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Congenital subcostal hernia with unusual contents
Journal of Pediatric Surgery (2010) 45, 435–437
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Congenital subcostal hernia with unusual contents Julie Monteagudo, Kimberly A. Ruscher, Elizabeth Margolis, Fabiola Balarezo, Christine M. Finck ⁎ Department of Pediatric Surgery, Connecticut Children's Medical Center, Hartford, CT, USA Received 28 September 2009; revised 8 December 2009; accepted 10 December 2009
Key words: Subcostal hernia; Fallopian tube; Ovary; Müllerian remnants
Abstract Congenital hernias arising in the subcostal region are rare. We describe a case of a former preterm infant, born with congenitally fused right 11th and 12th ribs and a protuberant mass in the right subcostal region. This mass was associated with a small fascial defect and herniation of abdominal contents. At operation, the mass was determined to be a hernia with an incarcerated ovarian remnant and fallopian tube. © 2010 Elsevier Inc. All rights reserved.
1. Case description A 15-month-old female infant presented for surgical evaluation of a protuberant subcostal mass. She was a former 27-week premature infant, with a medical history significant for bronchopulmonary dysplasia, grade III intraventricular hemorrhage, a stable conoventricular ventricular septal defect, and congenital fusion of the 11th and 12th ribs on her right side. She was born to a gravida 2 para 0-1 abortus 1 mother who received fertility treatments and intrauterine insemination. Her prenatal course was normal, with normal serum screening and a normal karyotype by chorionic villus sampling. At 6 weeks of age, a protuberant mass was noted on her right subcostal area. The mass was soft and did not increase with crying or straining and slowly enlarged with time. Magnetic resonance imaging (MRI) demonstrated a partially
⁎ Corresponding author. Tel.: +1 860 545 9520; fax: +1 860 545 9545. E-mail address: [email protected] (C.M. Finck). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.12.008
cystic, partially solid mass, consistent with a possible lymphangiomatous malformation versus herniated bowel (Fig. 1). The patient was noted to have normal intraabdominal viscera including 2 retroperitoneal kidneys on the MRI. On physical examination, the consulting surgeon noted a 2centimeter fascial defect, with a soft overlying hernia; contents were easily reducible. The overlying skin was thinned and hypopigmented, consistent with aplasia cutis congenita. Surgical exploration of the mass revealed a hernia sac containing a small cystic mass and a cord-like structure that coursed into the pelvis toward the uterus (Fig. 2). A laparoscope was used to visualize these structures through an umbilical incision. No normal ovary could be identified in the right pelvis; a normal ovary was identified on the left. The herniated cord-like structure and adjacent fibrous tissue were resected and sent to pathology. The fascial defect was repaired using 2-0 PDS (polydioxanone) in an interrupted fashion, and the subcutaneous layer and skin were closed separately. The patient did well postoperatively and was sent home after 1 day. The pathology was consistent with müllerian-type remnants comprising ducts lined by immature embryonic type epithelium embedded in fibroconnective
436
Fig. 1 Magnetic resonance imaging demonstrated a complex soft tissue mass in the region of the subcostal defect.
tissue and adjacent fragments of ovarian tissue (Fig. 3). The patient has done extremely well in follow-up.
