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The problem of “giant” omphalocele
The Problem of "Giant" Omphalocele By Barbara H. Towne, George Peters, and Jack H. T. Chang Denver, Colorado 9 "Giant" omphalocele implies an abdominal wall defect that is 5 cm or more in diameter with the liver in a central position, Giant omphalocele is often associated with other significant anomalies, Technically it is often difficult to close the abdominal wall defect and a staged repair utilizing prosthetic materials may be necessary. The morbidity and mortality associated with this entity remain significant despite advances in m a n a g e m e n t techniques. Hospitalization is often prolonged and costly. For the best outcome in managing patients with giant omphalocele early attention to hypothermia and other metabolic requirements and long-term attention to nutritional needs are important. Techniques of closing the omphalocele should be adapted to the individual characteristics of the defect, but mobilization and stretching of the abdominal muscles should be begun as a newborn, The term hepatomphalocele implying "liver-containing omphalocele'" is suggested to refer to this particular entity. INDEX W O R D : Omphalocele.
M P H A L O C E L E is a condition that has been recognized for centuries. Small omphaloceles have been closed successfully since the 1800s, but the management of "giant" omphalocele remains a challenge to pediatric surgeons. In recent years there have been several excellent reviews of large series of patients with omphalocele and gastroschisis. ~ ~ They have documented dramatic improvement in the results of management of patients with abdominal wall defects. However, for the small group of patients with giant omphalocele the morbidity and mortality statistics remain discouraging. The term giant omphalocele is not welldefined, but usually implies an abdominal wall defect greater than 5 cm in diameter. In addition, because of the large defect the liver assumes a central position and herniates into the omphalocele. It is the presence of the centrally located, nonyielding liver that makes these defects hard to close. Another striking feature in many patients with giant omphalocele is that the rectus muscles do not meet in the midline superiorly but, rather, attach to the costal margin laterally as shown diagramatically in Fig. 1. Although this feature has not been widely recognized, it is a logical consequence of the failure of complete development of the cephalic fold of
O
Journal of Pediatric Surgery, Vol. 15, No. 4 (August), 1980
embryonic mesenchyme in the upper celosomia or upper midline defects as described by Duhamel, 12 Izant, 13 and others. 1'14 Lateral attachment of the rectus muscles was noted in 45% of the patients with giant omphalocele in the series to be reviewed. At Denver Children's Hospital, which is a referral center for several surrounding states, we have recently encountered an unusual number of newborns with giant omphaloceles. Because their care has been so complicated and often so prolonged, we have chosen to focus on this problem. CLINICAL MATERIAL All cases of omphalocele seen at Denver Children's Hospital (DCH) in the 3-yr period from 1975 to 1978 were reviewed. There were 30 cases of omphalocele of which 7 were small or "simple" and 23 were "giant" as previously defined. No obvious etiology for omphalocele has been identified so far and there were no obvious predisposing maternal factors defined in this series, although 5 mothers had infections early in pregnancy that were treated with antibiotics in 4 cases. The mothers were between 15 and 35 yr of age. Of the infants, 18 were boys and 12 were girls. All but 5 of the patients weighed 5 lb or more. The majority of the babies were born at term and appropriate or large for gestational age. Due to evaporative heat lose, more than half of the patients were hypothermic when the D C H transport team arrived at the referring hospital. Wrapping the omphalocele in warm saline-soaked gauze and placing the lower half of the baby in a plastic bag prevented further temperature reduction. With few exceptions, normothermia had been achieved by the time of arrival at DCH. Intravenous solutions and antibiotics were given to all patients. It is well-recognized that omphalocele is often associated with other anomalies. The distribution of anomalies in this series is shown in Table 1. Omphalocele often accompanies chromosomal abnormalities as represented by one patient in this series. Two patients had Beekwith's syndrome of organ hypertrophy with hyperinsulinism and hypoglycemia. ~5
From the Children's Hospital, Denver, Colo. Presented before the 28th Annual Meeting of the Surgical Section o f the American Academy o f Pediatrics, San Francisco, California, October 13 14, 1979. Address reprint requests to Barbara H. Towne, Department o f General Pediatric Surgery, 1056 East Nineteenth Avenue, Denver, Colo. 80218. 9 1980 by Grune & Stratton, Inc. 0022-3468/80/1504~032501.00/0 543
544
TOWNE, PETERS, AND CHANG
Table 2. Results of Giant Omphalocele 23 Cases
Type Repair None Primary Staged Skin Silon Teflon mesh
S
Number of Patients
Complicated
Lived
14 6 7 1
5 12 5 6 1
1 5 17 7 9 1
Died 1 5 2 3
to be in other anomalies involving the diaphragm. It is not clear, however, whether reflux is intrinsic or secondary to the mechanics of repair. RESULTS
Fig. 1, Lateral displacement of rectus muscles in giant omphalocele.
