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Spontaneous postnatal involution of intraabdominal pulmonary sequestration
Spontaneous Postnatal Involution of Intraabdominal Pulmonary Sequestration By M. Chowdhury, M. Samuel, A. Ramsay, J. Constantinou, K. McHugh, and A. Pierro London, England
Prenatal ultrasonography in 2 pregnant women identified a suprarenal echogenic man. The lesions were treated conservatively and involuted with time. These masses were consistent with a suprarenal pulmonary sequestration.
J Pediatr Surg 39:1273-1275. © 2004 Elsevier Inc. All rights reserved.
CASE REPORTS
Routine prenatal US scans have become increasingly popular, resulting in the diagnosis of abdominal lesions that would otherwise have remained undetected. For instance, the increased incidence of antenatally diagnosed renal masses has induced many paediatric surgeons to operate on asymptomatic neonates with renal pelvic dilatations. It has been shown that many of these dilatations can regress spontaneously or be compatible with normal life with no deterioration in renal function.12,13 Antenatal diagnosis of fetal abdominal masses outwith the urinary tract include hepatic cysts, ovarian cysts, teratomas, intestinal duplications, mesenteric cysts, and neuroblastomas. In a recent review of 39 antenatally diagnosed fetal abdominal masses, 15% resolved spontaneously in utero and 21% after birth.14 The differential diagnosis of suprarenal masses in neonates include neuroblastomas, ganglioneuromas, teratomas, and lung sequestrations. The latter is very rare, representing only 2% of all antenatally diagnosed intraabdominal masses.14 However, intraabdominal lung sequestrations have characteristic features at US, CT; and MRI scans. On US, these lesions appear typically more homogenous and hyperechoic relative to the liver, spleen, and kidney.15 It has been suggested that the hyperechogenicity may result from the dilated bronchioles, ducts, and alveoli that may be contained within the sequestrated lobe. However, calcifications are not seen, thus differentiating these lesions from teratomas and neuroblastomas.16 In addition, an arterial-feeding vessel
F
ETAL ULTRASOUND scans performed in 2 mothers at 20 and 22 weeks’ gestation found the presence of echogenic masses (2.6 ⫻ 2.4 ⫻ 1.4 cm and 2.0 ⫻ 2.4 ⫻ 2.4 cm, respectively) in the left suprarenal region of the fetuses. Thereafter, the masses continued to grow in proportion to the fetuses. Prenatal differential diagnoses were either a suprarenal tumor or pulmonary sequestration.
Patient 1: Postnatal History The first child was born at 39 weeks’ gestation. Serum biochemistry, urinary catecholamines, serum ␣-fetoprotein (␣-FP) and -human chorionic gonadotrophin (- HCG) were all normal. Postnatal ultrasound (US) scan delineated a heterogeneous mass in the left suprarenal area with multiple cysts of various sizes. The presence of the mass, measuring 4.0 ⫻ 5.5 ⫻ 3.0 cm, was confirmed by computerized tomography (CT) scan (Fig 1A). CT-guided biopsy established the diagnosis of lung sequestration. In the first 6 months after birth the mass did not change in size while the child remained well and asymptomatic. CT scan at 8 months showed that the mass was smaller, measuring 2.0 ⫻ 2.0 ⫻ 4.0 cm (Fig 1B). Serial US scans thereafter showed progressive regression of the lesion and at 18 months of age complete involution was observed and confirmed by CT scan. Now at 4 years of age the child remains well with no evidence of suprarenal mass on US scan.
Patient 2: Postnatal History The second child was born at 38 weeks’ gestation. Urinary catecholamines, serum biochemistry, ␣-FP and -HCG were normal. US scan on the fifth day of life detected a homogenous 2.1- ⫻ 2.8- ⫻ 2.6-cm left paravertebral mass inferior to the dome of the diaphragm with a feeding vessel communicating from the abdominal aorta to the center of the mass. A magnetic resonance imaging (MRI) scan was consistent with a diagnosis of pulmonary sequestration. Serial US scans showed that the mass had not changed in size during the first 6 months of life but started to regress in size from 9 months of age. At the most recent follow-up (age 18 months) the mass measures 0.5 ⫻ 0.8 ⫻ 0.5 cm while the child continues to remain asymptomatic.