2. Discussion Although there has been a previous report of a subcostal hernia secondary to traumatic abdominal wall weakness [1], only one previous report of a congenital subcostal hernia is cited in the literature. Nicksa et al [2] described a patient with a 2-cm left-sided fascial defect, associated with abnormalities in the costal cartilages, intermittent herniation of the stomach, and ipsilateral renal agenesis. Similarities to our case include that both mothers received fertility treatments, and in both cases, abnormalities of the thoracic cage were present. Neither patient has a known genetic syndrome. The comorbid congenital abnormalities in the 2 patients are quite different. Our patient had a ventricular septal defect, congenitally fused ribs, with aplasia cutis congenita diagnosed by a dermatologist in the area overlying the hernia. Normal development of the musculature in this area was present, and both kidneys were identified in the MRI. The most significant aspect of this subcostal hernia was the unusual contents of the hernia sac. Inguinal hernias have been reported to contain both congenitally abnormal and normal müllerian structures [3-5]. Ovaries have been reported in lumbar hernias [6]. A recent review article by Barnett et al [7] described the associations between several genetic syndromes and increased incidence of inguinal hernias. Our patient does not have a known genetic disorder; however, she does have a constellation of abnormal physical findings. Given her local anomalies—aplasia cutis conge-
J. Monteagudo et al. nita, congenitally fused 11th and 12th ribs, failure of descent of the ovary, and the presence of the hernia—there is a possibility that failure of differentiation of the ectoderm and mesoderm in this area may have contributed to poor migration of müllerian tissue and a possible weakness of the tissues in the subcostal area. Aplasia cutis congenita is characterized by a focal absence of skin at birth. Most cases, approximately 90%, occur on the scalp, although in rare cases, it has occurred on the trunk. It is suggested that these cases may be caused by vascular events or ischemia, viral infections, or adherence of the amniotic sac during development causing skin trauma [8]. Several cases of fetal papyraceous associated with truncal aplasia cutis congenita have been reported [9]. Although this was not a known feature of the case we present, multiple gestational pregnancies are more common in women receiving fertility treatments, and this may be a risk factor for aplasia cutis congenita. Molecular research suggests failure of the ectoderm to develop adequately in that area may lead to underlying embryologic defects as a result of disrupted inductive interactions between these layers, which occur during normal development [10]. Normally, the gonads develop as a pair of genital ridges formed by proliferation of the epithelium and underlying mesenchyme. During the sixth week of development, germ cells appear among the endodermal cells in the wall of the yolk sac. These germ cells migrate by ameobic movements to the primitive gonads. Once they arrive, they induce the development of the gonad into the testis or the ovary. If they fail to reach this location, the gonads do not develop [11,12]. In our case, it is possible that the descent was interrupted as the developing tissue lacked the appropriate signaling from the overlying ectoderm. It is important to recognize that weakness in the tissues as a result of aplasia cutis congenita may predispose the infant to a hernia in that area potentially involving tissue, which has undergone abnormal development. Operative management should include consideration
Fig. 2 Contents of the hernia sac found at operative exploration; arrow indicates atretic, dilated cord, and müllerian remnants.
Congenital subcostal hernia with unusual contents
437
Fig. 3 Pathology: A, Müllerian-type remnants comprising ducts lined by immature embryonic type epithelium (arrow) in hernia sac contents (H&E, ×200). B, Ovarian tissue with fibrosis and cystic follicle (arrow; H&E, ×100). C, Fimbria of fallopian tube (arrow; H&E, ×100).
of unusual contents of the hernia sac in an area of aplasia cutis congenita.
References [1] Polk HC, Newton WT. Abdominal parietal hernias at the costal margin. Annals of Surgery 1963;158(6):1047-52. [2] Nicksa GA, Christensen EP, Buckmiller TP. A case report of a congenital left subcostal hernia in a neonate. J Pediatr Surg 2009;44: 1653-5. [3] Kamio M, Nagata T, Yamasaki H, et al. Inguinal hernia containing functioning, rudimentary uterine horn and endometriosis. Obstet Gynecol 2009;113:563-6. [4] Gurer A, Ozdogan M, Ozlem N, et al. Uncommon content in groin hernia sac. Hernia 2006;10:152-5.
[5] Bradshaw KD. Ovarian and tubal inguinal hernia. Obstet Gynecol 1986;68(3 Suppl):50S-2S. [6] Geis WP, Hodakowski GT. Lumbar hernia. In: Nyhus LM, Condon RE, editors. Hernia. 4th ed. Philadelphia (PA): J.B. Lippincott Company; 1995. p. 412-24. [7] Barnett C, Langer JC, Hinek A, et al. Looking past the lump: genetic aspects of inguinal hernia in children. J Pediatr Surg 2009;44:1423-31. [8] Fagan LL, Harris PA, Coran AG, et al. Sporadic aplasia cutis congenita. Pediatr Surg Int 2002;18(5-6):545-7. [9] Maccario S, Fasolato V, Brunelli A, et al. Aplasia cutis congenita: an association with vanishing twin syndrome. Eur J Dermatol 2009;19(4): 372-4. [10] Priolo M, Silengo M, Lerone M, et al. Ectodermal dysplasias: not only ‘skin’ deep. Clin Genet 2000;58(6):415-30. [11] Sadler TW. Medical embryology. 10th ed. Philadelphia (PA): Lippincott Williams & Wilkins; 2006. p. 242-56. [12] Skandalakis JE, Gray SW. Embryology for surgeons. 2nd ed. Baltimore (MD): Williams & Wilkins; 1994. p. 736-69.
