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K L N Rao

Publications and source records attributed to K L N Rao.

At least 19 recordsLinked to original sources

Fine needle aspiration biopsy in pediatric space-occupying lesions of liver: a retrospective study evaluating its role and diagnostic efficacy.

BACKGROUND: Liver space-occupying lesions (SOLs) in the pediatric population form a unique and distinct spectrum as compared with those in the adult population. There are no large studies available in the English literature regarding fine needle aspiration cytology (FNAC) in pediatric liver SOLs. AIM: This study was done to familiarize with the cytomorphological features of these distinctive benign and malignant lesions of liver and to determine the specificity, sensitivity, and diagnostic efficacy of FNAC. METHODS: A total of 41 FNACs from pediatric liver SOLs were reviewed, and the diagnoses were correlated with clinical and radiological findings, histopathological diagnoses, and/or follow-up information. These were categorized into malignant, benign, and indeterminate groups. RESULTS: There were 20 malignant lesions that included hepatoblastoma (7), hepatocellular carcinoma (4), undifferentiated embryonal sarcoma of liver (3), and metastases (6). The 12 benign lesions included abscess (8), 1 case each of mesenchymal hamartoma, focal nodular hyperplasia, hydatid cyst, and tuberculosis. In the overall FNAC performance calculation, the specificity of FNAC was 100%, sensitivity 95%, positive predictive value 100%, negative predictive value 92.3%, and diagnostic accuracy 96.9%. CONCLUSION: The recognition of the distinct cytomorphological features of pediatric focal liver lesions, in conjunction with the clinical data and radiological findings, make FNAC a useful primary diagnostic modality in these unique and distinctive lesions.

Adolescent↗

True hermaphroditism: clinical profile and management of six patients from North India.

True hermaphroditism (TH) is an uncommon cause of genital ambiguity. This is a retrospective compilation of six cases of true hermaphroditism seen at our institute over the last one and a half decades. Clinical presentation was wide-ranging, with age at presentation varying from 2 months to 41 years and symptoms ranging from ambiguous genitalia to a lower abdominal mass. All patients had perineoscrotal hypospadias with varying degrees of labioscrotal fusion. Clinically, gonads were not palpable in four cases and internally an ovotestis was observed in half of them. Karyotyping was 46, XX in all except one. The majority of the patients were reared as males. True hermaphroditism (TH) forms an important differential in the diagnostic algorithm of ambiguous genitalia especially if gonads are not palpable and congenital adrenal hyperplasia has been ruled out.

Adult↗

Childhood sporadic pheochromocytoma: clinical profile and outcome in 19 patients.

Sporadic pheochromocytoma is a rare tumor of childhood and accounts for less than 1% of cases of hypertension. We describe the presentation and outcome of 19 adolescents with sporadic pheochromocytoma seen over past 10 years at a tertiary care center in north India. The mean age (+/- SD) at presentation was 15.1 +/- 2.4 years with range from 9-18 years. The male to female ratio was 12:7. The lag time between onset of symptoms to diagnosis ranged from 1 month to 5 years with mean (+/- SD) of 1.09 +/- 1.02 years. The majority of children presented with hypertension and paroxysms. Paroxysms, characterized by the triad of headache, palpitations and sweating, was present in 13 (68%) of these patients. Twelve (63%) patients had postural fall in blood pressure, ten (53%) had abdominal pain, four (21%) had visual blurring, and three (16%) each had palpable abdominal mass and significant weight loss at presentation. Nausea and vomiting are common symptoms in children with pheochromocytoma and were present in six (32%) and three (16%) patients, respectively. Café-au-lait macule was present in only two (11%) patients. Urinary vanilyl mandelic acid (VMA) was found to be significantly high in ten (53%) patients, and urinary epinephrine and norepinephrine in eight (42%). Six (32%) patients had both VMA and urinary epinephrine and norepinephrine within normal limits and five (26%) had significant elevation of both. The tumor was localized by ultrasonography in 17 (89%) patients and by computed tomography in 18 (95%), and in one patient it was localized by 131I-MIBG scan. Sixteen (84%) patients had adrenal pheochromocytoma (including four with bilateral masses), while the remaining three (16%) had abdominal extra-adrenal pheochromocytoma originating from sympathetic ganglions. The mean (+/- SD) diameter of the tumor was 4.4 +/- 1.7 cm, ranging from 2.2-7.5 cm. Pre-operatively, hypertension was managed by phenoxybenzamine in six (32%), sustained release prazosin in 12 (63%), beta-blockers in 14 (74%), calcium channel blockers in 12 (63%), and angiotensin converting enzyme inhibitors and diuretics in only two (11%) patients. Eighteen (85%) patients underwent exploratory laparotomy for removal of the tumor. On follow-up, 13 (72%) patients became normotensive, while six (32%) patients continued to have hypertension. In conclusion, childhood pheochromocytoma is characterized by atypical symptomatology; ultrasonography is a useful modality in localizing the lesions in the majority of patients; surgery is rewarding in most patients.

