Supernumerary Kidney in a Term Infant with VATER ...

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postoperative course secondary to acute renal failure, new-onset .... bladder ultrasound at 3 years of age demonstrated per- .... Pensacola, FL 32504. E-mail: ...

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FIG. 1.

Monoclonal proliferation of plasma cells.

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widely. All areas of the colon, cecum to rectum, have been reported as sites of involvement. However, the sigmoid colon is the most frequently reported site of involvement. Most patients undergo resection. We present two male patients with sigmoid lesions, one patient status post orthotopic liver transplant and the other with concomitant carcinoid tumor. Neither of these clinical scenarios has been previously reported. Overall, primary colonie plasmacytomas are rare entities and our knowledge of their natural history is restricted to that described in case reports. These lesions present with a variety of symptoms and can be located throughout the colon. As a result of the rarity of this disease, no standardized treatment regimen has been recognized. Historically, treatment has been primarily surgical with segmental resection. Jamie E. Jones. M.D. Marc I. Brand. M.D. Theodore J. Saclarides, M.D. Section of Colon and Rectal Surgery Department of General Surgery Rush University Medical Center Chicago, Illinois Shriram Jakate, M.D., Department of Pathology Rush University Medical Center Chicago, Illinois


10 X 5 X 6 cm piasmacytoma of the sigmoid.

mass was found in the distal sigmoid colon; pathology suggested plasmacytoma. At laparotomy. a large inflamed mass involving the sigmoid colon was adherent to the dome of the bladder and the anterior abdominal wall. A Hartmann resection of the sigmoid colon was performed. Microscopy showed a 10 x 5 x 6-cm polypoid high-grade plasmacytoma involving the entire thickness of the bowel wall (Fig. 2). Regional lymph nodes were involved. This patient had a complicated postoperative course secondary to acute renal failure, new-onset atrial fibrillation, and aspiration pneumonia. The patient died on Day 19. Primary colonie plasmacytomas are exceedingly rare. Twenty-one cases have been referred to in the literature; only 16 have enough clinical detail to define the lesions as primary, without bone marrow involvement. The average age at presentation is 60.4 years. Our review showed an increased proportion of male patients (78%). The presenting symptoms reported simulate those of colonie adenocarcinoma and vary

REFERENCES 1. Shih LY. Dunn P. Leung WM, el al. Localised plasmacytomas in Taiwan: Comparison between extramedullary plasmacytoma and solitary plasmacytoma of bone. Br J Cancer 1995;71: 128. 2. Latteuneddii A. Fameti F. Lucci E. A case of primary extramedullary plasmacytoma of the colon. Int J Colorectal Dis 2(K)4; 19:289-91. 3. Schridde H. Weitere Untersuchungen über die Kornelungen der Plasmazellen. Centralbl Allg Pathol Pathol Anat t905;16: 433-5. 4. Gupta V. Nahak B. Sakhuja P. et al. Primary isolated extramedullary plasmacytoma of colon. World J Surg Oncol 2007;5: 47-51.

Supernumerary Kidney in a Term Infant with VATER Association To the Editor: We report a case of supernumerary kidney in a male term infant with VATER association. Gadoliniutn Address correspondence and reprint requests to Kenneth W. Gow, M.D., Division of Pédiatrie Surgery. Department of Surgery. Emory University School of Medicine, Atlanta, GA. Current address: Kenneth W. Gow. M.D.. 4800 Sand Point Way NE, M/S W-7729. P.O. Box 5731. Seattle, WA 98105. E-mail: [email protected]