www.elsevier.com/locate/jpedsurg
Congenital subcostal hernia with unusual contents Julie Monteagudo, Kimberly A. Ruscher, Elizabeth Margolis, Fabiola Balarezo, Christine M. Finck ⁎ Department of Pediatric Surgery, Connecticut Children's Medical Center, Hartford, CT, USA Received 28 September 2009; revised 8 December 2009; accepted 10 December 2009
Key words: Subcostal hernia; Fallopian tube; Ovary; Müllerian remnants
Abstract Congenital hernias arising in the subcostal region are rare. We describe a case of a former preterm infant, born with congenitally fused right 11th and 12th ribs and a protuberant mass in the right subcostal region. This mass was associated with a small fascial defect and herniation of abdominal contents. At operation, the mass was determined to be a hernia with an incarcerated ovarian remnant and fallopian tube. © 2010 Elsevier Inc. All rights reserved.
1. Case description A 15-month-old female infant presented for surgical evaluation of a protuberant subcostal mass. She was a former 27-week premature infant, with a medical history significant for bronchopulmonary dysplasia, grade III intraventricular hemorrhage, a stable conoventricular ventricular septal defect, and congenital fusion of the 11th and 12th ribs on her right side. She was born to a gravida 2 para 0-1 abortus 1 mother who received fertility treatments and intrauterine insemination. Her prenatal course was normal, with normal serum screening and a normal karyotype by chorionic villus sampling. At 6 weeks of age, a protuberant mass was noted on her right subcostal area. The mass was soft and did not increase with crying or straining and slowly enlarged with time. Magnetic resonance imaging (MRI) demonstrated a partially
⁎ Corresponding author. Tel.: +1 860 545 9520; fax: +1 860 545 9545. E-mail address: [email protected] (C.M. Finck). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.12.008
cystic, partially solid mass, consistent with a possible lymphangiomatous malformation versus herniated bowel (Fig. 1). The patient was noted to have normal intraabdominal viscera including 2 retroperitoneal kidneys on the MRI. On physical examination, the consulting surgeon noted a 2centimeter fascial defect, with a soft overlying hernia; contents were easily reducible. The overlying skin was thinned and hypopigmented, consistent with aplasia cutis congenita. Surgical exploration of the mass revealed a hernia sac containing a small cystic mass and a cord-like structure that coursed into the pelvis toward the uterus (Fig. 2). A laparoscope was used to visualize these structures through an umbilical incision. No normal ovary could be identified in the right pelvis; a normal ovary was identified on the left. The herniated cord-like structure and adjacent fibrous tissue were resected and sent to pathology. The fascial defect was repaired using 2-0 PDS (polydioxanone) in an interrupted fashion, and the subcutaneous layer and skin were closed separately. The patient did well postoperatively and was sent home after 1 day. The pathology was consistent with müllerian-type remnants comprising ducts lined by immature embryonic type epithelium embedded in fibroconnective
436
Fig. 1 Magnetic resonance imaging demonstrated a complex soft tissue mass in the region of the subcostal defect.
tissue and adjacent fragments of ovarian tissue (Fig. 3). The patient has done extremely well in follow-up.
