A Novel cause of Massive Hepatosplenomegaly with Fibrosis in two children: Transient Infantile Hypertriglyceridemia.

Background: Transient infantile hypertriglyceridemia (TIH) is a syndrome of hypertriglyceridemia, fatty liver, and deranged liver functions with progression to fibrosis and cirrhosis. It is an autosomal recessive disorder caused by mutations in Glycerol-3-phosphate dehydrogenase 1 gene present on Chromosome 12q12-q13, and has been reported in Israeli Arab families with high consanguinity. TIH is suspected by high serum triglyceride levels and steatosis on liver biopsy; however, diagnosis is confirmed on clinical exome sequencing. Clinical description: We present two cases of TIH belonging to the indigenous Hindu, hilly population of Himachal Pradesh in North India with no history of either consanguinity or family history. Management and outcome: The parents of both the cases were counselled regarding the disease and importance of growth and lipid level monitoring. Conclusion: Though TIH is an extremely rare entity, awareness about it is required as it is a contributor to non-alcoholic fatty liver disease (NAFLD) in children. Any child presenting with hepatomegaly and elevated fasting triglyceride levels should be further investigated for TIH.

Clinician's dilemma: Naproxen-induced liver injury.

Naproxen is a widely used nonsteroidal anti-inflammatory drug (NSAID) in pediatric population, used for mild-to-moderate pains, arthritis, and other immune-mediated disorders. It rarely causes clinically apparent liver injury in the adult population taking high doses of the drug over a prolonged period and is reported even rarer in pediatric population. We present a case of drug-induced liver injury (DILI) in a 13-year-old girl taking naproxen in therapeutic doses for juvenile rheumatoid arthritis. There was a complete recovery of liver function following discontinuation of naproxen therapy.

Signs and lines in lung ultrasound.

Point-of-care ultrasound has become firmly established in acute and critical care settings, and is now increasingly being used as an important tool in the assessment of the lungs. In this article, we briefly describe the technique of lung ultrasound and the various lines and signs commonly encountered during sonography of the lung, namely the normally visualised A- and T-lines and the bat sign, sliding sign (power slide sign on colour Doppler), sea-shore sign, curtain sign, and the lung pulse. We have also described signs seen in various pathological conditions like B-lines seen in cases of increased lung density; the quad sign, sinusoid sign, thoracic spine sign, plankton sign and the jelly fish sign seen in pleural effusion; the stratosphere sign and the lung point sign seen in pneumothorax; the shred/fractal sign and tissue-like sign in consolidation, and the double lung point sign seen in transient tachypnoea of the newborn. With adequate and appropriate training, lung ultrasound can be effectively utilised as a point-of-care investigation.

"Crown of Death"; Corona Mortis, a Common Vascular Variant in Pelvis: Identification at Routine 64-Slice CT-Angiography.

OBJECTIVE: To establish the incidence of arterial corona mortis variant in angiographic studies being performed using a 64 slice CT scan machine in a series of patients. METHODS: This was a prospective cross-sectional study including 100 consecutive patients undergoing routine clinically indicated, standard protocol, CT-angiography for the abdominal aorta and/or lower limbs using a 64 slice CT scanner. Patients having severe arterial insufficiency (Grade 4 stenosis on CT angiography), pelvic infections and tumours, patients with past pelvic trauma and those who had previous pelvic surgery were excluded from the study. In total 200 hemi-pelvises were evaluated for the presence or absence of corona morti. RESULTS: Overall, we included 100 patients in this series including 67 men and 33 women with mean age of 40.1±2.3 (ranging from 22-74) years. The arterial variant was identified on thin, 0.625-mm-thick images in 24 out of 100 patients studied (unilateral in 20 patients and bilateral in 4 patients; 28 out of 200 hemipelvises evaluated, having an incidence of 14%). We found that the distance of corona mortis artery from the symphysis was significantly greater for women compared to men, both on right (p=0.034) and left sides (p=0.046). CONCLUSION: Corona mortis may be prospectively identified at contrast-enhanced multidetector CT especially in pelvic trauma patients and help guide subsequent endovascular embolization or surgical interventions.