Three patients had a complex of anomalies known as the upper midline syndrome or the Pentology of Cantrell. ~6 This includes giant omphalocele associated with diaphragmatic, pericardial, sternal, and often severe cardiac anomalies. ~7-z~ One patient represented the lower midline syndrome or lower celosomia defect which includes bladder exstrophy and hindgut agenesis. All of these syndromes should be recognized early for appropriate medical management and prediction of outcome.
Genitourinary anomalies frequently accompany omphalocele and 5 of 12 males with giant omphalocele had 1 or both testicles undescended with a very small scrotal sac. It would appear that the mechanism responsible for incomplete development of the abdominal wall also interferes with testicular descent. Malrotation and malfixation of the bowel are considered by many to be an intrinsic feature of giant omphalocele. These anomalies were specifically noted or corrected in seven patients. Gastroesophageal reflux was a common problem in these patients as it has been recognized Table 1. Omphalocele Associated Anomalies Simple
Giant
Chromosomes
1
Musculoskeletal
6
15
Cardiac Pulmonary (1 ~ Genitourinary Gastrointestinal Vascular
1 1 8 3
10 4 13 17 5
The seven cases of simple or small omphalocele have all had good long-term results whether repaired primarily 4 or secondarily. 3 Similarly, the five cases of giant omphalocele that could be closed primarily have all lived, and no significant complications occurred as a result of their treatment (Table 2). However, of the 17 cases of giant omphalocele which required staged repair, 14 patients experienced major complications and 5 patients, or 29% of the total, died as a consequence of surgical repair or associated anomalies. Major complications were the rule rather than the exception in the patients who required staged repair because of the size of the defect (Table 3). Table 3. Complications Staged Repair "Giant'" Omphalocele 17 Cases Abdominal wall Cellulitis Breakdown 1 ~ repair Residual hernia After 1 o repair only After staged repair Pulmonary Prolonged atelect~sis Respirator support Pulmonary hypertension Tracheostomy Cardiac Congestive heart failure Vascular (Budd-Chiad Syndrome) Gastrointestinal Prolonged ileus Obstruction Chalasia Failure to thrive Central hyperalimentation Perforation or fistula Sepsis (documented)
12 9 1 8 2 12 4 1 9 1 4 4 4 6 8 3 5
OMPHALOCELE
Edema and erythema of the abdominal wall following surgery was common and in nine patients breakdown of some element of the primary repair occurred. One extreme example was a patient who developed systemic and abdominal candidiasis during a staged repair of a ruptured omphalocele utilizing a silon pouch. Massive abdominal wall cellulitis resulted in breakdown of skin and fascia. After several months of intensive therapy, including intravenous Amphotericin B, this patient was finally sent home with small area of healing skin. Nine patients were left with some residual hernia defect when last seen or at the time of death. Pulmonary and cardiac complications were common. Twelve patients required ventilator support, occasionally for months following one or more stages of repair; one required tracheostomy. Of 9 patients treated for congestive heart failure 4 were secondary to primary pulmonary hypertension. An interesting vascular complication has already been reported. 2~ Following a staged reduction with silon one patient developed ascites which proved to be secondary to a BuddChiari phenomenon due to obstruction of the inferior vena cava at the level of the diaphragm. This was thought to be secondary to rotation of the liver as a result of surgery. Waldman 2~ has recently reported angulation of the junction of the inferior vena cava and the right atrium (diaphragm) in three patients with repaired omphalocele undergoing cardiac catheterization. Abnormalities of the hepatic veins as well as the vena cava were noted either at surgery or autopsy in five patients in this series. Gastrointestinal complications were distressingly frequent. Four patients had prolonged ileus postoperatively, and four required exploratory laparotomy for obstruction after an initial repair. Eight patients required central intravenous nutrition during their treatment. In three patients intestinal perforation or fistula complicated staged repair with prosthetic materials. One patient who had teflon mesh inserted as a secondary procedure developed enterocutaneous fistulae in which the mesh was densely incorporated. Removing the mesh required resection of three segments of bowel. After eight surgical procedures, 274 days in the hospital, and a hospital bill to date of $112,000 this patient still has a large ventral hernia.