DISCUSSION
We report 2 cases of involuting suprarenal masses, 1 of which was confirmed histologically to be an intraabdominal lung sequestration in origin. Journal of Pediatric Surgery, Vol 39, No 8 (August), 2004: pp 1273-1275
INDEX WORDS: Prenatal ultrasonography, suprarenal pulmonary sequestration, nonoperative management.
From the Department of Paediatric Surgery, Institute of Child Health and Great Ormond Street Hospital for Children, London, England. Address reprint requests to Professor A. Pierro, Department of Paediatric Surgery, Institute of Child Health and Great Ormond Street Hospital for Children, London WC1N 1EH, United Kingdom. © 2004 Elsevier Inc. All rights reserved. 0022-3468/04/3908-0026$30.00/0 doi:10.1016/j.jpedsurg.2004.04.022 1273
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CHOWDHURY ET AL
Fig 1. (A) Computerized tomography scan at 4 days after birth shows the presence of a heterogeneous mass in the left suprarenal region. (B) Computerized tomography scan at 8 months of age shows that the mass in the left suprarenal region has significantly regressed in size.
into the mass is characteristically seen on US and CT scans. Coronal and sagittal MRI scans delineate the adrenal gland from the mass more distinctly than axial CT.17 In 1997 Daneman et al18 reported on 12 children with presumptive diagnosis of intraabdominal lung sequestration. Eleven were treated nonoperatively, and progressive decrease in the size of the masses was observed in all. However, the diagnosis was not based on histology in any of these cases. One child underwent laparotomy after disappearance of the mass, which showed some fibrous tissue only. In child 1 of this report we had confirmed the radiologic diagnosis by percutaneous, CT-guided biopsy. We did not perform a biopsy in the second child because
the radiologic features were identical and highly suggestive of intraabdominal lung sequestration. With respect to involution time of lung sequestrations, Daneman et al19 reported that the mean involution time of the 12 suspected lung sequestrations was 6 months. This contrasts to the comparatively late involution of our cases at 13 and 18 months. Intraabdominal lung sequestrations should be considered in the differential diagnosis of fetal suprarenal masses. These lesions, when isolated, have great potential to regress or resolve spontaneously. Therefore, pediatric surgeons should be aware of the natural history of these lesions in the context of both antenatal counselling and postnatal management.
REFERENCES 1. Samuel M, Burge DM: Extra-lobar intra-abdominal pulmonary sequestration. Eur J Pediatr Surg 6:107-109, 1996 2. Samuel M, Burge DM: Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation. Thorax 54:701-706, 1999 3. Buntain WL, Woolley MM, Mahour GH, et al: Pulmonary sequestration in children: A twenty-five year experience. Surgery 81:413420, 1977 4. Carter R: Pulmonary sequestration. Ann Thorac Surg 7:68-88, 1969 5. DeParedes CG, Pierce WS, Johnson DG, et al: Pulmonary sequestration in infants and children: A 20-year experience and review of the literature. J Pediatr Surg 5:136-147, 1970 6. Leijala M, Louhimo I: Extralobar sequestration of the lung in children. Prog Pediatr Surg 21:98-106, 1987 7. White J, Chan YF, Neuberger S, et al: Prenatal sonographic detection of intra-abdominal extralobar pulmonary sequestration: report of three cases and literature review. Prenat Diagn 14:653-658, 1994 8. White J, Chan YF, Neuberger S, et al: Prenatal sonographic