Classic Imaging Features of L-2-Hydroxyglutaric Aciduria in Young Adult Presenting as Seizures Associated with Fever.

L-2 Hydroxyglutaric aciduria is a rare metabolic disorder which is autosomal recessive in inheritance. It is characterised by the increased urinary excretion of L-2 hydroxyglutaric acid and the diagnosis is based on the increased levels of the L-2 hydroxy glutaric acid in the urine, serum & CSF. This is a neurometabolic disorder which is associated with slowly progressive psychomotor delay since childhood. We report a case of an 18 -year old female who presented at the emergency department with seizures, fever and on imaging show classic features.

Study of ultrasound-guided needle aspiration and catheter drainage in the management of liver abscesses.

PURPOSE: To evaluate and compare the efficacy of intermittent needle aspiration and continuous catheter drainage in ultrasound-guided management of liver abscesses. METHODS: This was a prospective, randomised study conducted on 100 patients (88 males and 12 females; age range 22-74 years) with liver abscess(es) and having abscess size more than 5 cm, divided into two groups: Percutaneous needle aspiration (PNA) (n = 50) and percutaneous catheter drainage (PCD) (n = 50). Criteria of exclusion were: rupture of abscess before intervention; prior intervention; uncorrectable coagulopathy; concomitant biliary tract malignancy. In the PNA group, pus was aspirated by an 18-gauge needle using freehand technique and the number of aspirations was limited to two. Failure of abscess size to decline below 50% of the original diameter or of clinical improvement after second aspiration was considered as failure of aspiration. In the PCD group, drainage was done by 12-French catheters using Seldinger technique. Drainage was considered as failure if abscess cavity did not resolve and laparotomy was needed to evacuate the pus cavity. RESULT: The success rate in the PNA group was 88% and 92% in the PCD group; however, this difference was statistically not significant, suggesting that both are equally efficacious. The total duration of hospital stay (mean 6.8 days [PNA] vs 10.5 days [PCD]; p value: 0.011) and the average duration between intervention and discharge (5.9 days [PNA] vs 10.2 days [PCD]; p value:0.026) were significantly less in the PNA group. One major complication was seen in our study: peritonitis due to peri-catheter leak in PCD group. CONCLUSION: Both procedures are equally efficacious in the management of liver abscesses; however in view of less duration of hospital stay, patient safety and comfort, procedure simplicity, and the reduced cost, needle aspiration should be used as the first-line procedure in the treatment of liver abscess (even in abscesses more than 5 cm). Catheter drainage should be reserved for cases that do not respond to a second attempt of aspiration.

To Compare Diagnostic Ability of Contrast-Enhanced Three-Dimensional T1-SPACE with Three-Dimensional Fluid-Attenuated Inversion Recovery and Three-Dimensional T1-Magnetization Prepared Rapid Gradient Echo Magnetic Resonance Sequences in Patients of Meningitis.

AIMS: The aim of this study is to compare postcontrast three-dimensional (3D) T1-Sampling perfection with application-optimized contrasts by using different flip angle evolutions, 3D fluid-attenuated inversion recovery (FLAIR), and 3D T1-magnetization prepared rapid gradient echo (MPRAGE) sequences in patients of meningitis. SETTINGS AND DESIGN: A hospital-based cross-sectional study done in the Department of Radiodiagnosis, IGMC Shimla for a period of 1 year from June 1, 2016, to May 30, 2017. SUBJECTS AND METHODS: A total of 30 patients suspected of meningitis underwent magnetic resonance imaging evaluation with postcontrast 3D T1-MPRAGE, 3D T1-SPACE, and 3D FLAIR sequences. The abnormal leptomeningeal enhancement was noted by two radiologists together on these sequences and scores were given to the abnormal leptomeningeal enhancement. STATISTICAL ANALYSIS USED: The sensitivity of 3D T1-SPACE, 3D T1-MPRAGE, and 3D FLAIR was calculated and compared. The level of agreement between these sequences was assessed by kappa coefficient. P < 0.05 was taken as statistically significant. RESULTS: 3D T1-SPACE shows superiority in meningeal enhancement along basal cisterns, Sylvian fissure and along cerebral convexities. It is also found to be better in delineating parenchymal lesions. 3D FLAIR failed to demonstrate enhancement along cerebral convexities however found to be better than 3D T1-MPRAGE in delineating enhancement along basal cisterns and Sylvian fissures. 3D T1-MPRAGE has shown subtle enhancement in basal cisterns, Sylvian fissure and along cerebral convexities. 3D T1-SPACE, 3D FLAIR, and 3D T1-MPRAGE has sensitivity of 91.67%, 66.67%, and 54.17%, respectively. CONCLUSION: Postcontrast 3D T1-SPACE sequence is an excellent tool than postcontrast 3D T1-MPRAGE and 3D FLAIR sequences in the evaluation of meningeal enhancement and depiction of additional lesions in brain parenchyma.

