Prenatal ultrasonographic findings of intra-abdominal cystic lymphangioma: A case report
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Case Report Prenatal Ultrasonographic Findings of Intra-Abdominal Cystic Lymphangioma: A Case Report Roque Devesa, MD, PhD, Ana Mun ˜ oz, MD, Margarita Torrents, MD, Jose ´ M. Carrera, MD, PhD Division of Prenatal Diagnosis, Department of Obstetrics and Gynecology, Instituto Universitario Dexeus, Paseo Bonanova, No 67, 08017 Barcelona, Spain. Received 6 February 1996; accepted 5 July 1996 Lymphangiomas are benign tumors of the lym- phatic vessels that are most commonly diagnosed in the neonatal period or early infancy. They are characterized by the appearance of a uni- or multi-septate cystic mass, most frequently local- ized in the neck or axilla, but can also be found in other areas (eg, face, tongue, abdomen, mediasti- num). Although these lesions are benign, they can create a mass effect and compress adjacent vital organs, which, in turn, determines the severity of the lesion. We present the prenatal sonographic images of an abdominal cystic lymphangioma. CASE REPORT The patient was a 29-year-old primiparous woman with a history of hepatitis A in childhood and an appendectomy; her gynecologic history was unremarkable. The patient was followed in our center from the beginning of her pregnancy with normal ultra- sound examinations at 8 and 20 weeks, menstrual age (MA). At 24 weeks, MA, a male fetus was observed with biometric measurements consis- tent with MA in addition to the appearance of an anechogenic image behind the abdominal wall. The unilocular mass measured 5.5 cm × 4.2 cm × 3.3 cm, was well delineated, and located in the infraumbilical and prevesical area (Figures 1 and 2). There was no evidence of other associated anomalies, abdominal compression, or urinary ob- struction. The intestinal images appeared normal as did the amniotic fluid volume. Subsequent serial ultrasound examinations showed no change in the described image until 32 weeks, MA, at which time, without an increase in size, fine septae were noted in the interior of the mass. By 39 weeks, MA, the mass had increased in size to 6.8 cm × 5.0 cm × 3.7 cm and had lat- eralized to the left, thus displacing loops of bowel. Elective cesarean delivery was discussed and ruled out by our perinatology committee because the abdominal circumference was normal. At 40 weeks, MA, the patient went into spon- taneous labor and was delivered by cesarean sec- tion for fetal distress and cervical dystocia. A male infant was delivered weighing 4070 g, with Apgar scores of 9 and 10 at 1 and 5 minutes, re- spectively. The postpartum course was normal. The neonatal examination was significant for an abdominal mass in the hypogastric region with a soft abdomen and without evidence of peritone- al irritation. The neonatal ultrasound studies confirmed the prenatal findings that suggested an abdominal lymphangioma. At 2 months after delivery, a CT scan was performed that noted an increase in the size of the tumor, in addition to a mass effect over the bladder and intestine. A py- elogram confirmed the presence of extrinsic com- pression over the anterior aspect of the bladder. Given the evolution of this lesion, a decision was made for surgical intervention. A laparotomy was performed with extension of the vertical in- cision to the supraumbilical region. A multicystic mass containing a yellow serous fluid was noted Correspondence to: R. Devesa J Clin Ultrasound 25:330–332, 1997 © 1997 John Wiley & Sons, Inc. CCC 0091-2751/97/060330-03 330 JOURNAL OF CLINICAL ULTRASOUND
Transcript of Prenatal ultrasonographic findings of intra-abdominal cystic lymphangioma: A case report
Case Report
Prenatal Ultrasonographic Findings ofIntra-Abdominal Cystic Lymphangioma:
A Case Report
Roque Devesa, MD, PhD, Ana Munoz, MD, Margarita Torrents, MD,Jose M. Carrera, MD, PhD
Division of Prenatal Diagnosis, Department of Obstetrics and Gynecology, InstitutoUniversitario Dexeus, Paseo Bonanova, No 67, 08017 Barcelona, Spain.
Received 6 February 1996; accepted 5 July 1996
Lymphangiomas are benign tumors of the lym-phatic vessels that are most commonly diagnosedin the neonatal period or early infancy. They arecharacterized by the appearance of a uni- ormulti-septate cystic mass, most frequently local-ized in the neck or axilla, but can also be found inother areas (eg, face, tongue, abdomen, mediasti-num). Although these lesions are benign, they cancreate a mass effect and compress adjacent vitalorgans, which, in turn, determines the severity ofthe lesion.
