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Retroperitoneal alimentary tract duplications detected in utero
Retroperitoneal
Alimentary
Tract Duplications
By Brian W. Duncan, N. Scott Adzick, and Angelo
Detected
In Utero
Eraklis
San Francisco, California and Boston, Massachusetts 0 The prenatal
diagnosis
of numerous
congenital
anoma-
become routine. The prenatal diagnosis of cystic lesions of the retroperitoneum can be due to a variety of renal, gastrointestinal, or adrenal lesions. This finding demands aggressive postnatal follow-up to rule out the possibility of cystic adrenal neuroblastoma. We report the first cases of retroperitoneal cystic masses diagnosed in utero that ultimately proved to be enteric duplications. Therefore, the differential diagnosis of cystic masses of the retroperitoneum found prenatally should be expanded to include enteric duplication cysts. Copyright Q 1992 by W. B. Saunders Company lies
has
INDEX
WORDS:
Enteric
duplication,
prenatal
diagnosis.
A
LIMENTARY tract duplications are rare congenital lesions. Presenting symptoms vary with the location, and a retroperitoneal site is extremely unusual. We report two cases of retroperitoneal alimentary tract duplications diagnosed in utero by routine prenatal ultrasound. CASE REPORTS Case f
The full-term product of an uncomplicated labor and delivery underwent routine screening ultrasound at 16 weeks’ gestation, which showed a cyst in the region of the left adrenal. Repeat ultrasound after delivery confirmed a 3.5 x 2 cm cystic mass contiguous with the medial limb of the left adrenal. Because a cystic neuroblastoma could not be excluded the child was explored during the first week of life. A retroperitoneal approach through a left flank incision was used. The cyst was excised, sparing the left adrenal gland, and the child recovered uneventfully. Again, there was no apparent communication between the cyst and any portion of the gastrointestinal tract or contiguous structures. Pathological examination showed an alimentary tract duplication.
Case
2
An 8-lb 7-0~ male was delivered by elective cesarean section to a healthy mother. The prenatal course was uneventful except for an ultrasound at 17 weeks of gestation that showed an abdominal cyst measurmg 2.5 x 4 cm (Fig 1). At birth a right-sided abdominal mass could be palpated. After delivery, ultrasound and computed tomography scan confirmed the diagnosis of a retroperitoneal cyst of unclear origin. An upper gastrointestinal series showed a widened duodenal C-loop and a HIDA scan was normal. It was elected to observe the patient until a repeat ultrasound at 6 months of age demonstrated the cyst to have increased in size to 8 x 10 cm. Due to the enlargement of the mass, the patient underwent exploratory laparotomy, which showed a retroperitoneal cyst posterior to the head of the pancreas (Fig 2). There was no evidence of communication with the gastrointestinal tract, pancreas, kidney, or adrenal gland. The cyst was completely removed, and the infant recovered uneventfully. Pathological examination showed an alimentary tract duplication cyst with an intestinal mucosal lining (Fig 3). Journaloffediatric
Surgery, Vol 27, No 9 (September),
1992: pp 1231-1233
Fig 1. Prenatal ultrasound showing right kidney (dark arrow) and retroperitoneal duplication cyst (clear arrow) measuring 2.5 x 4 cm.
DISCUSSION
Alimentary tract duplications are unusual congenital lesions. The majority are diagnosed within the first year of life.‘-” Duplications may arise at any location throughout the gastrointestinal tract, with the ileum being the most common site of occurrence. A retroperitoneal location for duplications is very unusual. In a series of 78 cases of alimentary tract duplication, only 2 were located in a retroperitoneal site.’ Approximately 20% of alimentary tract duplications are multiple. Associated congenital lesions occur in 20% of affected infants.1-3 Esophageal lesions may present with respiratory distress, hematemesis, or as an asymptomatic mass found on chest x-ray. Intraabdominal duplications can present as small bowel obstruction, gastrointestinal bleeding, or a palpable mass. Alimentary tract duplications should be completely excised whenever possible. If extensive lesions can not be safely removed without endangering nearby structures, the cyst mucosa should be stripped to prevent bleeding or perforation because ectopic gastric mucosa may be present. Duplications are cystic or
From the Department of Surgery, lJniversi& of California. San Francisco, CA, und the Depurtme~tt of StirgeT, Bostorr Children’s Hospital. Boston, MA. Address reprint requests to N. Scott Adzirk, MD, Univer& of California. San Francisco, 3rd and Pamassus Apses, 585-HSE, San Franci.yco. CA 94143. Copyright VL1992 by W!B. Saunders Cornpanv 0022-3468!92/2709-0017$03.0010 1231
1232
DUNCAN, ADZICK, AND ERAKLIS
Fig 3. Photomicrograph demonstrating intestinal mucosa lining of excised alimentary tract duplication cyst (H&E, original magnification x40).