3-Iodobenzylguanidine↗

Segmental dilatation of ileum: an unusual cause of severe malnutrition.

Segmental dilatation of the ileum causing blind loop syndrome was seen in a 6-year-old boy presenting with nonspecific symptoms and severe malnutrition. Barium meal was characteristic. Resection of the affected segment and an end-to-end anastomosis was curative and restored normal bowel function. Clinicians' awareness of this entity would help in appropriately managing such cases.

Child↗

Cytopathology of uncommon malignant renal neoplasms in the pediatric age group.

Malignant renal neoplasms are common solid tumors in pediatric oncology practice. These include the common Wilms' tumor/nephroblastoma and the uncommon neoplasms such as clear-cell sarcoma of the kidney (CCSK), rhabdoid tumor, renal-cell carcinoma, and others. The aim of this study was to describe in detail the cytopathological features of the histopathologically proven uncommon pediatric renal tumors. Aspirates from Wilms' tumor, which are mesenchyme predominant, show clusters of spindle cells associated with the matrix material. Evidence of rhabdomyoblastic differentiation may be present. CCSK, classic subtype, is characterized by round to oval cells arranged perivascularly and also in sheets and clusters intimately associated with a metachromatic matrix mucopolysaccharide material better appreciated in May-Grunwald-Giemsa (MGG)-stained smears. The cells also have more abundant cytoplasm and may show nuclear grooves. Spindle-cell pattern of CCSK is difficult to diagnose on aspiration cytology. Renal-cell carcinoma of childhood shows similar cytological features as its adult counterpart. Rhabdoid tumor of the kidney is characterized by a monomorphic population of cells with abundant cytoplasm, eccentric nuclei with prominent nucleoli. Intrarenal yolk sac tumor is a rare neoplasm and shows severely pleomorphic cells on aspiration. Awareness of these entities is important for the practicing cytopathologist. Further, non-Wilms' renal malignant neoplasms must be distinguished from the common Wilms' tumor so that appropriate chemotherapy protocols may be instituted in cases where the tumor is in an advanced stage of malignancy.

Adolescent↗

Esophageal atresia.

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Esophageal Atresia↗

Current status of fetal surgery.

The allure of fetal surgery is the possibility of interrupting in utero progression of an otherwise treatable condition. In spite of advances in prenatal diagnosis and refinements in surgical techniques, this field has not yet got off the ground because the risks to the mother and fetus, during and after the procedure far outweigh the benefits, and the infrastructure required to support such activity is prohibitively expensive. The various surgical conditions in which fetal surgery has been attempted and the present status of this specialty are discussed.

Cystic Adenomatoid Malformation of Lung, Congenita↗

Extrahepatic portal hypertension: quality of life and somatic growth after surgery.

AIM: This study was carried out in order to determine whether children with extrahepatic portal hypertension show any improvement in growth parameters and quality of life after elective surgery. PATIENTS AND METHODS: All patients with extrahepatic portal hypertension who underwent surgery between April 1999 and March 2002 were studied prospectively. Height and weight before and after surgery were converted into Z scores for comparison. The quality of life was assessed by changes in scholastic ability, physical activity, social interaction, and economic effects on the family. RESULTS: At the end of a minimum follow-up period of 1 year, of the 30 children, 50 % and 76 % showed improvement in weight and height Z scores, respectively, compared to their scores while on medical management. Z scores below - 2 SD for weight and height were seen in 57 % and 37 % of patients prior to surgery, and only in 40 % and 20 %, respectively at the end of follow-up, which ranged from 1 - 4 years. The improvement in height and weight was more in those who had undergone splenectomy with either devascularization or central splenorenal shunt compared to those who had undergone side-to-side lienorenal shunt without splenectomy. The children, who had dropped out of school while on medical management, either rejoined school or attended vocational courses after surgery. There was an improvement in school performance and physical activity in 85 % of the children. Improvement in personality was seen in 73 % of the affected patients. The cost incurred for surgery was one-third of the amount spent while on medical management. CONCLUSIONS: Growth parameters improved significantly in children after surgical intervention for portal hypertension. Overall improvement in scholastic abilities, physical activity, and social interaction was noted in a majority of the patients. As a one-time procedure in a developing country, surgery is also more cost effective.

