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Table of Contents
LETTER TO EDITOR
Year : 2015  |  Volume : 31  |  Issue : 2  |  Page : 280-281

Anesthetic management of a patient with Wilms tumor, aniridia, genital anomalies and mental retardation syndrome undergoing right nephrectomy


Department of Anaesthesiology, Krishna Institute of Medical Sciences, Karad, Maharashtra, India

Date of Web Publication16-Apr-2015

Correspondence Address:
Akshaya N Shetti
Department of Anaesthesiology, Krishna Institute of Medical Sciences, Karad, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9185.155216

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How to cite this article:
Shetti AN, Dhulkhed VK, Gujrathi D, Sangolli SG. Anesthetic management of a patient with Wilms tumor, aniridia, genital anomalies and mental retardation syndrome undergoing right nephrectomy. J Anaesthesiol Clin Pharmacol 2015;31:280-1

How to cite this URL:
Shetti AN, Dhulkhed VK, Gujrathi D, Sangolli SG. Anesthetic management of a patient with Wilms tumor, aniridia, genital anomalies and mental retardation syndrome undergoing right nephrectomy. J Anaesthesiol Clin Pharmacol [serial online] 2015 [cited 2019 Nov 18];31:280-1. Available from: http://www.joacp.org/text.asp?2015/31/2/280/155216

To the Editor,

Wilms tumor, aniridia, genital anomalies and mental retardation syndrome is a congenital disorder, consists of multiple systemic anomalies. It accounts 2% of patients with Wilms' tumor. [1] Anomalies uncommonly seen are congenital diaphragmatic hernia (CDH), [2],[3] cardiomyopathy, congenital heart diseases (CHD) and scoliosis. Wilms tumor and male genital anomalies are caused by deletion of the WT1 gene. Aniridia is due to deletion of PAX 6 gene and deletion of multiple genes leads to mental retardation. [4]

A 4-year-old, 12 kg female child presented with pain abdomen for 5 days and hematuria for 3 days. Mother gave history of abdominal distention since 1 month, which was gradual in onset, progressive and associated with delayed milestones. No significant birth or family history was noticed.

On examination child was moderately mentally retarded (slow in understanding, using language, and limited ability to communicate, could count fingers). Airway examination showed Mallampatti Grade I (MPG I). Per abdominal examination showed, distended abdomen with tenderness in right hypochondriac and iliac region. No abnormality detected in cardiorespiratory, musculoskeletal system. Eye examination showed bilateral partial aniridia [Figure 1], nystagmus with myopic degenerative fundus and normal intraocular pressure.
Figure 1: Right and left eyes with partial aniridia

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Complete blood count, renal function test, electrocardiogram (ECG), echocardiography and chest radiograph were normal. Ultrasound abdomen showed a large well-encapsulated mass in right renal area with normal flows in right renal vein and inferior vena cava, no hepatic metastasis, ascites or organomegaly. Computed tomography scan abdomen confirmed the diagnosis.

Patient was premedicated with syrup promethazine 0.5 mg/kg oral, previous night and 2 h before surgery. Child was kept nil per oral for 6 h. All standard monitoring like ECG, pulse oximetry, noninvasive blood pressure were done. Child received injection midazolam 0.05 mg/kg, injection fentanyl 1.5 mcg/kg intravenously. After preoxygenation (3 min), patient was induced with injection thiopentone 5 mg/kg and injection suxamethonium 2 mg/kg intravenously and 5 mm endotracheal tube was inserted. Anesthesia was maintained with O 2 , N 2 O (1:1), injection fentanyl, injection vecuronium and sevoflurane. Kidney position was given in left lateral position, caring pressure points and airway. Nephrectomy [Figure 2] was uneventful with allowable blood loss, which lasted for 2 h. Injection bupivacaine (0.25%, 10 ml) was infiltrated at incision site. Reversal was done with injection glycopyrrolate (0.04 mg/kg), injection neostigmine (0.05 mg/kg) and extubated once extubation criteria were met. Patient's temperature was maintained around 35.5-36.8°C. Patient received Isolyte-P intravenous (IV) fluid for maintenance and shifted to intensive care unit for further management. Injection paracetamol 20 mg/kg (TID, IV) and injection tramadol 2 mg/kg (BD, IV) received as postoperative analgesia. The histopathological report confirmed Wilm's tumor.
Figure 2: Diseased right kidney

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Wilms tumor, aniridia, genital anomalies and mental retardation is a rare genetic disorder and patients may come for multiple surgeries due to multiple systemic disorders. [1] Problems encountered are difficulty in securing airway due to obesity, micrognathia and kyphoscoliosis. [5] Inducing and weaning from the ventilator due to mental retardation is challenge to anesthesiologist. In our case, MPG was I and we could ventilate, intubate and also extubate the patient. Associated CHD and CDH make it challenging. In our patient, cardiorespiratory systems were normal. Genitourinary abnormalities may not be present in the female patient. [1]

In summary, procedure was safely performed under general anesthesia in this rare condition. Strong carry home message is, such patients may have multiple systemic disorder leading to perioperative morbidity and mortality.

 
  References Top

1.
Fischbach BV, Trout KL, Lewis J, Luis CA, Sika M. WAGR syndrome: A clinical review of 54 cases. Pediatrics 2005;116:984-8.  Back to cited text no. 1
    
2.
Scott DA, Cooper ML, Stankiewicz P, Patel A, Potocki L, Cheung SW. Congenital diaphragmatic hernia in WAGR syndrome. Am J Med Genet A 2005;134:430-3.  Back to cited text no. 2
    
3.
Martín Campagne E, Guerrero Fernández J, Gracia Bouthelier R, Tovar Larrucea JA. Association between WAGR syndrome and diaphragmatic hernia. An Pediatr (Barc) 2006;65:616-8.  Back to cited text no. 3
    
4.
Mahale A, Poornima V, Shrestha M. WAGR syndrome - a case report. Nepal Med Coll J 2007;9:138-40.  Back to cited text no. 4
    
5.
Butler MG, Hayes BG, Hathaway MM, Begleiter ML. Specific genetic diseases at risk for sedation/anesthesia complications. Anesth Analg 2000;91:837-55.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2]



 

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