Fig 2. Intraoperatfve appearance of retroperitoneal alimentary tract duplication. gb, gallbladder; a, appendix; sb, small bowel; C, colon; and M, mass, retroperitoneal alimentary tract duplication.
tubular and demonstrate a true wall with gastrointestinal mucosa, muscularis, and serosa. The prenatal diagnosis of cystic lesions of the retroperitoneum has been previously reported. The differential diagnosis includes adrenal, renal, and gastrointestinal cystic masses. Foremost among these is cystic neuroblastoma.5 The inability to exclude this lesion demands close follow-up postnatally with excision of the mass soon after birth. Other cystic retroperitoneal lesions which can be diagnosed in utero include simple adrenal cysts,6 hydronephrosis,7 ureteral duplication8 mesoblastic nephroma,9 and choledochal cyst.‘O
Prenatal ultrasound has become increasingly accurate in the diagnosis of congenital lesions as experience has been gained and resolution of present equipment has improved. 11~12 Although a variety of other cystic lesions of the retroperitoneum have been diagnosed in utero, these patients are the first cases of prenatally diagnosed retroperitoneal cystic masses that proved to be alimentary tract duplications. Therefore, cystic alimentary tract duplication should enter into the differential diagnosis of any retroperitoneal cyst diagnosed prenatally. The accurate in utero diagnosis of such lesions allows for their natural history to be followed before and after delivery. With affected children identified, postnatal follow-up can occur leading to the appropriate treatment. ACKNOWLEDGMENT The authors would like to thank Dr Marshall Carpenter providing copies of the prenatal ultrasound in case 1.
for
REFERENCES 1. Gross RE, Holcomb GW, Farber S: Duplications alimentary tract. Pediatrics 9:449-467,1952
of the
2. Bower RJ, Sieber WK, Kieswetter WB: Alimentary tract duplications in children. Ann Surg 188:669-674,1978 3. Ildstad ST, Tollerud DJ, Weiss RG, et al: Duplications of the alimentary tract. Ann Surg 208:184-189,1988 4. Holcomb
GW, Gheissari
A, O’Neill JA, et al: Surgical
management of alimentary tract duplications. Ann Surg 209:167174,1989 5. Giulian BB, Chang CCN, Yoss BS: Prenatal diagnosis of fetal adrenal neuroblastoma. J Clin Ultrasound 14:225-227,1986 6. Zivkovic SM, Jancic-Zguricas M, Jakonovic R, et al: Adrenal cysts in the newborn. J Uroi 129:1295-1031,1983 7. Flake AW, Harrison MR, Sauer L, et al: Ureteropelvic junction obstruction in the fetus. J Pediatr Surg 21:1058-1063,1986
RETROPERITONEAL
ALIMENTARY
TRACT DUPLICATIONS
8. Madarikan BA, Hayward C, Roberts GM, et al: Clinical outcome of fetal uropathy. Arch Dis Child 63:961-963,1988 9. Giulian BB: Prenatal ultrasonographic diagnosis of fetal renal tumors. Radiology 152:69-70,1984 10. O’Neill JA, Templeton JM, Schnaufer L, et al: Recent experience with choledochal cyst. Ann Surg 205:533-540, 1987
1233
11. Vintzileos AM, Campbell WA, Nocbimson DJ, et al: Antenatal evaluation and management of ultrasonically detected fetal anomalies. Obstet Gynecol69:640-660,1987 12. Hill MC, Lande IM, Larsen JW: Prenatal diagnosis of fetal anomalies using ultrasound and MRI. Radio1 Clin North Am 26:287-307, 1988
Alimentary
Tract Duplications
By Brian W. Duncan, N. Scott Adzick, and Angelo
Detected
In Utero
Eraklis
San Francisco, California and Boston, Massachusetts 0 The prenatal
diagnosis
of numerous
congenital
anoma-
become routine. The prenatal diagnosis of cystic lesions of the retroperitoneum can be due to a variety of renal, gastrointestinal, or adrenal lesions. This finding demands aggressive postnatal follow-up to rule out the possibility of cystic adrenal neuroblastoma. We report the first cases of retroperitoneal cystic masses diagnosed in utero that ultimately proved to be enteric duplications. Therefore, the differential diagnosis of cystic masses of the retroperitoneum found prenatally should be expanded to include enteric duplication cysts. Copyright Q 1992 by W. B. Saunders Company lies
has
INDEX
WORDS:
Enteric
duplication,
prenatal
diagnosis.