detection of intra-abdominal extralobar pulmonary sequestration: Report of three cases and literature review. Prenat Diagn 14:653-658, 1994 9. White J, Chan YF, Neuberger S, et al: Prenatal sonographic detection of intra-abdominal extralobar pulmonary sequestration: Report of three cases and literature review. Prenat Diagn 14:653-658, 1994 10. Sargent MA, Liu PC, Smith CR, et al: Infradiaphragmatic pulmonary sequestration. Can Assoc Radiol J 43:208-211, 1992 11. Daneman A, Baunin C, Lobo E, et al: Disappearing suprarenal masses in fetuses and infants. Pediatr Radiol 27:675-681, 1997 12. Ulman I, Jayanthi VR, Koff SA: The long-term followup of newborns with severe unilateral hydronephrosis initially treated nonoperatively. J Urol 164:1101-1105, 2000 13. Dillon HK: Prenatally diagnosed hydronephrosis; selective postnatal intervention. Dialogues in Pediatric Urol 25:5-7, 2002 14. B. Masturzo LCMZLSAP: Fetal abdominal masses: Antenatal and postnatal course. Abstract presented at the annual congress of the Canadian Association of Paediatric Surgeons 2000
SPONTANEOUS INVOLUTION OF PULMONARY SEQUESTRATION
15. Daneman A, Baunin C, Lobo E, et al: Disappearing suprarenal masses in fetuses and infants. Pediatr Radiol 27:675-681, 1997 16. Brink DA, Balsara ZN: Prenatal ultrasound detection of intraabdominal pulmonary sequestration with postnatal MRI correlation. Pediatr Radiol 21:227, 1991 17. Curtis MR, Mooney DP, Vaccaro TJ, et al: Prenatal ultrasound
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characterization of the suprarenal mass: Distinction between neuroblastoma and subdiaphragmatic extralobar pulmonary sequestration. J Ultrasound Med 16:75-83, 1997 18. Daneman A, Baunin C, Lobo E, et al: Disappearing suprarenal masses in fetuses and infants. Pediatr Radiol 27:675-681, 1997 19. Daneman A, Baunin C, Lobo E, et al: Disappearing suprarenal masses in fetuses and infants. Pediatr Radiol 27:675-681, 1997
Prenatal ultrasonography in 2 pregnant women identified a suprarenal echogenic man. The lesions were treated conservatively and involuted with time. These masses were consistent with a suprarenal pulmonary sequestration.
J Pediatr Surg 39:1273-1275. © 2004 Elsevier Inc. All rights reserved.
CASE REPORTS
Routine prenatal US scans have become increasingly popular, resulting in the diagnosis of abdominal lesions that would otherwise have remained undetected. For instance, the increased incidence of antenatally diagnosed renal masses has induced many paediatric surgeons to operate on asymptomatic neonates with renal pelvic dilatations. It has been shown that many of these dilatations can regress spontaneously or be compatible with normal life with no deterioration in renal function.12,13 Antenatal diagnosis of fetal abdominal masses outwith the urinary tract include hepatic cysts, ovarian cysts, teratomas, intestinal duplications, mesenteric cysts, and neuroblastomas. In a recent review of 39 antenatally diagnosed fetal abdominal masses, 15% resolved spontaneously in utero and 21% after birth.14 The differential diagnosis of suprarenal masses in neonates include neuroblastomas, ganglioneuromas, teratomas, and lung sequestrations. The latter is very rare, representing only 2% of all antenatally diagnosed intraabdominal masses.14 However, intraabdominal lung sequestrations have characteristic features at US, CT; and MRI scans. On US, these lesions appear typically more homogenous and hyperechoic relative to the liver, spleen, and kidney.15 It has been suggested that the hyperechogenicity may result from the dilated bronchioles, ducts, and alveoli that may be contained within the sequestrated lobe. However, calcifications are not seen, thus differentiating these lesions from teratomas and neuroblastomas.16 In addition, an arterial-feeding vessel
F
ETAL ULTRASOUND scans performed in 2 mothers at 20 and 22 weeks’ gestation found the presence of echogenic masses (2.6 ⫻ 2.4 ⫻ 1.4 cm and 2.0 ⫻ 2.4 ⫻ 2.4 cm, respectively) in the left suprarenal region of the fetuses. Thereafter, the masses continued to grow in proportion to the fetuses. Prenatal differential diagnoses were either a suprarenal tumor or pulmonary sequestration.
