Year : 2014 | Volume
: 25 | Issue : 2 | Page : 398--401
Intussusception in two pediatric patients associated with nephrotic syndrome
Reham I Al-Mardini, Rana E Haddad
Department of Pediatric Nephrology, Queen Rania Abdulla Children Hospital, Amman, Jordan
Reham I Al-Mardini
Department of Pediatric Nephrology, Queen Rania Abdulla Children Hospital, Amman
We report two cases of intussusception in two Jordanian boys during a relapse of nephrotic syndrome. The first patient had minimal change disease and the second patient had diffuse mesangial sclerosis. The most prominent symptom was abdominal pain, but the other classic sign of currant jelly stool was absent. In spite of the fact that both patients could have had other causes of abdominal pain, intussusception was suspected and confirmed by ultrasound and was treated successfully. Intussusception should be considered in the differential diagnosis in nephrotic patients presenting with abdominal pain.
|How to cite this article:|
Al-Mardini RI, Haddad RE. Intussusception in two pediatric patients associated with nephrotic syndrome.Saudi J Kidney Dis Transpl 2014;25:398-401
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Al-Mardini RI, Haddad RE. Intussusception in two pediatric patients associated with nephrotic syndrome. Saudi J Kidney Dis Transpl [serial online] 2014 [cited 2021 Jul 27 ];25:398-401
Available from: https://www.sjkdt.org/text.asp?2014/25/2/398/128584
Intussusception is one of the most common causes of acute abdomen in infancy due to telescoping of one portion of the intestine into the other. It causes an obstruction to the passage of intestinal contents beyond the affected segment.  It occurs most commonly in children between six months and two years of age, and a vast majority of cases in this age group are idiopathic.
Nephrotic syndrome is characterized by proteinuria, hypoalbuminemia and edema; abdominal pain in nephrotic patients is related to intravascular volume depletion or secondary to peritonitis, other causes of acute abdomen can occur. Intussusception has not been described in nephrotic patients except in few case reports. We report these two cases to demonstrate the clinical presentation and the management of intussusception in nephrotic patients with the need to have a high index of suspicion for the diagnosis as the management is life saving.
M. N. is a 7-year-old male patient who has steroid-dependent nephrotic syndrome due to minimal change disease since the age of two years. He was complaining of mild periorbital edema with a picture of relapse and was started on treatment with prednisone 60 mg/m 2 / day; four days later, he presented with a history of sudden onset of colicky abdominal pain associated with vomiting but had passed normal stool that morning. On examination, he was in pain and afebrile and his blood pressure was on the 10 th percentile for his age and height, his heart rate was 90 beats/min and he had mild periorbital and lower limb edema, cold extremities and delayed capillary refill time. The abdomen was distended with tenderness and guarding but there were no palpable masses.
Urgent abdominal ultrasound showed a targetlike lesion in the right upper abdomen containing bowel and some lymph nodes representing intussusceptions, with free fluid in the pelvis. Lab investigations showed white blood cells (WBCs) 27.3 × 10 6 /L, packed cell volume (PCV) 45%, hemoglobin (HB) 14.5 mg/dL and platelets 539 × 10 9 /L. Blood urea nitrogen (BUN) was 52 mg/dL, creatinine was 0.37 mg/dL and total protein was 43 with serum albumin 13 mg/dL. He was admitted and put on nothing per Os; human albumin was given at dose of 2 g/kg and iv methylprednisolon was given at dose of 60 mg/m 2 with iv Ceftriaxon for the suspension of having peritonitis. Aafter stabilization of his condition, abdominal ultrasound was repeated hoping that the intussusception would have reduced. Unfortunately, he still had the lesion and therefore open reduction was performed. Operative findings showed an ilio-cecal intussusception with lymph node at the ileocecal valve as the leading point. Open reduction was done easily and he was discharged after two days in good general condition. Histopathology of the lymph node showed reactive lymphadenopathy.
S. H. is a 3-year-old male child diagnosed to have congenital nephrotic syndrome at the age of 1.5 years due to diffuse mesangial sclerosis (DMS). He had relatively slow deterioration of kidney function tests, with a baseline creatinine of 1.5 mg/dL. He was maintained on vitamin D, L-thyroxin and captopril. He continued to have significant proteinuria but with mild puffiness. He presented to the emergency room with a two days history of vomiting with sudden-onset colicky abdominal pain and passage of soft but not currant jelly stool. On examination, he was in pain, pale with dry mucus membrane and afebrile and his blood pressure was 95/55 mm Hg and heart rate was 132 beats/min with the presence of generalized abdominal guarding and normal PR examination. Abdominal ultrasound showed target-like lesion with sausage-like mass of 13 cm in length. The bowels appeared thick edematous, measuring 1 cm in thickness, with enlargement of multiple mesenteric lymph nodes. Laboratory investigations showed creatinine of 2.0 mg/dL, BUN of 28 mg/dL, total protein of 3.78 mg/dL and serum albumin of 1.52 mg/dL, calcium of 5.7 mg/dL, WBC 18 × 10 3 /mm 3 , PCV 28%, platelets of 729,000/mm 3 , pH 7.25 and HC03 18 with base excess of -8. He was stabilized by keeping him NPO, naso-gastric drain and intravenous fluids. Successful pneumatic reduction of intussusception under fluoroscopy was performed. He was discharged after two days in good general condition.