545
Table 4. Length and Cost of Hospitalization in Staged Repair of "Giant" Omphaloeele--17 Cases Range
Hospitalization Operations Hospital days Hospital bit[
1-6 1- 10 7-274 $1 O- 112,000
Average 2 3 88 $42,000
In reviewing this series of patients the economic morbidity was examined (Table 4). The average hospital stay was 88 days, and the average bill for hospitalization alone was $42,000. Giant omphalocele is one of the most expensive entities treated by pediatric surgeons, and the toll it takes on both patients and parents is obvious. The causes of death in the five patients are listed in Table 5. Most had prolonged hospitalizations prior to death. Only the last of these patients left the hospital for any period of time, but the last five months of his life were spent in the hospital where a series of operations were performed to repair his abdominal wall only to have him die of aspiration secondary to chalasia at 1 yr of age. DISCUSSION
The history of management of omphalocele is dramatic and marked by significant breakthroughs in recommended treatment with associated improvement in survival statistics. The metabolic and nutritional needs of infants with omphalocele have long been of concern to pediatric surgeons. Philippart 23 defined the fluid requirements of patients with abdominal wall defects and Filler 24 stressed the need for total intravenous nutrition as an adjunct to the management of infants with ruptured omphalocele. Gutenberger 25 documented the hypoproteinemia which accompanies ruptured omphalocele and gastroschisis. However, the technical aspects of providing coverage of the often huge Table 5. Mortality in Staged Repair of "'Giant" Omphalocele ( 5 / 1 7 Cases = 29%) Patient J.S. C.C. S.S. C.S. L.M.
Causeof Death Cardiac dysfunction
Respiratory insufficiency Pneumonia, renal failure Bowel dysfunction, chf Aspiration pneumonitis
Age at Death 7 days 2 mo 3 mon 5 mo 1 yr
546
defects has provided the greatest challenge in the management of giant omphalocele. Because of high mortality associated with surgical management, many surgeons have advocated nonoperative treatment of giant omphalocele.26 29 Early non-operative management included painting the sac with sclerosing agents, usually mercurochrome, and allowing skin to migrate over the defect. Because of reported cases of toxicity secondary to sclerosing agents, 3~ others have recommended that the intact sac or a staged closure which has failed, be treated by covering the defect with skin, 3~ amniotic, 32 or synthetic membranes. 33 In 1948, Gross 34 described surgically mobilizing skin flaps to cover the defect over an intact sac. The problems with this mode of therapy are often dense adhesions between intestines and skin, and failure to enlarge the abdominal cavity by mobilizing the rectus muscles. However, staged repair following p r i m a r y skin closure has been described by Boles35 and others. The modern era of omphalocele management began with the demonstration by Schuster 36 and others 37 39 of the use of prosthetic materials such as silon to stretch the rectus muscles and to bridge any residual defect. Shermeta, 4~ Wexler, 4~ and Othersen 4z have all described interesting variations on the silon pouch that have facilitated reduction in their hands. Pickett has been credited ~3 with the idea of bluntly stretching the abdominal wall at the time of definitive repair to enlarge the abdominal cavity. Spratt, 43 Ravitch, 44 and Jones 45 have described utilization of internal or external pneumatic compression to stretch the abdominal cavity prior to staged repair of the residual ventral hernia. Talbert 46'47 has advocated using teflon mesh to bridge ventral hernia defects emphasing the material's nonreactivity with the intestine. Our only experience with teflon mesh resulted in massive adhesions and multiple enteric fistulae. Several authors describe leaving small pieces of silon as long-term protheses, and we have utilized lightweight (0.02 inches) silon as a prosthesis, especially over the liver. Heavy silon is too stiff and can create problems by eroding through the overlying skin. CantreW 6 described reflecting the anterior rectus sheath to bridge the defect between laterally displaced recti in the upper midline syndrome. The use of rotational flaps of
TOWNE, PETERS, AND CHANG
muscle and skin to bridge abdominal wall defects has been described by Croom and Thomas 48 in the repair of gastroschisis; however, their method has not been widely adopted since gastroschisis can usually be treated by either primary repair or skin closure. A recent report from Japan by Aoyama 49 describes utilizing flaps of reflected rectus abdominus and external oblique muscles to bridge ventral hernia defects in older children. However, following staged repair with skin or silon in the newborn, these muscle layers are often distorted or densely adhesed and might make creation of adequate flaps difficult. This method has not been tried as a primary procedure in newborns, although the concept of using the patients own tissues for repair is appealing. CONCLUSIONS
This series of 23 patients with giant omphalocele has allowed us to come to several conclusions about the management of this difficult defect. Early attention to hypothermia and fluid loss are important, especially if the patient is to be transported long distances. An omphalocele should prompt pediatricians and surgeons alike to observe for commonly associated syndromes and anomalies, especially those which may present additional metabolic problems such as hypoglycemia or hypoxia prior to or during initial surgery. Early nutritional support for gastrointestinal dysfunction is essential. Peripheral intravenous nutrition should be utilized until sepsis is ruled out and all exposed prosthetic materials removed. Central intravenous nutrition may be required, although the risk of its being a nidus for infection must be considered. As advocated by Mahour, 5~ initial abdominal exploration is indicated in most cases to diagnose and treat associated intraabdominal anomalies. Mobilization and stretching of the rectus muscles and at least partial fascial closure should be attempted in the newborn period; otherwise the abdominal parites gradually retract as the infant grows making the definitive repair difficult if not impossible. There are various ways this goal can be achieved. As described by Wesselhoeft and Randolph, 51 management should be based on the individual characteristics of the defect. In addition, the pediatric surgeon should be alert to the significant gastrointestinal,
OMPHALOCELE
547
pulmonary and cardiac complications associated w i t h g i a n t o m p h a l o c e l e in o r d e r to m i n i m i z e them. M a n y i n v e s t i g a t o r s a r e w o r k i n g o n w a y s to improve the use of prosthetic materials and m u s c l e flaps. H o w e v e r , t h e b e s t m a t e r i a l or method has not yet been identified.
