be gently explained during the preoperative discusslon with the patient’s father and grandparents. The patient is pIaced in a warm IsoIette@* in an atmosphere of high humidity. A blood sample is obtained for bIood type and Rh factor and IOO to 200 m1. of compatible whole bIood readied for transfusion. As the newborn infant is reIativeIy hypervoIemic, the initial blood repIacement may be 25 to 50 ml. of single donor fresh frozen plasma, especiaIIy if the hemoglobin IeveI is very high 161. (The normal variation is 14.5 to 22.5 gm.) A smaI1 pIastic nasogastric tube is carefuIIy passed into the stomach and attached to constant intermittent suction. The Comco thermotic drainage pump (Unit No. 763-N) works admirabIy we11 for this purpose. (Fig. 2.) With * Air-Shields
449
Corp., Hatboro,
Pennsylvania.
StanIey-Brown There is wide variation in the amount of Auid per drop depending on the type of infusion apparatus used. Vitamin K, (phytonadione) should be administered in a singIe parentera dose of 0.5 to 1.0 mg. [IO]. A dose of 0.1 mg. atropine suIfate is administered subcutaneously to a11patients unless IocaI anesthesia aIone is to be used. OPERATION
The patient is transported to the operating room in the IsoIette, which is pIugged in and kept warm during surgery. To maintain norma body temperature during operation we have found the Aquamatic-K Pad* most usefu1. (Fig. 3.) The patient is placed directly upon this pIastic, warm water mattress and the extremities restrained with fIanne1. For premature infants the extremities may be wrapped in cotton sheet-wadding or fIanne1 strips to further conserve body heat. (Fig. 4.) A thermocoupIe electrode is pIaced in the patient’s rectum and the Tele-thermometer? empIoyed for constant monitoring of the patient’s body temperature. (Fig. 5.) The nasogastric tube is Ieft open for gravity drainage. It may be necessary to aspirate the stomach from time to time during surgery especiaIIy if this organ becomes distended with air or anesthetic gases. It is not within the scope of this presentation to discuss the anesthetic management of patients with atresia of the smaI1 bowe1; however, for premature infants, in particuIar those weighing Iess than 4 pounds, we have used IocaI procaine (0.5 or I.0 per cent) infiItration of the abdomina1 waI1 and sedation in the form of brandy injected through the nasogastric tube into the stomach (3 to 4 cc. of a mixture of one part brandy to three parts IO per cent glucose in water [II].) When IocaI anesthesia is empIoyed oxygen may be bIown over the patient’s face. The newborn infant with a body weight of 4 pounds or more may usuaIIy be managed successfuIIy with endotrachea1 intubation and cycIopropane or nitrous oxide as the anesthetic agent combined with a generous supply of oxygen. FolIowing appropriate preparation of the skin, with specia1 attention to the umbiIicus and stump of the umbiIica1 cord, the operative site is draped with specia1 singIe Iayer, light
FIG. 2. Gomco Thermotic Aspirator No. 763-N. Unit produces intermittent mild suction at 70 or IOO mm. of mercury and automaticaIIy operates at 25 second intervaIs.
tubes of such smaI1 caIiber frequent cIearing or irrigation with air or smaIl amounts of saIine soIution is vitaI. A venous cutdown is performed using IocaI anesthesia and if possibIe the Iargest size poIyethyIene catheter (Iumen accepts a No. 20 needIe) is inserted into the saphenous vein at the ankIe. DetaiIs of this frequentIy vexing and often time-consuming procedure have been documented by RandoIph and others [7,8]. A properIy functioning venous cutdown is absoIuteIy essential to the safe management of the patient and surgery must not be started unti1 the catheter is securely in pIace and functioning weI1. Five per cent gIucase in one-third or one-quarter norma saIine soIution is infused at the start. (Input is caIcuIated on a basis of 2,000 cc. per square meter of body surface area per twenty-four hours [g].) It is wise to order the rate of ffow as so many cubic centimeters per hour, rather than so many drops per minute as is commonIy done.