Patient 1: Postnatal History The first child was born at 39 weeks’ gestation. Serum biochemistry, urinary catecholamines, serum ␣-fetoprotein (␣-FP) and -human chorionic gonadotrophin (- HCG) were all normal. Postnatal ultrasound (US) scan delineated a heterogeneous mass in the left suprarenal area with multiple cysts of various sizes. The presence of the mass, measuring 4.0 ⫻ 5.5 ⫻ 3.0 cm, was confirmed by computerized tomography (CT) scan (Fig 1A). CT-guided biopsy established the diagnosis of lung sequestration. In the first 6 months after birth the mass did not change in size while the child remained well and asymptomatic. CT scan at 8 months showed that the mass was smaller, measuring 2.0 ⫻ 2.0 ⫻ 4.0 cm (Fig 1B). Serial US scans thereafter showed progressive regression of the lesion and at 18 months of age complete involution was observed and confirmed by CT scan. Now at 4 years of age the child remains well with no evidence of suprarenal mass on US scan.
Patient 2: Postnatal History The second child was born at 38 weeks’ gestation. Urinary catecholamines, serum biochemistry, ␣-FP and -HCG were normal. US scan on the fifth day of life detected a homogenous 2.1- ⫻ 2.8- ⫻ 2.6-cm left paravertebral mass inferior to the dome of the diaphragm with a feeding vessel communicating from the abdominal aorta to the center of the mass. A magnetic resonance imaging (MRI) scan was consistent with a diagnosis of pulmonary sequestration. Serial US scans showed that the mass had not changed in size during the first 6 months of life but started to regress in size from 9 months of age. At the most recent follow-up (age 18 months) the mass measures 0.5 ⫻ 0.8 ⫻ 0.5 cm while the child continues to remain asymptomatic.
DISCUSSION
We report 2 cases of involuting suprarenal masses, 1 of which was confirmed histologically to be an intraabdominal lung sequestration in origin. Journal of Pediatric Surgery, Vol 39, No 8 (August), 2004: pp 1273-1275
INDEX WORDS: Prenatal ultrasonography, suprarenal pulmonary sequestration, nonoperative management.
From the Department of Paediatric Surgery, Institute of Child Health and Great Ormond Street Hospital for Children, London, England. Address reprint requests to Professor A. Pierro, Department of Paediatric Surgery, Institute of Child Health and Great Ormond Street Hospital for Children, London WC1N 1EH, United Kingdom. © 2004 Elsevier Inc. All rights reserved. 0022-3468/04/3908-0026$30.00/0 doi:10.1016/j.jpedsurg.2004.04.022 1273
1274
CHOWDHURY ET AL
Fig 1. (A) Computerized tomography scan at 4 days after birth shows the presence of a heterogeneous mass in the left suprarenal region. (B) Computerized tomography scan at 8 months of age shows that the mass in the left suprarenal region has significantly regressed in size.
into the mass is characteristically seen on US and CT scans. Coronal and sagittal MRI scans delineate the adrenal gland from the mass more distinctly than axial CT.17 In 1997 Daneman et al18 reported on 12 children with presumptive diagnosis of intraabdominal lung sequestration. Eleven were treated nonoperatively, and progressive decrease in the size of the masses was observed in all. However, the diagnosis was not based on histology in any of these cases. One child underwent laparotomy after disappearance of the mass, which showed some fibrous tissue only. In child 1 of this report we had confirmed the radiologic diagnosis by percutaneous, CT-guided biopsy. We did not perform a biopsy in the second child because
the radiologic features were identical and highly suggestive of intraabdominal lung sequestration. With respect to involution time of lung sequestrations, Daneman et al19 reported that the mean involution time of the 12 suspected lung sequestrations was 6 months. This contrasts to the comparatively late involution of our cases at 13 and 18 months. Intraabdominal lung sequestrations should be considered in the differential diagnosis of fetal suprarenal masses. These lesions, when isolated, have great potential to regress or resolve spontaneously. Therefore, pediatric surgeons should be aware of the natural history of these lesions in the context of both antenatal counselling and postnatal management.