Finally, a more scientific term than giant o m p h a l o c e l e s h o u l d b e u s e d to s p e c i f i c a l l y i d e n t i f y t h i s e n t i t y w h i c h c a n b e so d i f f i c u l t t o t r e a t . We suggest that the term hepatomphalocele implying a "liver-containing" omphalocele be u t i l i z e d b e c a u s e it is t h i s a n o m a l y t h a t so c o m p l i cates the problem of giant omphalocele.
REFERENCES
1. Aitken J: Exomphalos: Analysis of a 10 year series of 32 cases. Arch Dis Child 38:126-129, 1963 2. Jones PG: Exomphalos (syn. omphalocele). A review of 45 cases. Arch Dis Child 38:180-187, 1963 3. Johnson AH: Omphalocele and related defects. Am J Surg 114:279-284, 1967 4. Simpson TE, Lynn HB: Omphalocele: Results of surgical treatment. Mayo Clin Proc 43:65-69, 1968 5. Mahour GH, Weitzman J J, Rosenkrantz JG: Omphalocele and gastroschisis. Ann Surg 177:478-482, 1973 6. Girvan DP, Webster DM, Shandling B: The treatment of omphalocele and gastroschisis. Surg Gynecol Obstet 139:222-224, 1974 7. Raffensperger JG: Congenital defects of the gastrointestinal tract and abdominal wall: A three year review. Am J Dis Child 129:1145-1150, 1975 8. Venugopal S, Zachary RB, Spitz L: Exomphalos and gastroschisis: A 10-year review. Br J Surg 63:523-525, 1976 9. Kim SH: Omphalocele. Surg Clin North Am 56:361371, 1976 10. Moore TC: Gastroschisis and omphalocele: Clinical differences. Surg 82:561 568, 1977 11. Seashore JH: Congenital abdominal wall defects. Clin Perinatol 5:61-77, 1978 12. Duhamel B: Embryology of exomphalos and allied malformations. Arch Dis Child 38:142-147, 1963 13. Izant R J, Brown F, Rothmann BF: Current embryology and treatment of gastroschisis and omphalocele. Arch Surg 93:49-53, 1966 14. Janosko EO, Jona JZ, Belin RP: Congenital anomalies of the umbilicus. Am Surg 43:177-185, 1977 15. Beckwith JB: Macroglossia, omphalocele, adrenal cytomegaly, gigantism and hyperplastic visceromegaly, in Birth Defects: Original Article Series V:188-196, 1969 16. Cantrell JR, Haller JA, Ravitch MM: A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet 107:602 614, 1958 17. Crittenden I, Adams FH, Mulder DG: A syndrome featuring defects of the heart, sternum, diaphragm and anterior abdominal wall. Circulation 20:396-404, 1959 18. Eraklis A J, Trump DS, Longino LA: Omphalocele with diaphragmatic and pericardial defects: Diagnosis and repair. J Pediatr Surg 2:354-358, 1967 19. Toyama WM: Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: A case report and review of the syndrome. Pediatrics 50:778-792, 1972 20. Greenwood RD, Rosenthal A, Nadas AS: Cardiovas-
cular malformations associated with omphalocele. J Pediatr 85:818-821, 1974 21. Carlton GR, Towne BH, Bryan RW, et al: Obstruction of the suprahepatic inferior vena cava as a complication of giant omphalocele repair. J Pediatr Surg (in press) 22. Waldman JD, Fellows KE, Paul MH, et al: Angulation of the inferior vena cava-right atrial junction in children with repaired omphalocele. Pediatr Radiol 5:142 144, 1977 23. Philippart A|, Canty TG, Filler RM: Acute fluid volume requirements in infants with anterior abdominal wall defects. J Pediatr Surg 7:553-558, 1972 24. Filler RM, Eraklis A J, Das JB: Total intravenous nutrition: An adjunct to the management of infants with ruptured omphalocele. Am J Surg 121:454-459, 1971 25. Gutenberger JE, Miller DL, Dibbins AW et al: Hypogammaglobulinemia and hypoalbuminemia in neonates with ruptured omphaloceles and gastroschisis. J Pediatr Surg 8:353 359, 1973 26. Grob M: Conservative treatment of exomphalos. Arch Dis Child 38:148-150, 1963 27. Soave F: Conservative treatment of giant omphalocele. Arch Dis Child 38:130-134, 1963 28. Drescher E: Observations on the conservative treatment of giant omphalocele. Arch Dis Child 38:135-137, 1963 29. Fior HV: Omphalocele--An appraisal of therapeutic approaches. Surg 69:208-214, 1971 30. Fagan DG, Pritchard JS, Clarkson TW, et al: Organ mercury levels in infants with omphaloceles treated with organic mercurial antiseptic. Arch Dis Child 52:962-964, 1977 31. KlingS: Massive omphalocele: Amethodoftreatrnent employing skin allograft. Can J Surg 10:445, 1967 32. Seashore JH, MacNaughton R J, Talbert JL: Treatment of gastroschisis and omphalocele with biological dressings. J Pediatr Surg 10:9 17, 1975 33. Ein SH, Shandling B: A new nonoperative treatment of large omphaloceles with a polymer membrane. J Pediatr Surg 13:255-257, 1978 34. Gross RE: New method for surgical treatment of large omphaloceles. Surg 24:277-292, 1948 35. Boles ET: Staged repair of huge ventral hernias. J Pediatr Surg 6:618-626, 1971 36. Schuster SR: A new method for the staged repair of large omphaloceles. Surg Gynecol Obstet 125:837-850, 1967 37. Gilbert MG, Meneia LF, Brown WT, et al: Staged repair of large omphaloceles and gastroschisis. J Pediatr Surg 3:702-709, 1968