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gadolinium contrast, indicating nonfunction. The right and left kidneys appeared normal with equal difierentiai renal function. Hydroureteronephrosis was noted to the level of the bladder on the left without evidence of obstruction. Voiding cystourethrogram and renalbladder ultrasound at 3 years of age demonstrated persistent right ureteral reflux and dilation of the leftsided collecting system. The patient underwent cystoscopy and laparotomy, which revealed a low Y configuration of a duplicated right ureteral system and an anterior urethral stricture. A bilateral cross-trigonal ureteral reimplantation and appendiceal Mitrofanoff were performed to address both problems. The patient continues to have normal renal clearance assessed by La.six renal scan with a creatinine of 0.4 mg/dL. The VATER association is a constellation of anomalies of the vertebrae, anorectal region, tracheoesophagecil region, and renal or genitourinary tract. In those infants that present with anorectal malformation, approximately half will also have one or more genitourinary anomalies.' Genitourinary anomalies described include renal agenesis, horseshoe kidney, multicystic dysplastic kidney, urolithiasis. ureteropelvic obstruction, retlux, ambiguous genitalia, cryptorchidism, bifid scrotum, and hypospadias.' Although synchronous cryptorchidism and unilateral renal agenesis are common, supernumerary kidney has not been described to have a relationship to testis development. Indeed, the presence of this unique anomaly may be a mere chance occurrence and not related to the VATER association because no clear genetic etiology has been defined. Nonetheless, the presence of this type of anomaly with the other known anomalies would suggest that there is likely an association. Finally, although we describe the first living case of a patient with VATER and a supernumerary kidney, there have been two case reports of children who had similar findings. The first had a supernumerary kidney; ventricular septal defect, radial, and ulnar deformities; and underdevelopment of testes and epididymis.- The second had supernumerary kidney, cloacal exstrophy, exomphalos, absence of testes, anorectal malformation, a penis on each side of the perineum, myelomeningocele, and he m i vertebrae.-^ Supernumerary kidney is a rare anomaly with less than 100 cases described in the literature. It differs from a double kidney in that it is considered an accessory organ with its own collecting system, blood supply, and distinct encapsulated tissue.^ Because supernumerary kidneys are rarely seen, there are few described associated anomalies. However, those li.sted include ureteral atresia. vaginal atresia, complete duFicj. 1. Magnciii: rcsunaiicc Lirogram demonstrates left megaureler. right ureteral reflux, and supernumerary kidney in the pel- plication of the urethra and penis, ectopic ureterai vic region (arrow). opening, horseshoe kidney, imperforate anus, ven-

magnetic resonance urogram (MRU) and voiding cystourethrogram were used to define the anatomic relationship of the kidney. This represents, to our knowledge, the first living case of supernumerary kidney in the VATER association cotnplex and the first to use MRU in the diagnosis. A male term infant was noted at birth to have an impertorate anus, clubbed feet, right cryptorchidism, and a two-vessel umbilical cord. He underwent diverting colostomy for a suspected high imperforation and cystoscopy. which revealed a bladder diverticulum and urethral stricture leading to vesicostomy for urinary diversion. Further investigations revealed a tethered spinal cord and right ureteral duplication. In light of the constellation of anomalies, the patient was considered to have the VATER association. At 15 months of age. he underwent laparoscopic takedown of the colostomy with anal pull-through at which time it was noted that he had a rectoprostatic fistula and left megaureter. A repeat voiding cystourethrogram and urodynamics demonstrated Grade III right ureteral reflux and a neurogenic bladder. Additionally, there was retlux into a second right-sided structure, which was postulated to be an atretic ureter, or supernumerary kidney. MRU (Fig. I ) revealed a predominantly cystic stmcture appearing to connect to the bladder confirming the presence of a dysplastic supernumerary kidney (see arrow. Fig. 1). The kidney measured 2.5 cm in leniith and did not enhance with the administration of