REFERENCES 1. Samuel M, Burge DM: Extra-lobar intra-abdominal pulmonary sequestration. Eur J Pediatr Surg 6:107-109, 1996 2. Samuel M, Burge DM: Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation. Thorax 54:701-706, 1999 3. Buntain WL, Woolley MM, Mahour GH, et al: Pulmonary sequestration in children: A twenty-five year experience. Surgery 81:413420, 1977 4. Carter R: Pulmonary sequestration. Ann Thorac Surg 7:68-88, 1969 5. DeParedes CG, Pierce WS, Johnson DG, et al: Pulmonary sequestration in infants and children: A 20-year experience and review of the literature. J Pediatr Surg 5:136-147, 1970 6. Leijala M, Louhimo I: Extralobar sequestration of the lung in children. Prog Pediatr Surg 21:98-106, 1987 7. White J, Chan YF, Neuberger S, et al: Prenatal sonographic detection of intra-abdominal extralobar pulmonary sequestration: report of three cases and literature review. Prenat Diagn 14:653-658, 1994 8. White J, Chan YF, Neuberger S, et al: Prenatal sonographic
detection of intra-abdominal extralobar pulmonary sequestration: Report of three cases and literature review. Prenat Diagn 14:653-658, 1994 9. White J, Chan YF, Neuberger S, et al: Prenatal sonographic detection of intra-abdominal extralobar pulmonary sequestration: Report of three cases and literature review. Prenat Diagn 14:653-658, 1994 10. Sargent MA, Liu PC, Smith CR, et al: Infradiaphragmatic pulmonary sequestration. Can Assoc Radiol J 43:208-211, 1992 11. Daneman A, Baunin C, Lobo E, et al: Disappearing suprarenal masses in fetuses and infants. Pediatr Radiol 27:675-681, 1997 12. Ulman I, Jayanthi VR, Koff SA: The long-term followup of newborns with severe unilateral hydronephrosis initially treated nonoperatively. J Urol 164:1101-1105, 2000 13. Dillon HK: Prenatally diagnosed hydronephrosis; selective postnatal intervention. Dialogues in Pediatric Urol 25:5-7, 2002 14. B. Masturzo LCMZLSAP: Fetal abdominal masses: Antenatal and postnatal course. Abstract presented at the annual congress of the Canadian Association of Paediatric Surgeons 2000
SPONTANEOUS INVOLUTION OF PULMONARY SEQUESTRATION
15. Daneman A, Baunin C, Lobo E, et al: Disappearing suprarenal masses in fetuses and infants. Pediatr Radiol 27:675-681, 1997 16. Brink DA, Balsara ZN: Prenatal ultrasound detection of intraabdominal pulmonary sequestration with postnatal MRI correlation. Pediatr Radiol 21:227, 1991 17. Curtis MR, Mooney DP, Vaccaro TJ, et al: Prenatal ultrasound
1275
characterization of the suprarenal mass: Distinction between neuroblastoma and subdiaphragmatic extralobar pulmonary sequestration. J Ultrasound Med 16:75-83, 1997 18. Daneman A, Baunin C, Lobo E, et al: Disappearing suprarenal masses in fetuses and infants. Pediatr Radiol 27:675-681, 1997 19. Daneman A, Baunin C, Lobo E, et al: Disappearing suprarenal masses in fetuses and infants. Pediatr Radiol 27:675-681, 1997