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38. Allen RG; Wrenn EL: Sil0n as a sac in the treatment of omphalocele and gaslroschisis. J Pediatr Surg 4:3-8, 1969 , 39. Rubin SZ, Ein SH: Experience with 55 silon pouches. J Pediatr Surg 11:803-807, 1976 40. Shermeta DW: Simplified treatment of large congenital ventral wall defects. Am J Surg 133:78-80, 1977 41. Wexler HR, Hailer JA: A noninvasive method for controlled reduction of omphalocele prostheses. J Pediatr Surg 6:774--778, 1971 42. Othersen HB, Hargest TS: A pneumatic reduction device for gastroschisis and omphalocele. Surg Gynecol Obstet 144:243-248, 1977 43. Spratt JS: Artificial pneumothorax: Efficacy in enlarging the peritoneal cavity for early secondary closure of congenital omphalocele. Am J Surg 101:375-377, 1961 44. Ravitch MM: Omphalocele: Secondary repair with the aid of pneumoperitoneum. Arch Surg 99:166-170, 1969 45. Jones PG: Delayed repair of large omphaloceles; preparation by external pneumatic compression. J Pediatr Surg 6:507, 1971 (abstr)
TOWNE, PETERS, AND CHANG
46. Talbert JL, Rodgers BM, Moazam F: Surgical management of massiveventral hernias in children. J Pediatr Surg 12:63-67~ 1977 47. Moazam F, Rodgers BM, Talbert JL: Use of teflon mesh for repair of abdominal wall defects in neonates. J Pediatr Surg 14:347-351, 1979 48. Croom RD, Thomas CG: Repair of gastroschisis. Surg Gynecol Obstet 132:689-692, 1971 49. Aoyama K: A new operation for repair of large ventral hernias following giant omphalocele and gastroschisis. J Pediatr Surg 14:172-176, 1979 50. Mahout GH: Intact omphalocele: Perennial dilemma of operative or "conservative" management. Am J Surg 128:419 420, 1974 51. Wesselhoeft CW, Randolph JR: Treatment of omphalocele based on individual characteristics of the defect. Pediatrics 44:101-108, 1969 52. Agnew LRC, et al.: Dorland's Illustrated Medical Dictionary (ed 24). Philadelphia, Saunders, 1965, p 668
M P H A L O C E L E is a condition that has been recognized for centuries. Small omphaloceles have been closed successfully since the 1800s, but the management of "giant" omphalocele remains a challenge to pediatric surgeons. In recent years there have been several excellent reviews of large series of patients with omphalocele and gastroschisis. ~ ~ They have documented dramatic improvement in the results of management of patients with abdominal wall defects. However, for the small group of patients with giant omphalocele the morbidity and mortality statistics remain discouraging. The term giant omphalocele is not welldefined, but usually implies an abdominal wall defect greater than 5 cm in diameter. In addition, because of the large defect the liver assumes a central position and herniates into the omphalocele. It is the presence of the centrally located, nonyielding liver that makes these defects hard to close. Another striking feature in many patients with giant omphalocele is that the rectus muscles do not meet in the midline superiorly but, rather, attach to the costal margin laterally as shown diagramatically in Fig. 1. Although this feature has not been widely recognized, it is a logical consequence of the failure of complete development of the cephalic fold of
O
Journal of Pediatric Surgery, Vol. 15, No. 4 (August), 1980
