LETTERS TO THE EDITOR
|Year : 2023 | Volume
| Issue : 6 | Page : 550-551
VACTERL association with dorsal pancreatic agenesis
Gaurav, Suryansh Arora, Kavita Vani
Department of Radiodiagnosis, ABVIMS and Dr. RML Hospital, New Delhi, India
|Date of Submission||11-Aug-2023|
|Date of Decision||09-Sep-2023|
|Date of Acceptance||13-Sep-2023|
|Date of Web Publication||02-Nov-2023|
Department of Radiodiagnosis, ABVIMS and Dr. RML Hospital, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gaurav, Arora S, Vani K. VACTERL association with dorsal pancreatic agenesis. J Indian Assoc Pediatr Surg 2023;28:550-1
We report a case of a 3-year-old boy, diagnosed with VACTERL association with sacral spina bifida occulta [Figure 1]a, absent anal opening with rectal atresia [diagnosed on high-pressure colostogram – [Figure 1]b], ostium secundum atrial septal defect [Figure 1]c, right renal hypoplasia [Figure 1]d, right side refluxing megaureter with ectopic insertion of the right ureter [Figure 1]e, and bilateral radial club hands [Figure 1]f.
|Figure 1: (a) Computed tomography (CT) volumetric rendering technique image (dorsal view) of the sacrum showing sacral spina bifida, (b) high-pressure colostogram lateral view showing the distal limit of contrast above the pubococcygeal line with no fistulous communication of bowel with genitourinary system suggestive of high anorectal malformation – rectal atresia; (c) echocardiography (subxiphoid view) showing small atrial septal defect with left-to-right shunting of blood; (d) CT coronal image in soft-tissue window showing small right kidney, with normal-sized left kidney; (e) micturating cystourethrogram (right anterior oblique view) showing ectopic insertion of the right ureter at the bladder neck and reflux of contrast into the right ureter reaching up to the renal pelvis with the dilated and tortuous right ureter; (f) X-ray postero-anterior view of the bilateral hands showing complete absence of the radius and the thumb with radial deviation of the hand and wrist, diagnostic of radial club hand|
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A further detailed search for other congenital anomalies showed that there was nonvisualization of the body and tail of the pancreas, with normal size and attenuation of the head, neck, and uncinate process [Figure 2]. A diagnosis of dorsal pancreatic agenesis was made as there was no prior history of acute pancreatitis or severe abdominal pain.
|Figure 2: Computed tomography axial image showing nonvisualization of the neck, body, and tail of the pancreas – dorsal pancreatic agenesis|
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The child did not report any feeding difficulties or recurrent respiratory infections. No abnormality was identified on chest radiographs. In our case, five of six malformations were present to conclusively diagnose VACTERL association.
VACTERL association does not include other disorders of the gastrointestinal tract, including dorsal pancreatic agenesis. However, VACTERL includes foregut malformations in the form of tracheal and esophageal abnormalities. The pancreas develops from progenitor cells of the foregut, which leads to the development of the dorsal and ventral pancreatic buds. In dorsal pancreatic agenesis, there is absent development of the dorsal pancreatic primordium.
Yoon et al., in 2018, reported a case of VACTERL association with bile duct confluence abnormality. The bile ducts and liver are also derivates of the foregut, similar to the pancreas.
The exact etiology of both conditions remains unknown. However, no case has yet been reported showing an association of dorsal pancreatic agenesis with VACTERL association. Dorsal pancreatic agenesis in a patient with VACTERL association, as in the above-mentioned case, can be an incidental finding or can be a variant of foregut malformation of VACTERL association. There may be an etiological association between VACTERL and other foregut malformations, including pancreatic and biliary anomalies, for which further studies are required.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Cienfuegos JA, Rotellar F, Salguero J, Benito A, Solórzano JL, Sangro B. Agenesis of the dorsal pancreas: Systematic review of a clinical challenge. Rev Esp Enferm Dig 2016;108:479-84.
Yoon Y, Kim K, Yeom SK, Lee J, Lee Y. A case report of intrahepatic bile duct confluence anomalies in VACTERL syndrome. Medicine (Baltimore) 2018;97:e12411.
Faure S, de Santa Barbara P. Molecular embryology of the foregut. J Pediatr Gastroenterol Nutr 2011;52 Suppl 1:S2-3.
[Figure 1], [Figure 2]