* Gorman-RuppCo., BeIIeviIIe, Ohio. t Yellow Ohio.
450
Springs
Instrument
Co.,
Yellow
Springs,
Congenital
Atresia
of Small Intestine
FIG. 3. The Aquamatic-K Pad. The control unit worms distilled water to desired temperature and continuously circulates fluid through plastic pad. There are no electric wires in the mattress. which is available in several sizes.
FIG. 4. A premature infant, weighing 41 ounces, with duodenal atresia who survived. Note absence of abdominal distention. Patient lies on Aquamatic-K Pad with Telethermometer probe in rectum.
weight, steriIe linen. Large and heavy linen drapes must not be used as the weight itself may suffice to seriously impair respiration. Also, such drapes will certainly contribute to heat retention, a complication to be avoided since febrile convulsions may occur. In generaI, we have employed generous paramedian incisions (3 or 4 inches long), retracting the rectus muscle Iaterally. Once the anterior rectus sheath has been divided, its media1 edge is grasped with mosquito forceps and elevated by an assistant. The dehcate rectus muscIe must be freed from the sheath by sharp dissection. MeticuIous hemostasis with No. 4-o or 5-o plain catgut is empIoyed. If the upper pole of the incision lies over the
liver, the posterior sheath and peritoneum may convenientIy be entered at this point without fear of injury to underlying distended small intestine. Once the initia1 opening through the peritoneum is accomphshed the remainder of this layer is divided with scissors aIong the entire length of the incision. Care is exercised at the extremities of the incision where the extraperitoneal umbilical vessek, still patent, are located and may easily be transected. A small quantity of peritoneal fluid usually emerges (if cIoudy or odoriferous perforation is suspected), immediately followed by coiIs of distended small intestine. Rather than trying to replace these Ioops of bowel, time may be saved by withdrawing all of the small bowel. 45’
Stanley-Brown
FIG. 5. The TeIethermometer. Numerous probes are avaiIabIe for rectaI, skin or air recording. EIectricaI source is from AashIight batteries.
This maneuver facihtates early Iocation of the most proxima1 site of atresia and does not unduly disturb the infant. As the surgeon traces gentIy aIong the distended bowel (which is easiIy ruptured) he wiI1 soon Iocate the first or most proximal atresic area. An assistant may retain this area with a moist sponge whiIe the
distal collapsed and deffated bowe1 is carefuhy examined for possibIe second or third atresic areas. Although duodena1 atresia is commonIy isolated, multipIe atresias are frequent in the distal smaI1 intestine. An aid to examination of the intestine beyond the first site of atresia is injection of saline soIution or minera 0iI aIong with some air dehvered through a No. 23 or 24 size needle. The liquid and bubbIes may then be milked along and successfu1 distal intraIuminaI passage observed if the atresia is isolated. (Fig. 6.) If a barium enema has been performed inspection of the coIon is unnecessary . Having examined the entire bowel, the surgeon is now ready to perform the anastomosis at one or more sites. The enormous size of the proxima1 segment, balIooned with trapped gas and meconium Auid, may suggest to the inexperienced surgeon that side to side anastomosis represents the onIy conceivable soIution. Such an anastomosis is undesirabIe [la] and shouId be avoided if possible. An end to end or end to side anastomosis is the ideal means of re-estabIishing intestinaf continuity. As Swenson has recommended, the dilated proxima1 bowel is resected taking care to divide the mesentery cIose to the bowel wall [a]. The outer Iayer of the anastomosis is perbIack formed with No. 4-o or 5-o atraumatic siIk suture whiIe No. 5-o to 6-o chromic catgut with swaged on needIe is used for the inner Iayer. Despite resection of the bIind proxima1
FIG. 6. Liquid and air bubbIes distend coIIapsed segment distal to atresia of the smaI1 bowe1. Note proximal diIated segment above site of atresia.