We present the prenatal sonographic images ofan abdominal cystic lymphangioma.
CASE REPORT
The patient was a 29-year-old primiparouswoman with a history of hepatitis A in childhoodand an appendectomy; her gynecologic historywas unremarkable.
The patient was followed in our center from thebeginning of her pregnancy with normal ultra-sound examinations at 8 and 20 weeks, menstrualage (MA). At 24 weeks, MA, a male fetus wasobserved with biometric measurements consis-tent with MA in addition to the appearance of ananechogenic image behind the abdominal wall.The unilocular mass measured 5.5 cm × 4.2 cm ×3.3 cm, was well delineated, and located in theinfraumbilical and prevesical area (Figures 1 and2). There was no evidence of other associated
anomalies, abdominal compression, or urinary ob-struction. The intestinal images appeared normalas did the amniotic fluid volume.
Subsequent serial ultrasound examinationsshowed no change in the described image until 32weeks, MA, at which time, without an increase insize, fine septae were noted in the interior of themass. By 39 weeks, MA, the mass had increasedin size to 6.8 cm × 5.0 cm × 3.7 cm and had lat-eralized to the left, thus displacing loops of bowel.Elective cesarean delivery was discussed andruled out by our perinatology committee becausethe abdominal circumference was normal.
At 40 weeks, MA, the patient went into spon-taneous labor and was delivered by cesarean sec-tion for fetal distress and cervical dystocia. Amale infant was delivered weighing 4070 g, withApgar scores of 9 and 10 at 1 and 5 minutes, re-spectively. The postpartum course was normal.
The neonatal examination was significant foran abdominal mass in the hypogastric region witha soft abdomen and without evidence of peritone-al irritation. The neonatal ultrasound studiesconfirmed the prenatal findings that suggestedan abdominal lymphangioma. At 2 months afterdelivery, a CT scan was performed that noted anincrease in the size of the tumor, in addition to amass effect over the bladder and intestine. A py-elogram confirmed the presence of extrinsic com-pression over the anterior aspect of the bladder.
Given the evolution of this lesion, a decisionwas made for surgical intervention. A laparotomywas performed with extension of the vertical in-cision to the supraumbilical region. A multicysticmass containing a yellow serous fluid was noted
Correspondence to: R. DevesaJ Clin Ultrasound 25:330–332, 1997
© 1997 John Wiley & Sons, Inc. CCC 0091-2751/97/060330-03
330 JOURNAL OF CLINICAL ULTRASOUND
in the prevesical space and extending superiorly.The mass was completely resected. The pathologyreport confirmed the diagnosis of a cystic lymph-angioma (Figure 3). The postoperative course wasunremarkable. Presently, the infant is 18 monthsold and is asymptomatic without evidence of re-currence.
DISCUSSION
Lymphangiomas are lesions of the lymphatic ves-sels, and like hemangiomas, it is difficult to as-sess whether they are true tumors (ie, hamarto-mas) or lymphangiectasias.1 Given the benign
nature of these lesions, such a differentiation is oflittle practical clinical value.
The most common location for these tumors isthe neck (cystic hygroma) accounting for 75% ofcases,2,3 followed by the axilla in 20% of cases,with the remaining 5% occurring in other regions,such as the face, tongue, abdomen, mediastinum,abdominal viscera, or bone.4
Cystic hygroma colli probably results from afailure of the jugular lymphatics to drain into theinternal jugular vein and subsequent dilatation ofthe lymphatic sacs5; the neck location has beenassociated with chromosomal anomalies, princi-pally Turner’s syndrome,2,3 but not the other lo-cations.
In general, it is estimated that 50%–65% oflymphangiomas are present at birth, with 90% ofthem manifesting before the second year of life.6
Abdominal lymphangiomas are a known patho-logic process in the postnatal period and in adults,often with long asymptomatic periods.7–9 Never-theless, case reports of prenatal diagnosis of ab-dominal lymphangiomas or hemangiolymphomasare scarce.10–13
Abdominal lymphangiomas are very rare tu-mors14 that present before 5 years of age in 60% ofcases and often not until the adult years.
The ultrasound image is that of a hypoecho-genic cystic mass (with different levels of attenu-ation depending on the nature of the fluid con-tained within, serous or chylous), of varying size,well delineated, uni- or multi-loculated, and withfine septations. The lymphangioma occasionallyproduces intracystic hemorrhage that can modifythe appearance.