tricular septal defects, meningomyeloceles, and coarctation of the aorta.-- ^ Supernumerary kidneys may be diagnosed with a variety of diagnostic studies, including intravenous urogram. nuclear scintigraphy, ultrasonography. CT. or MRI."* In our case, MRU was useful in defining the renal anatomy for diagnosis and management. The management of this rare disorder is based on careful monitoring and early consideration for nephrectomy of the supernumerary kidney should any complication arise such as hydronephrosis, carcinoma, adenomas, pyonephrosis, pyelonephritis, cysts, or calculi. Because our patient did not have any of these, he was managed with observation alone. In conclusion, we have reported the first living patient with VATER and a supernumerary kidney. The case underscores the need to carefully investigate the genitourinary tract in such children. We would advocate that MRU would be the best investigation in this scenario to define both anatomy and function. Finally, asymptomatic patients can be followed for the potential development of complications. Kelvin A. Moses, M.D. Andrew J. Kirsch, M.D. Department of Urology Emory University School of Medicine Atlanta, Georgia Erik A. Heiss, M.D. Faculty of Medicine Emory University School of Medicine Atlanta, Georgia Kenneth W. Gow, M.D. Division qf Pédiatrie Surgery Department of Surgery Emory University School of Medicine Atlanta, Georgia REFERENCES 1. Cho S. Moore SP. Fangman T. Otie hundred three consecutive patients with anorectal malformations and their associated anomalies. Arch Pcdiatr Adolesc Med 2001; 155:587-91.

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Serial Hemoglobin Levels Play No Significant Role in the Decision-Making Process of Nonoperative Management of Blunt Splenic Trauma To the Editor: Injury to the spleen was traditionally considered a potentially life-threatening situation requiring emergent splenectomy; however, nonoperative management strategies have found the spleen to be more forgiving than previously thought, allowing increasing grades of injury to be safely and successfully managed without surgery. Current efforts have focused on better defining key factors that identify and better predict patients who fail nonoperative management (FNOM). Multiple studies have presented combinations of the following factors as their defining end points for FNOM and splenectomy: 1) hemodynamic instability; 2) worsening abdominal examination with progression to peritonitis; 3) transfusion of one or more units of blood; 4) worsening CT scan findings; and 5) a decrease in hemoglobin levels.'-- In the large multiinstitution EAST trial, indications for FNOM and operation were as follows: 1) decreasing hemoglobin levels (36%); 2) changes on follow-up CT scans (22%); 3) worsening abdominal examination (17%); 4) hypotension (16%); and 5) tachycardia (5%). Interestingly, nearly twice as many patients in that study were taken for splenectomy for decreasing hemoglobin levels as compared with hypotension (36% vs 16%).-* In a continually increasing cost-sensitive healthcare environment, re-evaluation of treatment protocols focusing on elimination of unnecessary laboratory tests seems prudent and necessary. We propose that emergent operative intervention for FNOM is dictated primarily by the presence of shock (hypotension and tachycardia) and that serial hemoglobin levels may not play a significant role in the decision-making process and could be eliminated from standard observational protocols. Elimination of these tests will cut down on unnecessary patient discomfort, physicianinduced anemia, and provide significant cost savings. To determine the role of serial hemoglobin levels in declaring FNOM for blunt splenic trauma, a retrospective review of the trauma registry at a Level I trauma center was performed from 1998 to 20G5. A total of 865 patients with blunt trauma and splenic injury were identified. After excluding 546 patients with multiple traumatic injuries, 319 with isolated splenic injury re-

2. Zondek LH. Zotidek T. Re: A pitfall in the diagnosis of supernumerary kidney. Br J Urol I991;68:332. 3. N'Guessan G. Stephens FD. Supernumerary kidney. J Urol 1983;l30:649-53. 4. Oto A. Kerimoglu U. Eskicorapci S. et al. Bilateral supernumerary kidney: Imaging findings. JBR-BTR 2002;85:300-3.

Address correspondence and reprint requests to Indermeet S. Bhullar. M.D.. Director of Trauma Intensive Care Unit. Department of Trauma Services. Sacred Heart Hospital, 5151 North 9th Avenue. Pensacola, FL 32504. E-mail: [email protected]

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