452
Congenital
Atresia
of Small Intestine
FIG. 7. A, the dilated proxima1 bowe1 which is to be resected. B, This shows distention of the dista1 seement and detaiIs of the end to side anastomosis in C, D and E.
pouch there is apt to he considerabIe disparity in the diameter of the bowel to be reunited. As an aid to placing the first layer of silk sutures a Pott,‘s cIamp may be used to occlude the most proximal segment of the distal collapsed bowel which is then intIated with air by injection. (Fig. 7.j Each catgut and silk suture must be preciseIy pIaced 2 to 3 mm. apart with no ever&on of the mucosa. The outer layer of sutures must not incIude more than the seromuscular coat because penetration of the mucosa wit1 permit Ieakage and peritonitis. Special care in placing sutures into the dista1 segment is necessary to avoid compromise of the aIready miniscule lumen. Once the anastomosis has been compIeted the surgeon should demonstrate patency by milking air through the new connection. The mesenteric defect is carefully closed with interrupted fine chromicized catgut sutures. I n patients having more than one atresia muItipIe anastomoses wiI1 be required. When atresic areas are closely situated resection of a short segment of normal intervening bowe1 is preferable thus making it possible to reestablish continuity with a single anastomosis.
When several sausage shaped bits of atresic bowel are left in situ they may grow and cause cvsts, abdominal pain or obstruction. (Fig. 8.) With more than one anastomosis the possibiIity for leakage is greater and when several anastomoses are required the chances of a successful outcome are diminished. In other words, the fewer anastomoses the better, but it is vital to preserve as much Iength of the smaII intestine as possibIe. Blood loss must constantly be appraised by weighing soaked sponges and measuring blood in suction bottIes. AlI blood lost should be replaced during operation and insofar as possible when it is lost. A three-way stopcock is incorporated into the tubing Ieading to the intravenous catheter. Ideally an additional member of the house staff is assigned the sole responsibility of blood repIacement. Pre- and postoperative body weight of the patient (adjusted for organs removed and addition of catheters, bandages and the like) serves as a useful guide to the exactness of blood replacement. Once the anastomosis or anastomoses are completed the surgeon must decide whether 453
StanIey-Brown
FIG. 8. Cystic masses containing cheesy materia1 resected in an older child who initially underwent side to side iIeocoIostomy bypassing segmental atresic areas in the terminal ileum. (Patient of Dr. HaroId A. ZinteI.)
sheath is closed with interrupted sutures of the same materia1 placed 3 to 4 mm. apart. The skin edges are approximated with interrupted sutures of No. 4-o or 5-o bIack siIk. The ques-
or not to construct a temporary gastrostomy. With premature infants and those with highlying obstruction, we have found gastrostomy imme?seIy useful. The details of this operation are we11documented by HoIder and Gross [13]. It is important to suture the stomach securely to the peritoneum and posterior fascia1 layers to prevent Ieakage and peritonitis. CarefuI cIosure of the abdomina1 waI1 is undertaken with No. 4-0 atraumatic chromic catgut as a running suture for the peritoneum and posterior rectus sheath. The anterior rectus
tion of using retention
sutures
depends
some-
what on the Iocation of the incision. In generaI, a high paramedian incision so pIaced that the Iiver wiI1 under-lie and buttress the upper two thirds of the incision may be expected to hea safeIy without retention sutures. If the paramedian incision is IargeIy beIow the Ever or so pIaced that the umbiIicus is at the IeveI 454
Congenital
Atresia
