Indian J Pediatr DOI 10.1007/s12098-014-1418-3
SCIENTIFIC LETTER
Visceral Sequestration with Bladder Exstrophy: Associated or Coincidence? Vishesh Jain & Mamta Sengar & Anup Mohta & Nita Khurana
Received: 16 November 2013 / Accepted: 12 March 2014 # Dr. K C Chaudhuri Foundation 2014
To the Editor: We report an extremely rare case of classic bladder exstrophy with visceral sequestration. A one-mo-old girl presented to us with a lower abdominal mass with continuous dribbling of urine from its surface. The child was born of a full term normal vaginal delivery with uneventful antenatal period. The vitals were normal on examination. The child had lower abdominal defect with exstrophied bladder plate, bifid clitoris and pubic diastasis. A tubular mucosal structure was present on the superior edge of the bladder plate (Fig. 1). There was no discharge from the tubular structure. The child was passing stool through an anteriorly placed anus and a small blind ending anal pit was present anterior to it. Renal ultrasonography and echocardiography were normal. Primary turn-in of the bladder plate with excision of the sequestered bowel was performed. The histopathology of the sequestrated bowel revealed normal colonic mucosa (Fig. 2). The postoperative period was uneventful. Bladder exstrophy variants account for 8–10 % of bladder exstrophy cases with an incidence of 1 in 400,000–500,000 live births [1]. Visceral sequestration is also extremely rare malformation and has been reported along with covered exstrophy [2–4]. About 10 cases of covered exstrophy with visceral sequestration usually near the genital region have been reported [2, 3]. Classic bladder exstrophy along with sequestrated bowel attached to bladder plate has not been reported till date. The management of our case was not enigmatic and only excision of the sequestrated bowel with primary turn was needed. However, the etiology and the
embryology of the index case need further elaboration. It has been proposed that the sequestration along with exstrophy is due to over-persistence of the cloacal membrane with a snared-in loop of intestine [3]. This theory seems unlikely as none of the reported cases in literature had any associated gastrointestinal abnormalities like intestinal atresia. It is unlikely that intestinal continuity was restored spontaneously in all these reported cases of visceral sequestration. Craido et al. proposed that the visceral sequestration was due to rupture of gastrointestinal duplication [5]. Since visceral sequestration and bladder exstrophy variants are frequently associated, we believe that duplication may
V. Jain (*) : M. Sengar : A. Mohta Department of Pediatric Surgery, Chacha Nehru Bal Chikitsalaya, Delhi 110031, India e-mail: [email protected] N. Khurana Department of Pathology, Maulana Azad Medical College, Bahadur Shah Zafar Marg, New Delhi, India
Fig. 1 Photograph after partial mobilization of bladder plate showing visceral sequestration (black arrow), ureteric orifices (black arrowheads) and bifid clitoris (white arrowheads)
Indian J Pediatr
which otherwise may have persisted intracorporeally with or without symptoms. Conflict of Interest None. Role of Funding Source None.
References
Fig. 2 Microphotograph (Hematoxylin & Eosin×250) showing the large intestinal mucosa on the surface (black arrowhead) and the transitional mucosa underneath (white arrowhead)
contribute to the “wedge effect” which prevents mesodermal infiltration of the anterior abdominal wall. Another probable theory for association of these two malformations is that presence of bladder exstrophy with musculoskeletal defect and diastatic pelvis allows colonic duplication to rupture externally
1. Lowentritt BH, Van Zijl PS, Frimberger D, Baird A, Lakshmanan Y, Gearhart JP. Variants of the exstrophy complex: A single institution experience. J Urol. 2005;173:1732–7. 2. Kajbafzadeh AM, Aghdas FS, Tajik P. Complete covered duplication of the bladder, urethra, vagina, uterus and visceral sequestration. Int J Urol. 2006;13:1129–31. 3. Sarin YK, Sinha A, Ojha S. Covered exstrophy with visceral sequestration: Case report and review of literature. Eur J Pediatr Surg. 2005;15:437–40. 4. Mahdavi R. Covered exstrophy and visceral sequestration with complete double bladder. J Urol. 1994;151:455–6. 5. Criado E, Mesrobian HGJ, Bethea MC, Azizkhan RG. Heterotopic hindgut duplication: A cloacal remnant associated with exstrophy of the bladder. J Pediatr Surg. 1992;27:1605–7.