embryonic mesenchyme in the upper celosomia or upper midline defects as described by Duhamel, 12 Izant, 13 and others. 1'14 Lateral attachment of the rectus muscles was noted in 45% of the patients with giant omphalocele in the series to be reviewed. At Denver Children's Hospital, which is a referral center for several surrounding states, we have recently encountered an unusual number of newborns with giant omphaloceles. Because their care has been so complicated and often so prolonged, we have chosen to focus on this problem. CLINICAL MATERIAL All cases of omphalocele seen at Denver Children's Hospital (DCH) in the 3-yr period from 1975 to 1978 were reviewed. There were 30 cases of omphalocele of which 7 were small or "simple" and 23 were "giant" as previously defined. No obvious etiology for omphalocele has been identified so far and there were no obvious predisposing maternal factors defined in this series, although 5 mothers had infections early in pregnancy that were treated with antibiotics in 4 cases. The mothers were between 15 and 35 yr of age. Of the infants, 18 were boys and 12 were girls. All but 5 of the patients weighed 5 lb or more. The majority of the babies were born at term and appropriate or large for gestational age. Due to evaporative heat lose, more than half of the patients were hypothermic when the D C H transport team arrived at the referring hospital. Wrapping the omphalocele in warm saline-soaked gauze and placing the lower half of the baby in a plastic bag prevented further temperature reduction. With few exceptions, normothermia had been achieved by the time of arrival at DCH. Intravenous solutions and antibiotics were given to all patients. It is well-recognized that omphalocele is often associated with other anomalies. The distribution of anomalies in this series is shown in Table 1. Omphalocele often accompanies chromosomal abnormalities as represented by one patient in this series. Two patients had Beekwith's syndrome of organ hypertrophy with hyperinsulinism and hypoglycemia. ~5
From the Children's Hospital, Denver, Colo. Presented before the 28th Annual Meeting of the Surgical Section o f the American Academy o f Pediatrics, San Francisco, California, October 13 14, 1979. Address reprint requests to Barbara H. Towne, Department o f General Pediatric Surgery, 1056 East Nineteenth Avenue, Denver, Colo. 80218. 9 1980 by Grune & Stratton, Inc. 0022-3468/80/1504~032501.00/0 543
544
TOWNE, PETERS, AND CHANG
Table 2. Results of Giant Omphalocele 23 Cases
Type Repair None Primary Staged Skin Silon Teflon mesh
S
Number of Patients
Complicated
Lived
14 6 7 1
5 12 5 6 1
1 5 17 7 9 1
Died 1 5 2 3
to be in other anomalies involving the diaphragm. It is not clear, however, whether reflux is intrinsic or secondary to the mechanics of repair. RESULTS
Fig. 1, Lateral displacement of rectus muscles in giant omphalocele.
Three patients had a complex of anomalies known as the upper midline syndrome or the Pentology of Cantrell. ~6 This includes giant omphalocele associated with diaphragmatic, pericardial, sternal, and often severe cardiac anomalies. ~7-z~ One patient represented the lower midline syndrome or lower celosomia defect which includes bladder exstrophy and hindgut agenesis. All of these syndromes should be recognized early for appropriate medical management and prediction of outcome.
Genitourinary anomalies frequently accompany omphalocele and 5 of 12 males with giant omphalocele had 1 or both testicles undescended with a very small scrotal sac. It would appear that the mechanism responsible for incomplete development of the abdominal wall also interferes with testicular descent. Malrotation and malfixation of the bowel are considered by many to be an intrinsic feature of giant omphalocele. These anomalies were specifically noted or corrected in seven patients. Gastroesophageal reflux was a common problem in these patients as it has been recognized Table 1. Omphalocele Associated Anomalies Simple
Giant
Chromosomes
1
Musculoskeletal
6
15
Cardiac Pulmonary (1 ~ Genitourinary Gastrointestinal Vascular
1 1 8 3
10 4 13 17 5
The seven cases of simple or small omphalocele have all had good long-term results whether repaired primarily 4 or secondarily. 3 Similarly, the five cases of giant omphalocele that could be closed primarily have all lived, and no significant complications occurred as a result of their treatment (Table 2). However, of the 17 cases of giant omphalocele which required staged repair, 14 patients experienced major complications and 5 patients, or 29% of the total, died as a consequence of surgical repair or associated anomalies. Major complications were the rule rather than the exception in the patients who required staged repair because of the size of the defect (Table 3). Table 3. Complications Staged Repair "Giant'" Omphalocele 17 Cases Abdominal wall Cellulitis Breakdown 1 ~ repair Residual hernia After 1 o repair only After staged repair Pulmonary Prolonged atelect~sis Respirator support Pulmonary hypertension Tracheostomy Cardiac Congestive heart failure Vascular (Budd-Chiad Syndrome) Gastrointestinal Prolonged ileus Obstruction Chalasia Failure to thrive Central hyperalimentation Perforation or fistula Sepsis (documented)