of SmaII Intestine studies obtained. The antibiotic to which the organisms are sensitive shouId be administered intravenousIy thus disturbing the patient as IittIe as possibIe during a period when rest is of the essence. GentIe nasopharyngeal suction is instituted as required and vital signs (pulse and respiration) are observed at regular frequent intervals. We have given a 3 per cent gIucose in one quarter normal saIine soIution intravenousIy, changing to poIyionic preparations containing potassium and other eIectroIytes after twentyfour hours. Hemoglobin and microhematocrit are determined six to eight hours postoperativeIy and, if needed, blood may be transfused. SmaII pIasma and bIood transfusions are helpful over the postoperative period. After forty-eight hours, nasogastric suction is discontinued and the first ora or gastrostomy feeding started with 3 to IO m1. of gIucose and water repeated every two to three hours. If such feedings are absorbed in two to three hours, as proved by gastric aspiration, dilute formula of evaporated milk and water (one part to ten) is cautiousIy begun, IO to 15 cc. at a time. If vomiting occurs the stomach should be Iavaged with warm water, aspirated until empty and feedings restarted after a rest of three or four hours, beginning again with gIucose and water. In some patients the anastomosis may be exasperatingIy SIOW to function and intermittent nasogastric suction will have to be continued for several days. The first stools passed are usually composed of meconium and oId bIood; however, once bile appears in the stool, the surgeon may reIax in the knowledge that the gastrointestina1 tract is now patent. In the absence of vomiting, the formula may be increased in strength and amount but the suction machine and Iaryngoscope are kept near at hand, because of the ever-present possibihty of vomiting and aspiration. At our Hospital aI1 nurses have been instructed in the use of the Iaryngoscope by members of the Anesthesia Staff. Since inauguration of this teaching program, many instances of successfu1 resuscitation by the nurse have been recorded. For the patient who has undergone surgery for atresia of the smaII bowel the postoperative road to recovery is long, bordered by many pitfaIls and open trenches. A wound infection may Iead to dehiscence, which is apt to prove
of the middle pole of the incision, retention sutures of No. 2-o or 3-o bIack silk may be pIaced through al1 Iayers except the peritoneum. These may be secured with buttons as described by Potts [14] or tied over a smaI1 gauze rol1. The dressing should be smaI1 since buIky dressings obscure the abdomen and do not allow auscuItation or adequate inspection. A narrow gauze strip may be secured with one or two 35 inch widths of adhesive tape and then held in pIace by three or four singIe turns of gauze rolIer bandage compIeteIy encircIing the patient’s abdomen. It is convenient and advisable to dispense with the dressing aItogether after twenty-four hours. An alternative is to cIose the skin with subcuticuIar sutures of continuous or interrupted fine siIk or catgut and thereafter to coat the skin surface with pIastic spray or coIIodion. If gastrostomy has been performed it is vita1 that the tube be securely anchored in place, for even premature infants have demonstrated incredibIe ski11 in pulling out the gastrostomy tube. POSTOPERATIVE
CARE
Postoperative care commences the instant the skin closure is compIeted. The patient is returned to his Isolette which has been kept warm during operation. The nasogastric or gastrostomy tube is re-attached to intermittent suction with the Gomco Thermotic Drainage pump.* With the miniature nasogastric tubes necessary for the premature or normal newborn infant, a tiny Aeck of mucus or a bit of secretion may compIeteIy occlude the lumen; thus, the tube should be irrigated at Ieast once every hour with 5 to IO m1. of norma saIine soIution. The efficacy of these tubes is aImost entireIy dependent upon carefu1 supervision. The experienced surgeon will check and aspirate the tube himseIf at least once every twenty-four hours. An atmosphere of high humidity is heIpfu1 and the Vapojette Supersaturation Attachment@t (Fig. g) produces an exceIIent fog. We have not used antibiotics routineIy except for those infants with a moist “soupyappearing” umbilica1 cord stump. Such an obvious site of contamination shouId be cuItured and appropriate antibiotic sensitivity * Gomco Surgical Manufacturing Corp., New York. t Air-Shields Corp., Hatboro, PennsyIvania.