A
LIMENTARY tract duplications are rare congenital lesions. Presenting symptoms vary with the location, and a retroperitoneal site is extremely unusual. We report two cases of retroperitoneal alimentary tract duplications diagnosed in utero by routine prenatal ultrasound. CASE REPORTS Case f
The full-term product of an uncomplicated labor and delivery underwent routine screening ultrasound at 16 weeks’ gestation, which showed a cyst in the region of the left adrenal. Repeat ultrasound after delivery confirmed a 3.5 x 2 cm cystic mass contiguous with the medial limb of the left adrenal. Because a cystic neuroblastoma could not be excluded the child was explored during the first week of life. A retroperitoneal approach through a left flank incision was used. The cyst was excised, sparing the left adrenal gland, and the child recovered uneventfully. Again, there was no apparent communication between the cyst and any portion of the gastrointestinal tract or contiguous structures. Pathological examination showed an alimentary tract duplication.
Case
2
An 8-lb 7-0~ male was delivered by elective cesarean section to a healthy mother. The prenatal course was uneventful except for an ultrasound at 17 weeks of gestation that showed an abdominal cyst measurmg 2.5 x 4 cm (Fig 1). At birth a right-sided abdominal mass could be palpated. After delivery, ultrasound and computed tomography scan confirmed the diagnosis of a retroperitoneal cyst of unclear origin. An upper gastrointestinal series showed a widened duodenal C-loop and a HIDA scan was normal. It was elected to observe the patient until a repeat ultrasound at 6 months of age demonstrated the cyst to have increased in size to 8 x 10 cm. Due to the enlargement of the mass, the patient underwent exploratory laparotomy, which showed a retroperitoneal cyst posterior to the head of the pancreas (Fig 2). There was no evidence of communication with the gastrointestinal tract, pancreas, kidney, or adrenal gland. The cyst was completely removed, and the infant recovered uneventfully. Pathological examination showed an alimentary tract duplication cyst with an intestinal mucosal lining (Fig 3). Journaloffediatric
Surgery, Vol 27, No 9 (September),
1992: pp 1231-1233
Fig 1. Prenatal ultrasound showing right kidney (dark arrow) and retroperitoneal duplication cyst (clear arrow) measuring 2.5 x 4 cm.
DISCUSSION
Alimentary tract duplications are unusual congenital lesions. The majority are diagnosed within the first year of life.‘-” Duplications may arise at any location throughout the gastrointestinal tract, with the ileum being the most common site of occurrence. A retroperitoneal location for duplications is very unusual. In a series of 78 cases of alimentary tract duplication, only 2 were located in a retroperitoneal site.’ Approximately 20% of alimentary tract duplications are multiple. Associated congenital lesions occur in 20% of affected infants.1-3 Esophageal lesions may present with respiratory distress, hematemesis, or as an asymptomatic mass found on chest x-ray. Intraabdominal duplications can present as small bowel obstruction, gastrointestinal bleeding, or a palpable mass. Alimentary tract duplications should be completely excised whenever possible. If extensive lesions can not be safely removed without endangering nearby structures, the cyst mucosa should be stripped to prevent bleeding or perforation because ectopic gastric mucosa may be present. Duplications are cystic or
From the Department of Surgery, lJniversi& of California. San Francisco, CA, und the Depurtme~tt of StirgeT, Bostorr Children’s Hospital. Boston, MA. Address reprint requests to N. Scott Adzirk, MD, Univer& of California. San Francisco, 3rd and Pamassus Apses, 585-HSE, San Franci.yco. CA 94143. Copyright VL1992 by W!B. Saunders Cornpanv 0022-3468!92/2709-0017$03.0010 1231
1232
DUNCAN, ADZICK, AND ERAKLIS
Fig 3. Photomicrograph demonstrating intestinal mucosa lining of excised alimentary tract duplication cyst (H&E, original magnification x40).