12 9 1 8 2 12 4 1 9 1 4 4 4 6 8 3 5
OMPHALOCELE
Edema and erythema of the abdominal wall following surgery was common and in nine patients breakdown of some element of the primary repair occurred. One extreme example was a patient who developed systemic and abdominal candidiasis during a staged repair of a ruptured omphalocele utilizing a silon pouch. Massive abdominal wall cellulitis resulted in breakdown of skin and fascia. After several months of intensive therapy, including intravenous Amphotericin B, this patient was finally sent home with small area of healing skin. Nine patients were left with some residual hernia defect when last seen or at the time of death. Pulmonary and cardiac complications were common. Twelve patients required ventilator support, occasionally for months following one or more stages of repair; one required tracheostomy. Of 9 patients treated for congestive heart failure 4 were secondary to primary pulmonary hypertension. An interesting vascular complication has already been reported. 2~ Following a staged reduction with silon one patient developed ascites which proved to be secondary to a BuddChiari phenomenon due to obstruction of the inferior vena cava at the level of the diaphragm. This was thought to be secondary to rotation of the liver as a result of surgery. Waldman 2~ has recently reported angulation of the junction of the inferior vena cava and the right atrium (diaphragm) in three patients with repaired omphalocele undergoing cardiac catheterization. Abnormalities of the hepatic veins as well as the vena cava were noted either at surgery or autopsy in five patients in this series. Gastrointestinal complications were distressingly frequent. Four patients had prolonged ileus postoperatively, and four required exploratory laparotomy for obstruction after an initial repair. Eight patients required central intravenous nutrition during their treatment. In three patients intestinal perforation or fistula complicated staged repair with prosthetic materials. One patient who had teflon mesh inserted as a secondary procedure developed enterocutaneous fistulae in which the mesh was densely incorporated. Removing the mesh required resection of three segments of bowel. After eight surgical procedures, 274 days in the hospital, and a hospital bill to date of $112,000 this patient still has a large ventral hernia.
545
Table 4. Length and Cost of Hospitalization in Staged Repair of "Giant" Omphaloeele--17 Cases Range
Hospitalization Operations Hospital days Hospital bit[
1-6 1- 10 7-274 $1 O- 112,000
Average 2 3 88 $42,000
In reviewing this series of patients the economic morbidity was examined (Table 4). The average hospital stay was 88 days, and the average bill for hospitalization alone was $42,000. Giant omphalocele is one of the most expensive entities treated by pediatric surgeons, and the toll it takes on both patients and parents is obvious. The causes of death in the five patients are listed in Table 5. Most had prolonged hospitalizations prior to death. Only the last of these patients left the hospital for any period of time, but the last five months of his life were spent in the hospital where a series of operations were performed to repair his abdominal wall only to have him die of aspiration secondary to chalasia at 1 yr of age. DISCUSSION
The history of management of omphalocele is dramatic and marked by significant breakthroughs in recommended treatment with associated improvement in survival statistics. The metabolic and nutritional needs of infants with omphalocele have long been of concern to pediatric surgeons. Philippart 23 defined the fluid requirements of patients with abdominal wall defects and Filler 24 stressed the need for total intravenous nutrition as an adjunct to the management of infants with ruptured omphalocele. Gutenberger 25 documented the hypoproteinemia which accompanies ruptured omphalocele and gastroschisis. However, the technical aspects of providing coverage of the often huge Table 5. Mortality in Staged Repair of "'Giant" Omphalocele ( 5 / 1 7 Cases = 29%) Patient J.S. C.C. S.S. C.S. L.M.