Intussusception has not been described to be associated with nephrotic syndrome in the text books. It is only published as a few reported cases in the literature in both adult and mainly pediatric patients. Both males and females can be affected with the diagnosis of familial or idiopathic nephrotic syndrome. The first reported case was in 1975, which was diagnosed by post-mortem autopsy as ileocolic intussusception in a 19-year-old male patient who had steroid-resistant nephrotic syndrome. 
In 1991, the second case was reported from Nottingham, United Kingdom, in a 6-month-old female infant who was diagnosed by kidney biopsy to have difuse mesengial sclerosis after the initial presentation of intussusceptions, which failed to be reduced by barium enema and was reduced surgically. 
In 1992, a 4-year-old female patient who had ileocolic intussusception presented during the first presentation of minimal change nephrotic syndrome.  Another 5-year-old male patient who had a diagnosis of nephrotic syndrome secondary to IgM nephropathy developed ileocolic intussusception during a relapse, described in 2003. 
The last case was reported in 2005 in a 2-year-old male who had ileoileal intussusception during the first presentation of nephrotic syndrome. 
Intussusception is the most common cause of intestinal obstruction during early childhood and occurs most commonly at six months to two years of age. If not recognized and treated, it will lead to abdominal catastropheis and can ultimately result in mortality. Usually, it is idiopathic; but, if it presents at older ages, a pathologic leading point should be ruled out. The age of our patients is three and seven years. Therefore, we looked for a pathologic leading point, and found that the small reactive lymph node was the leading point in the first case and, although multiple lymph node enlargements were seen by ultrasound in the second case, no specific leading point was found. These lymph node enlargements may be due to associated peritonitis, especially with the elevated WBC in both patients. The differentiation between idiopathic causes or the presence of pathologic leading point is very important as the pathophysiology and the management is different.
The most significant associated risk factor is the presence of focal lesion of thickening of the bowel wall, which leads to abnormal bowel motility. The common focal lesions as leading points include Meckel diverticulum, intestinal polyp, duplication cyst and lymphoma. Diseases that involve the bowel more diffusely by causing wall thickening and/or affecting the bowel motility include Henoch-Schonlein purpura, cystic fibrosis and celiac disease. 
In nephrotic patients, we noticed that they developed intussusception during the period of relapse and hypoalbuminemia, when generalized edema is present, which could indicate that edema in the bowel wall as a part of anasarca may predispose to intussuscption. More over, the presence of peritonitis would result in lymph node enlargement, which could also act as a leading point for intussusception.
The classic triad presentation of intussusception is abdominal pain, vomiting and currant jelly stool, which were lacking in our patients. Whereas the first patient was lacking the classic currant jelly stool, he had a picture of intravascular volume depletion; the second case had only abdominal pain and vomiting. The full blown picture of intussusception may be masked in a patient with nephritic syndrome as the abdominal pain is severe and presents early, which did not give time for currant jelly stool to form.
The intussusception in the first patient was reduced surgically without a trial of enema reduction, taking into consideration the age of the patient, while the other was reduced successfully by barium enema.  The first patient was intravascular volume depleted and treated with human albumin with the hope of reversal of intussusception; but, unfortunately, follow-up ultrasound failed to reveal any improvement.
Ultrasound is the procedure of choice for the diagnosis of intussusception, which is operator dependent. Ultrasound is a sensitive and specific test for detecting intussusception and should be utilized as a first-line examination for the assessment of possible pediatric intussusceptions.  In these two patients, the diagnosis was easily made by ultrasound.
A high index of suspicion of intussusception is needed in the differential diagnosis of nephrotic patients who present with abdominal pain or vomiting. The diagnosis can be confirmed easily by performing an abdominal ultrasound. Stabilization of the patients is needed before attempting surgical or pneumatic reduction, and the outcome is favorable with timely treatment; if the diagnosis is missed, the condition is fatal.
|1||del-Pozo G, Albillos JC, Tejedor D, et al. Intussusception in children; Current concept in diagnosis and enema reduction. Radiographics 1999;19:299-319.|
|2||Takano T, Nakagawa M, Ishiwara M. Jaundice, vomiting and abdominal pain (nephrotic syndrome): Intussusception (with lipoid nephrosis). Nihon Rinsho 1975;SpecNo:978-9,1502-5.|
|3||Crawshaw PA, Watson AR, Ranee CH. Intussusception nephrosis and Drash syndrome. Eur J Pediatr 1991;150:813-4.|
|4||Fitz-Henry J, Watson AR, Ranee CH, Broderick NJ. Intussusception associated with nephrotic syndrome. Br J Surg 1992;79:1201.|
|5||Kietkajornkul C, Vithayasai N, Ratanaprakarn W, Buranakitjaroen V. Intussusception associated with a relapsing nephrotic patient: A case report. J Med Assoc Thai 2003;86 Suppl 3:S596-9.|
|6||Asai K, Tanaka S, Tanaka N, Tsumura K, Kato F, Kikuchi K. Intussusception of the small bowel associated with nephrotic syndrome. Pediatr Nephrol 2005;20:1818-20.|
|7||Navarro O, Daneman A. Diagnosis and management of those with an identifiable or predisposing cause and those that reduce spontaneously. Pediatr Radiol 2004;34:305-12.|
|8||Cho MH, Hwang HH, Choe BH, et al. The reversal of intussusception associated with nephrotic syndrome by infusion of albumin. Pediatr Nephrol 2009;24:421-2.|
|9||Hryhorczuk AL, Strouse PJ. Validation of US as a first-line diagnostic test for assessment of pediatric ileocolic intussusception. Pediatr Radiol 2009;39:1075-9.|