Fig 2. Intraoperatfve appearance of retroperitoneal alimentary tract duplication. gb, gallbladder; a, appendix; sb, small bowel; C, colon; and M, mass, retroperitoneal alimentary tract duplication.
tubular and demonstrate a true wall with gastrointestinal mucosa, muscularis, and serosa. The prenatal diagnosis of cystic lesions of the retroperitoneum has been previously reported. The differential diagnosis includes adrenal, renal, and gastrointestinal cystic masses. Foremost among these is cystic neuroblastoma.5 The inability to exclude this lesion demands close follow-up postnatally with excision of the mass soon after birth. Other cystic retroperitoneal lesions which can be diagnosed in utero include simple adrenal cysts,6 hydronephrosis,7 ureteral duplication8 mesoblastic nephroma,9 and choledochal cyst.‘O
Prenatal ultrasound has become increasingly accurate in the diagnosis of congenital lesions as experience has been gained and resolution of present equipment has improved. 11~12 Although a variety of other cystic lesions of the retroperitoneum have been diagnosed in utero, these patients are the first cases of prenatally diagnosed retroperitoneal cystic masses that proved to be alimentary tract duplications. Therefore, cystic alimentary tract duplication should enter into the differential diagnosis of any retroperitoneal cyst diagnosed prenatally. The accurate in utero diagnosis of such lesions allows for their natural history to be followed before and after delivery. With affected children identified, postnatal follow-up can occur leading to the appropriate treatment. ACKNOWLEDGMENT The authors would like to thank Dr Marshall Carpenter providing copies of the prenatal ultrasound in case 1.
for
REFERENCES 1. Gross RE, Holcomb GW, Farber S: Duplications alimentary tract. Pediatrics 9:449-467,1952
of the
2. Bower RJ, Sieber WK, Kieswetter WB: Alimentary tract duplications in children. Ann Surg 188:669-674,1978 3. Ildstad ST, Tollerud DJ, Weiss RG, et al: Duplications of the alimentary tract. Ann Surg 208:184-189,1988 4. Holcomb
GW, Gheissari
A, O’Neill JA, et al: Surgical
management of alimentary tract duplications. Ann Surg 209:167174,1989 5. Giulian BB, Chang CCN, Yoss BS: Prenatal diagnosis of fetal adrenal neuroblastoma. J Clin Ultrasound 14:225-227,1986 6. Zivkovic SM, Jancic-Zguricas M, Jakonovic R, et al: Adrenal cysts in the newborn. J Uroi 129:1295-1031,1983 7. Flake AW, Harrison MR, Sauer L, et al: Ureteropelvic junction obstruction in the fetus. J Pediatr Surg 21:1058-1063,1986
RETROPERITONEAL
ALIMENTARY
TRACT DUPLICATIONS
8. Madarikan BA, Hayward C, Roberts GM, et al: Clinical outcome of fetal uropathy. Arch Dis Child 63:961-963,1988 9. Giulian BB: Prenatal ultrasonographic diagnosis of fetal renal tumors. Radiology 152:69-70,1984 10. O’Neill JA, Templeton JM, Schnaufer L, et al: Recent experience with choledochal cyst. Ann Surg 205:533-540, 1987
1233
11. Vintzileos AM, Campbell WA, Nocbimson DJ, et al: Antenatal evaluation and management of ultrasonically detected fetal anomalies. Obstet Gynecol69:640-660,1987 12. Hill MC, Lande IM, Larsen JW: Prenatal diagnosis of fetal anomalies using ultrasound and MRI. Radio1 Clin North Am 26:287-307, 1988