Causeof Death Cardiac dysfunction
Respiratory insufficiency Pneumonia, renal failure Bowel dysfunction, chf Aspiration pneumonitis
Age at Death 7 days 2 mo 3 mon 5 mo 1 yr
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defects has provided the greatest challenge in the management of giant omphalocele. Because of high mortality associated with surgical management, many surgeons have advocated nonoperative treatment of giant omphalocele.26 29 Early non-operative management included painting the sac with sclerosing agents, usually mercurochrome, and allowing skin to migrate over the defect. Because of reported cases of toxicity secondary to sclerosing agents, 3~ others have recommended that the intact sac or a staged closure which has failed, be treated by covering the defect with skin, 3~ amniotic, 32 or synthetic membranes. 33 In 1948, Gross 34 described surgically mobilizing skin flaps to cover the defect over an intact sac. The problems with this mode of therapy are often dense adhesions between intestines and skin, and failure to enlarge the abdominal cavity by mobilizing the rectus muscles. However, staged repair following p r i m a r y skin closure has been described by Boles35 and others. The modern era of omphalocele management began with the demonstration by Schuster 36 and others 37 39 of the use of prosthetic materials such as silon to stretch the rectus muscles and to bridge any residual defect. Shermeta, 4~ Wexler, 4~ and Othersen 4z have all described interesting variations on the silon pouch that have facilitated reduction in their hands. Pickett has been credited ~3 with the idea of bluntly stretching the abdominal wall at the time of definitive repair to enlarge the abdominal cavity. Spratt, 43 Ravitch, 44 and Jones 45 have described utilization of internal or external pneumatic compression to stretch the abdominal cavity prior to staged repair of the residual ventral hernia. Talbert 46'47 has advocated using teflon mesh to bridge ventral hernia defects emphasing the material's nonreactivity with the intestine. Our only experience with teflon mesh resulted in massive adhesions and multiple enteric fistulae. Several authors describe leaving small pieces of silon as long-term protheses, and we have utilized lightweight (0.02 inches) silon as a prosthesis, especially over the liver. Heavy silon is too stiff and can create problems by eroding through the overlying skin. CantreW 6 described reflecting the anterior rectus sheath to bridge the defect between laterally displaced recti in the upper midline syndrome. The use of rotational flaps of
TOWNE, PETERS, AND CHANG
muscle and skin to bridge abdominal wall defects has been described by Croom and Thomas 48 in the repair of gastroschisis; however, their method has not been widely adopted since gastroschisis can usually be treated by either primary repair or skin closure. A recent report from Japan by Aoyama 49 describes utilizing flaps of reflected rectus abdominus and external oblique muscles to bridge ventral hernia defects in older children. However, following staged repair with skin or silon in the newborn, these muscle layers are often distorted or densely adhesed and might make creation of adequate flaps difficult. This method has not been tried as a primary procedure in newborns, although the concept of using the patients own tissues for repair is appealing. CONCLUSIONS
This series of 23 patients with giant omphalocele has allowed us to come to several conclusions about the management of this difficult defect. Early attention to hypothermia and fluid loss are important, especially if the patient is to be transported long distances. An omphalocele should prompt pediatricians and surgeons alike to observe for commonly associated syndromes and anomalies, especially those which may present additional metabolic problems such as hypoglycemia or hypoxia prior to or during initial surgery. Early nutritional support for gastrointestinal dysfunction is essential. Peripheral intravenous nutrition should be utilized until sepsis is ruled out and all exposed prosthetic materials removed. Central intravenous nutrition may be required, although the risk of its being a nidus for infection must be considered. As advocated by Mahour, 5~ initial abdominal exploration is indicated in most cases to diagnose and treat associated intraabdominal anomalies. Mobilization and stretching of the rectus muscles and at least partial fascial closure should be attempted in the newborn period; otherwise the abdominal parites gradually retract as the infant grows making the definitive repair difficult if not impossible. There are various ways this goal can be achieved. As described by Wesselhoeft and Randolph, 51 management should be based on the individual characteristics of the defect. In addition, the pediatric surgeon should be alert to the significant gastrointestinal,
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pulmonary and cardiac complications associated w i t h g i a n t o m p h a l o c e l e in o r d e r to m i n i m i z e them. M a n y i n v e s t i g a t o r s a r e w o r k i n g o n w a y s to improve the use of prosthetic materials and m u s c l e flaps. H o w e v e r , t h e b e s t m a t e r i a l or method has not yet been identified.
Finally, a more scientific term than giant o m p h a l o c e l e s h o u l d b e u s e d to s p e c i f i c a l l y i d e n t i f y t h i s e n t i t y w h i c h c a n b e so d i f f i c u l t t o t r e a t . We suggest that the term hepatomphalocele implying a "liver-containing" omphalocele be u t i l i z e d b e c a u s e it is t h i s a n o m a l y t h a t so c o m p l i cates the problem of giant omphalocele.
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46. Talbert JL, Rodgers BM, Moazam F: Surgical management of massiveventral hernias in children. J Pediatr Surg 12:63-67~ 1977 47. Moazam F, Rodgers BM, Talbert JL: Use of teflon mesh for repair of abdominal wall defects in neonates. J Pediatr Surg 14:347-351, 1979 48. Croom RD, Thomas CG: Repair of gastroschisis. Surg Gynecol Obstet 132:689-692, 1971 49. Aoyama K: A new operation for repair of large ventral hernias following giant omphalocele and gastroschisis. J Pediatr Surg 14:172-176, 1979 50. Mahout GH: Intact omphalocele: Perennial dilemma of operative or "conservative" management. Am J Surg 128:419 420, 1974 51. Wesselhoeft CW, Randolph JR: Treatment of omphalocele based on individual characteristics of the defect. Pediatrics 44:101-108, 1969 52. Agnew LRC, et al.: Dorland's Illustrated Medical Dictionary (ed 24). Philadelphia, Saunders, 1965, p 668