SCIENTIFIC LETTER
Visceral Sequestration with Bladder Exstrophy: Associated or Coincidence? Vishesh Jain & Mamta Sengar & Anup Mohta & Nita Khurana
Received: 16 November 2013 / Accepted: 12 March 2014 # Dr. K C Chaudhuri Foundation 2014
To the Editor: We report an extremely rare case of classic bladder exstrophy with visceral sequestration. A one-mo-old girl presented to us with a lower abdominal mass with continuous dribbling of urine from its surface. The child was born of a full term normal vaginal delivery with uneventful antenatal period. The vitals were normal on examination. The child had lower abdominal defect with exstrophied bladder plate, bifid clitoris and pubic diastasis. A tubular mucosal structure was present on the superior edge of the bladder plate (Fig. 1). There was no discharge from the tubular structure. The child was passing stool through an anteriorly placed anus and a small blind ending anal pit was present anterior to it. Renal ultrasonography and echocardiography were normal. Primary turn-in of the bladder plate with excision of the sequestered bowel was performed. The histopathology of the sequestrated bowel revealed normal colonic mucosa (Fig. 2). The postoperative period was uneventful. Bladder exstrophy variants account for 8–10 % of bladder exstrophy cases with an incidence of 1 in 400,000–500,000 live births [1]. Visceral sequestration is also extremely rare malformation and has been reported along with covered exstrophy [2–4]. About 10 cases of covered exstrophy with visceral sequestration usually near the genital region have been reported [2, 3]. Classic bladder exstrophy along with sequestrated bowel attached to bladder plate has not been reported till date. The management of our case was not enigmatic and only excision of the sequestrated bowel with primary turn was needed. However, the etiology and the
embryology of the index case need further elaboration. It has been proposed that the sequestration along with exstrophy is due to over-persistence of the cloacal membrane with a snared-in loop of intestine [3]. This theory seems unlikely as none of the reported cases in literature had any associated gastrointestinal abnormalities like intestinal atresia. It is unlikely that intestinal continuity was restored spontaneously in all these reported cases of visceral sequestration. Craido et al. proposed that the visceral sequestration was due to rupture of gastrointestinal duplication [5]. Since visceral sequestration and bladder exstrophy variants are frequently associated, we believe that duplication may
V. Jain (*) : M. Sengar : A. Mohta Department of Pediatric Surgery, Chacha Nehru Bal Chikitsalaya, Delhi 110031, India e-mail: [email protected] N. Khurana Department of Pathology, Maulana Azad Medical College, Bahadur Shah Zafar Marg, New Delhi, India
Fig. 1 Photograph after partial mobilization of bladder plate showing visceral sequestration (black arrow), ureteric orifices (black arrowheads) and bifid clitoris (white arrowheads)
Indian J Pediatr
which otherwise may have persisted intracorporeally with or without symptoms. Conflict of Interest None. Role of Funding Source None.
References
Fig. 2 Microphotograph (Hematoxylin & Eosin×250) showing the large intestinal mucosa on the surface (black arrowhead) and the transitional mucosa underneath (white arrowhead)
contribute to the “wedge effect” which prevents mesodermal infiltration of the anterior abdominal wall. Another probable theory for association of these two malformations is that presence of bladder exstrophy with musculoskeletal defect and diastatic pelvis allows colonic duplication to rupture externally
1. Lowentritt BH, Van Zijl PS, Frimberger D, Baird A, Lakshmanan Y, Gearhart JP. Variants of the exstrophy complex: A single institution experience. J Urol. 2005;173:1732–7. 2. Kajbafzadeh AM, Aghdas FS, Tajik P. Complete covered duplication of the bladder, urethra, vagina, uterus and visceral sequestration. Int J Urol. 2006;13:1129–31. 3. Sarin YK, Sinha A, Ojha S. Covered exstrophy with visceral sequestration: Case report and review of literature. Eur J Pediatr Surg. 2005;15:437–40. 4. Mahdavi R. Covered exstrophy and visceral sequestration with complete double bladder. J Urol. 1994;151:455–6. 5. Criado E, Mesrobian HGJ, Bethea MC, Azizkhan RG. Heterotopic hindgut duplication: A cloacal remnant associated with exstrophy of the bladder. J Pediatr Surg. 1992;27:1605–7.