Buffalo,
455
StanIey-Brown
FIG. g. The IsoIette photographed with the Vapojette Supersaturation Attachment in pIace. This device may be activated by a flow of oxygen or through use of a specia1 pump avaiIabIe from Air-Shields Corporation.
accIimatization is particuIarIy important for a mother with her first baby. It is wise to reassure the mother that the infant wiI1 not “come apart” and that he may be handIed and fondIed without fear. The premature infant may be discharged once body weight has reached 555 pounds. In the absence of postoperative compIications patients with normal birth weights usuaIIy remain in the nursery an average of ten to fifteen days after operation.
fataI. An anastomotic Ieak with peritonitis is rareIy saIvabIe and emphasizes the absoIute importance of precise and exact technic in constructing the anastomosis. IntestinaI obstruction caused by adhesions may require reoperation. Constant nursing observation is essentia1, known in some hospitals as direct reIief. SimpIy stated, the nurse may not Ieave her patient unattended for so much as a singIe second. The greatest possible credit goes to our nurses who with skiI1, Iove and devoted interest have safeIy guided the majority of our patients out of the VaIIey of the Shadow of Birth [IT], a journey made a11 the more precarious by surgical trauma. When the patient progresses to fuI1 formuIa and gains weight daiIy it is important to have the mother feed her new baby and “get acquainted” in the hospita1 nursery. Such
SUMMARY
CongenitaI atresia of the smaI1 intestine is rare, uniformIy fatal if untreated and demands earIy diagnosis and careful preoperative preparation. Corrective surgery must be precise, designed to remedy the Iesion in a singIe procedure and conducted with carefu1 attention to bIood repIacement and maintenance of body warmth. SkiIIed anesthetic management is of 456
CongenitaI the essence and the postoperative demanding in the extreme.
Atresia care
of SmaII -.
is
2. 3.
4.
5.
6.
RANDOLPH, J. Technique for insertion of plastic catheter into saphenous vein. Pediaks, 24: 631,
1959. 8. STAXEY-BKOWU, E. G. The venous cutdown. Arch. Pediat., 75: 480, 1958. g. TALROT, N. B., CRAWFORD, J. D. and Br TLU, A. 51. Homeostatic limits to safe parenteral fluid therapy. New England J. A/ied., 248: I loo, 1953. IO. Report of Committee on Nutrition, American Academy of Pediatrics, hlarch I 961. Pediatrics, 28: 501, 1961. r I. SMITH, R. Cl. Anesthesia for Infants and Children, p. 78. St. Louis, 1959, C. V. hlosby Co. 12. HURWITZ, A. Reappraisal of side-to-side and endto-side intestinal anastomoses. Surgeyt-, 43: 864,
REFERENCES I.
Intestine
T. Harveian lectures on the mode of death from acute intestinal strangulation and chronic intestinal obstruction. Ann. Surg., I: 177, 1885. WANGENSTEEN, 0. H. Intestinal Obstructions, Ed. 3. Springfield, III., 1955. CharIes C Thomas. STANL.EY-BROWN,E. C., ZINTEL, H. A. and EAGLE, J. F. An anaIysis of operative deaths in infants and children. Surg. Gynec, @ Obst., I 14: 137, 1962. SWENSON, 0. and FISHER, J. H. SmaIl bowel atrcsia: treatment by resection and primary aseptic anastamosis. Surgery, 47: 823, 1960. BISHOP, H. C. Safe transportation of newborn infants for emergency surgery. J. A. M. A., 165: 123o, 1957. BISHOP, H. C. Factors affecting success and faiIure in neonatal surgery. Pediat. C&n. North America, 6: 945, ‘959. BRYANT,
1958. 13. HOLDER, T. ,\I. and GROSS, R. E. Temporarv gastrostomy in pediatric surgthry. Pediatrics. 26’: 36, rg6o. 14. POTTS, W. J. The Surgeon and the Child, p. 4;. Philadelphia, 1959. W. B. Saunders Co. 15. SMUITH,C. A. The vaIIey of the shadow of birth. Am. J. Dis. Cbild., 82: 171, 1951.
457