The location of the lesion is of important diag-
FIGURE 1. Fetal sonography (24 weeks, MA, low transverse abdomi-nal section); an anechogenic image behind the abdominal wall in theprevesical space (large arrow) is seen. Color Doppler identified twointra-abdominal umbilical arteries (small arrows) that surrounded thebladder.
FIGURE 2. Fetal sonography (32 weeks, MA, modified low transverseabdominal section); the appearance of fine septations (arrow) in themass is described in Figure 1.
FIGURE 3. Cystic lesion limited by fibromuscular wall and internallylined with flattened cells (×250).
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nostic value; however, localization can be difficultif it is unusual, as in this case.
In the prenatal period, a differential diagnosismust be made with other cystic intra-abdominaland intestinal anomalies (intestinal obstructions,enteric cysts) as well as non-intestinal anomalies(ovarian cysts, hydrometrocolpos, obstructiveuropathy, anorectal atresia, persistent cloaca,urachal cysts, mesenteric or retroperitonealcysts),3 and other rare postnatal tumors, such asa cystic mesothelioma or microcystic adenoma ofthe pancreas.
We managed our case expectantly in the pre-natal period with serial ultrasound examinationsgiven the lack of complications and an exact di-agnosis. At no time was there any evidence of fe-tal compromise that would have necessitatedearly delivery. The prenatal diagnosis was impor-tant because it allowed for very close observationof the newborn before the occurrence of complica-tions, such as intestinal or urinary obstruction, avalvulus, or intestinal infarct.
The treatment of these lesions is surgical; inour case we intervened after we had allowed foradequate weight gain and after the first signs ofcompression. The prognosis is good althoughthese tumors can recur.7 Long-term follow-up isnecessary after the primary intervention.
REFERENCES
1. Enzinger FM, Weiss SW, eds: Tumors of lymphvessels. In Soft Tissue Tumors. St. Louis, Mosby–Year Book, 1995, pp. 679–700.
2. Romero R, Pilu G, Jeanty P, Ghidini A, HobbinsJC, eds: The Neck. In Prenatal Diagnosis of Con-
genital Anomalies. Norwalk, CT, Appleton &Lange, 1988, pp. 113–123.
3. Nyberg DA: Intra-abdominal abnormalities. In Ny-berg DA, Mahony BS, Pretorius DH, eds: Diagnos-tic Ultrasound of Fetal Anomalies. St. Louis, Mos-by–Year Book, 1990, pp. 342–394.
4. Singhs S, Baboo ML, Pathak LC: Cystic lymphan-gioma in children: report of 32 cases including le-sions at rare sites. Surgery 69:947–951, 1971.
5. Chervenak FA, Isaacson G, Blakemore KJ, et al:Fetal cystic hygroma: cause and natural history. NEngl J Med 308:822–825, 1983.
6. Bill AH, Summer DS: A unified concept of lymph-angioma and cystic hygroma. Surg Gynecol Obstet120:79–84, 1965.
7. Heether J, Whalen T, Doollin E: Follow-up of com-plex unresectable lymphangiomas. Am Surg 60:840–841, 1994.
8. Schmidt M: Intra-abdominal lymphangioma. KansMed 93:149–150, 1992.
9. Ruess L, Frazier AA, Sivit CJ: CT of the mesentery,omentum and peritoneum in children. Radio-graphics 15:89–104, 1995.
10. Shah KD, Chervenak FA, Marchevsky AM, et al:Fetal giant hemangiolymphangioma: report of acase. Am J Perinatol 4:212–214, 1987.
11. Kozlowski KJ, Frazier CN, Quirk JG: Prenatal di-agnosis of abdominal cystic hygroma. Prenat Diagn8:405–409, 1988.
12. Giacalone PL, Boulot P, Deschamps P, et al: Pre-natal diagnosis of a multifocal lymphangioma. Pre-nat Diagn 8:1133–1137, 1993.
13. Giacalone PL, Boulot P, Marty M, et al: Fetalhemangiolymphangioma: a case report. Fetal Di-agn Ther 8:338–340, 1993.
14. Galifer RB, Pous JG, Juskiewenski S, et al: Intra-abdominal cystic lymphangioma in childhood. Pre-nat Pediatr Surg 11:173–176, 1978.
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