2. Discussion Although there has been a previous report of a subcostal hernia secondary to traumatic abdominal wall weakness [1], only one previous report of a congenital subcostal hernia is cited in the literature. Nicksa et al [2] described a patient with a 2-cm left-sided fascial defect, associated with abnormalities in the costal cartilages, intermittent herniation of the stomach, and ipsilateral renal agenesis. Similarities to our case include that both mothers received fertility treatments, and in both cases, abnormalities of the thoracic cage were present. Neither patient has a known genetic syndrome. The comorbid congenital abnormalities in the 2 patients are quite different. Our patient had a ventricular septal defect, congenitally fused ribs, with aplasia cutis congenita diagnosed by a dermatologist in the area overlying the hernia. Normal development of the musculature in this area was present, and both kidneys were identified in the MRI. The most significant aspect of this subcostal hernia was the unusual contents of the hernia sac. Inguinal hernias have been reported to contain both congenitally abnormal and normal müllerian structures [3-5]. Ovaries have been reported in lumbar hernias [6]. A recent review article by Barnett et al [7] described the associations between several genetic syndromes and increased incidence of inguinal hernias. Our patient does not have a known genetic disorder; however, she does have a constellation of abnormal physical findings. Given her local anomalies—aplasia cutis conge-
J. Monteagudo et al. nita, congenitally fused 11th and 12th ribs, failure of descent of the ovary, and the presence of the hernia—there is a possibility that failure of differentiation of the ectoderm and mesoderm in this area may have contributed to poor migration of müllerian tissue and a possible weakness of the tissues in the subcostal area. Aplasia cutis congenita is characterized by a focal absence of skin at birth. Most cases, approximately 90%, occur on the scalp, although in rare cases, it has occurred on the trunk. It is suggested that these cases may be caused by vascular events or ischemia, viral infections, or adherence of the amniotic sac during development causing skin trauma [8]. Several cases of fetal papyraceous associated with truncal aplasia cutis congenita have been reported [9]. Although this was not a known feature of the case we present, multiple gestational pregnancies are more common in women receiving fertility treatments, and this may be a risk factor for aplasia cutis congenita. Molecular research suggests failure of the ectoderm to develop adequately in that area may lead to underlying embryologic defects as a result of disrupted inductive interactions between these layers, which occur during normal development [10]. Normally, the gonads develop as a pair of genital ridges formed by proliferation of the epithelium and underlying mesenchyme. During the sixth week of development, germ cells appear among the endodermal cells in the wall of the yolk sac. These germ cells migrate by ameobic movements to the primitive gonads. Once they arrive, they induce the development of the gonad into the testis or the ovary. If they fail to reach this location, the gonads do not develop [11,12]. In our case, it is possible that the descent was interrupted as the developing tissue lacked the appropriate signaling from the overlying ectoderm. It is important to recognize that weakness in the tissues as a result of aplasia cutis congenita may predispose the infant to a hernia in that area potentially involving tissue, which has undergone abnormal development. Operative management should include consideration
Fig. 2 Contents of the hernia sac found at operative exploration; arrow indicates atretic, dilated cord, and müllerian remnants.
Congenital subcostal hernia with unusual contents
437
Fig. 3 Pathology: A, Müllerian-type remnants comprising ducts lined by immature embryonic type epithelium (arrow) in hernia sac contents (H&E, ×200). B, Ovarian tissue with fibrosis and cystic follicle (arrow; H&E, ×100). C, Fimbria of fallopian tube (arrow; H&E, ×100).
of unusual contents of the hernia sac in an area of aplasia cutis congenita.
References [1] Polk HC, Newton WT. Abdominal parietal hernias at the costal margin. Annals of Surgery 1963;158(6):1047-52. [2] Nicksa GA, Christensen EP, Buckmiller TP. A case report of a congenital left subcostal hernia in a neonate. J Pediatr Surg 2009;44: 1653-5. [3] Kamio M, Nagata T, Yamasaki H, et al. Inguinal hernia containing functioning, rudimentary uterine horn and endometriosis. Obstet Gynecol 2009;113:563-6. [4] Gurer A, Ozdogan M, Ozlem N, et al. Uncommon content in groin hernia sac. Hernia 2006;10:152-5.
[5] Bradshaw KD. Ovarian and tubal inguinal hernia. Obstet Gynecol 1986;68(3 Suppl):50S-2S. [6] Geis WP, Hodakowski GT. Lumbar hernia. In: Nyhus LM, Condon RE, editors. Hernia. 4th ed. Philadelphia (PA): J.B. Lippincott Company; 1995. p. 412-24. [7] Barnett C, Langer JC, Hinek A, et al. Looking past the lump: genetic aspects of inguinal hernia in children. J Pediatr Surg 2009;44:1423-31. [8] Fagan LL, Harris PA, Coran AG, et al. Sporadic aplasia cutis congenita. Pediatr Surg Int 2002;18(5-6):545-7. [9] Maccario S, Fasolato V, Brunelli A, et al. Aplasia cutis congenita: an association with vanishing twin syndrome. Eur J Dermatol 2009;19(4): 372-4. [10] Priolo M, Silengo M, Lerone M, et al. Ectodermal dysplasias: not only ‘skin’ deep. Clin Genet 2000;58(6):415-30. [11] Sadler TW. Medical embryology. 10th ed. Philadelphia (PA): Lippincott Williams & Wilkins; 2006. p. 242-56. [12] Skandalakis JE, Gray SW. Embryology for surgeons. 2nd ed. Baltimore (MD): Williams & Wilkins; 1994. p. 736-69.