Prabudh Goel

Giant primary posterior mediastinal hydatid cyst in a child: report of a case and review of literature

Primary posterior mediastinal cyst is an exceptionally uncommon manifestation of hydatid disease especially in pediatric age group. We herein present the account of a giant posterior mediastinal hydatid cyst in an 8-year-old boy that was peculiar due to the absence of typical clinical and radiological features of hydatid disease. The diagnosis was established perioperatively by a combination of surgical and pathological findings. The report depicts one of the myriad presentations of hydatid disease and also emphasizes the verity that it should be considered in the differential diagnoses of any mediastinal cystic lesion even in pediatric patients.

Locally invasive pulmonary inflammatory myofibroblastic tumors in children.

Two uncommon cases of locally invasive pulmonary inflammatory myofibroblastic tumor are reported. Diagnosis was established by a prior thoracotomy and incisional biopsy. Complete excision was curative and both children remain asymptomatic at last follow up.

Pleuropulmonary blastoma with intrabronchial extension

A 3‐year‐old male presented with an episode of respiratory distress that was superimposed on a 2½ years history of recurrent chest infections. The chest radiograph and a contrast enhanced CT scan revealed a cystic cavity in the left upper lobe with an endobronchial soft tissue mass in left bronchus. Left bronchotomy and removal of the endobronchial soft tissue mass and left upper lobectomy were performed. The histology of the specimen revealed a pleuropulmonary blastoma (PPB) type II. The presentation, diagnosis, and management of this uncommon case of PPB with intrabronchial extension are described. Pediatr Blood Cancer 2010;54:1026–1028

The role of nitric oxide in portal hypertension caused by extrahepatic portal vein obstruction

Aims: Nitric oxide (NO) in peripheral venous blood has been shown to be elevated in experimental portal hypertension. This study aims to determine the serum NO levels in patients with extrahepatic portal venous hypertension (EHPVO) pre- and postoperatively and to analyze whether these can serve as a reliable and early indicator of shunt blockage or malfunction. Materials and Methods: During the period 2006–2010, 48 children with EHPVO were included in this prospective study; 40 underwent porto-systemic shunt and eight underwent splenectomy with devascularization. NO was evaluated preoperatively, 14 days after surgery, at 3 months and then 6 monthly thereafter. The median follow-up duration was 21 months. Shunt patency was confirmed with Doppler and computed tomography portography. Forty-eight age-matched children with hypospadias served as controls. Results: NO was higher in EHPVO patients as compared with controls (43.16 ± 16.27 vs. 5.76 ± 2.62 μmol/l) (P = 0.0001). There was a significant decline in the NO levels (4.64 ± 3.18 μmol/l) following shunt surgery (P = 0.0001), and it continued to remain low till the shunt was patent. A shunt block was indicated by rising NO levels in all five patients. The devascularization group also demonstrated a significant decline in the NO levels (27.06 ± 3.56 μmol/l) (P = 0.002), but it was less as compared with the shunted patients. The decline in the portal pressure after shunt surgery was found to correlate with the decline in the serum NO levels. Conclusions: The blood levels of NO can be used in the diagnosis of portal hypertension, and are useful for monitoring the patency of the shunt.

‘Mutiny on the crown’: two cases of rare cephalic malformations

The presentation and management of two bizarre congenital cephalic curiosities at the two extremes of the clinical spectrum are discussed herewith. Case 1: presented to us as a neonate with a scalp swelling mirror-imaging her head and face. The journey from clinics to wards and to the operation theatre and to her home is introspected. Case 2: presented to us as dicephalous dibrachius dipus parapagus conjoined twins. The detailed work-up of individual organ systems, the multidisciplinary approach to management and the final outcome are discussed. This is an unsolved mystery for the anatomists, paediatric surgeons, radiologists and the medical fraternity at large.

Evaluation of blood levels of nitric oxide as a means of differentiation between neonatal hepatitis and extrahepatic biliary atresia: A pilot study.

Aim: The differentiation between neonatal hepatitis (NH) and extrahepatic biliary atresia (EHBA) is not always possible despite all the currently available diagnostic modalities. In this study, an attempt has been made to evaluate the role of nitric oxide (NO) levels in the peripheral blood to differentiate between the two conditions, one requiring early surgical intervention (EHBA) and the other amenable to conservative medical management (NH). Patients and Methods: Twenty patients who presented to the pediatric surgical service, over a 2 years period, with features of neonatal cholestasis were enrolled in the study. The diagnostic workup included documentation of history and clinical examination, biochemical liver function tests, ultrasonography, hepatobiliary scintigraphy (HS), and magnetic resonance cholangio-pancreaticography (MRCP). These patients did not show excretion on HS and intrahepatic ducts on MRCP. Hence, they were subjected to mini-laparotomy and operative cholangiography (OC). The EHBA patients were treated with the Kasai′s portoenterostomy procedure, and the extrahepatic ducts were flushed with normal saline in NH patients. All patients were evaluated preoperatively for levels of NO in the peripheral blood by the Greiss reaction spectrophotometrically at 540 nm. Normal values were determined from a cohort of controls. The median (range) levels of NO in patients with EHBA and NH were compared, and the statistical significance of the difference was calculated by applying the Wilcox Rank Sum test. A P = 0.05 was considered as significant. Results: Of the 20 patients enrolled in the study, 17 patients were treated for EHBA (Group I) and the remaining 3 patients had patent ducts on OC and were thus diagnosed as NH (Group II). The mean age of the patients in Groups I and II was comparable: 2.79 ± 0.75 and 2.67 ± 0.58 months, respectively (P = 0.866). The median NO levels were significantly elevated in each of the two groups as compared to the controls (5.6 μmol/l, range 1.26-11.34 μmol/l); when compared among themselves, the NO levels were significantly higher in Group I, 64.05 μmol/l (range 24.11-89.43 μmol/l), when compared with Group II, 41.72 μmol/l (range 23.53-45.63 μmol/l) (P = 0.022). Conclusion: The serum levels of NO were found to be significantly higher in patients with EHBA as compared to those with NH. Hence, this may be a useful biochemical marker for the preoperative differentiation of EHBA from NH. However, a larger study is required for establishing the validity of the statistical significance.

Novel Presentation of Labial Synechiae: Two Urinary Streams

Labial synechiae, also referred to as labial adhesion or labial agglutination, is a disorder of the female genitalia characterized by thin, membranous adherence of the labia minora. Typically, the fusion originates from the posterior fourchette and advances toward the clitoris. Complete labial fusion may conceal the vaginal introitus completely. Partial labial fusion is also possible and may occur near the posterior fourchette.

Massive ossifying fibroma of the mandible in a child

An interesting case of large ossifying fibroma of the mandible in a child with a sickle-cell trait is reported.