Adolescent↗

Pseudoexstrophy with epispadias.

The exstrophy variants are uncommon anomalies. The variants of the exstrophy complex have all the stigmata of the classical exstrophy such as divergent recti, widened symphysis pubis, and low-set umbilicus; however, the urinary tract is intact to a varying degree. Pseudoexstrophy, an exstrophy variant, is very rarely associated with epispadias. We report an unusual case of pseudoexstrophy with epispadias, in whom the intact bladder was initially covered by a mucous membrane which later epithelialized. The epispadias was repaired using a penile disassembly technique with posterior and ventral placement of bladder neck and urethra. Although the urethral meatus was hypospadiac, the child had achieved dry intervals with occasional stress incontinence.

Bladder Exstrophy↗

Extrahepatic portal hypertension in children: observations on three surgical procedures.

This paper presents a comparative prospective study of three modalities of surgical treatment for extrahepatic portal hypertension in children: central splenorenal shunt after splenectomy (CSS), side-to-side lienorenal shunt (SSLR) without splenectomy, and splenectomy and gastroesophageal devascularization (SGD). In an 18-month period, 27 procedures were performed: 10 CSS, 10 SSLR, and seven SGD. The outcomes were evaluated by fall in portal pressures, hematological parameters, shunt patency, splenic regression, and disappearance of esophageal varices. All three procedures were comparable in the fall of portal pressure after surgery. The average blood loss and operating time were statistically significant in favor of SSLR compared with CSS. At 3-month follow-up, shunt patency was confirmed by duplex Doppler study in all the patients in the SSLR group and in nine out of 10 patients in the CSS group. In the CSS and SGD groups, hypersplenism resolved in all the patients. In the SSLR group, blood counts improved in only five out of eight affected children. No patient re-bled during a follow-up of 3-5 years. There were no cases of hepatic encephalopathy or overwhelming postsplenectomy sepsis. In conclusion, CSS is useful when there is a large spleen, severe hypersplenism, and a shuntable splenic vein. SSLR is suitable when there is only mild splenomegaly, mild hypersplenism, and a shuntable splenic vein. Splenectomy and devascularization is the choice when there is no shuntable splenic vein.

Adolescent↗

Risk factors in the primary management of anorectal malformations in Northern India.

The purpose of this study was to determine the major factors influencing the survival of babies with anorectal malformation (ARM) during the primary management in the neonatal period in a tertiary referral center. The outcome of 125 neonates with ARM, treated in a single hospital during a 2-year period, was analyzed retrospectively with particular reference to birth weight, time of arrival, and the type of ARM, including pouch colon and major associated anomalies, to see whether any of them had a significant effect on initial survival. High ARM (HARM) was present in 75, low ARM (LARM) in 36, and pouch colon in 14 babies. There were a total of 28 deaths (22%). In babies with isolated HARM weighing >2.5 kg and brought to the hospital within the first 48 h of life, the survival was 100%, which dropped to 80% when brought later than 48 h. Of the babies with birth weight <2.5 kg and isolated HARM but who were brought to the hospital within the first 48 h of life, 80% survived, whereas of those reaching the hospital after 48 h, only 55% survived. Two babies with pouch colon and two of the four babies with LARM and associated anomalies died. In the HARM group, of the 31 babies with major associated anomalies, only 37% with birth weight <2.5 kg and 58% with birth weight >2.5 kg survived. Low birth weight, major associated anomalies, and delay in referral were the three important factors that influenced the outcomes of babies with ARM during the neonatal period.

Anal Canal↗

Oesophageal atresia and biliary atresia: a rare association.

Oesophageal atresia is known to be associated with cardiovascular, gastrointestinal, genitourinary and skeletal anomalies. Its association with biliary atresia is very rare and only a few cases have been reported in the literature. Although biliary atresia could be part of a multiple anomaly syndrome, its isolated occurrence is even rare. We present a case of oesophageal atresia associated with biliary atresia.

Abnormalities, Multiple↗