Isolated Fracture of Lateral Pterygoid Plate by Penetrating Foreign Body - A Rarity Indeed.

BACKGROUND: Fractures of mid-face were first described by Le Fort and are classified into three categories - Le Fort I, Le Fort II and Le Fort III. The pterygoid processes of the sphenoid bone are fractured in all the three categories of Le Fort fractures as the sphenoid bone connects the cranium vault to the facial bones. Fractures of the pterygoid processes without associated Le Fort fractures are rare and are usually associated with fractures of the mandible, temporal bone or other facial bones. An isolated fracture of pterygoid plates without associated Le Fort fractures or fractures of other mid-face bones are exceedingly rare. CASE REPORT: We present a case of an isolated fracture of the right lateral pterygoid plate by a penetrating foreign body (wooden twig) in an adult male who presented with discharging sinus in the oropharynx. The presence of the foreign body was confirmed on computed tomography and was removed under general anesthesia via submandibular incision. The patient had an uneventful postoperative hospital stay and was asymptomatic on a follow-up five months later. CONCLUSIONS: This article emphasizes the fact that pterygoid plates may be fractured without an associated Le Fort fracture or a fracture of the mandible. This is the first case of an isolated pterygoid plate fracture in the literature.

Spontaneous Cholecysto-Cutaneous Fistula Draining Through an Old Abdominal Surgical Scar.

BACKGROUND: Though cholelithiasis and cholecystitis are common clinical problems, spontaneous cholecysto-cutaneous fistula is a rare complication of cholelithiasis in present-day practice. Very few cases have been reported in literature after 1950s. CASE REPORT: A 60-year-old male presented with pain and discharging sinus in the upper abdomen. Ultrasonography and computed tomography confirmed the presence of perforation of the gall bladder and cholecysto-cutaneous fistula. External opening of the fistula was overlying an old surgical scar. Less than five cases of spontaneous cholecysto-cutaneous fistula draining through an old surgical scar have been reported in literature. Initially, the patient was managed conservatively, followed by elective open cholecystectomy and excision of the fistula tract. CONCLUSIONS: Though very rare in the present era of advanced medical care, cholecysto-cutaneous fistula as a potential diagnosis should be kept in mind in patients presenting with acute upper abdomen pain and discharging sinus. High degree of suspicion is required for early diagnosis and treatment of this condition.

Giant Atretic Occipital Lipoencephalocele in an Adult with Bony Outgrowth.

BACKGROUND: We present unique case of a giant extracranial atretic occipital lipoencephalocele in an adult patient with new bone formation within it which was not associated with any developmental malformation of brain. Resection of the lipoencephalocele was performed for esthetic reasons. CASE REPORT: 18 year old female patient presented to the surgery OPD with complains of a large mass in the occipital region present since birth. It was of size of a betel nut at the time of birth and gradually increased in size over a long period of time. It was painless and not associated with any other constitutional symptoms. On examination the rounded fluctuant mass was present in the midline in occipital region covered with alopecic skin with dimpling in the overlying skin. On MRI there was mass showing both T1 and T2 hyperintense signal area suggestive of fat component. Herniation of meninges and atretic brain parenchyma was also seen through a defect in the occipital bone in the midline. There was a Y shaped bony outgrowth seen arising from occipital bone into the mass which was quite unusual in association with an atretic lipoencephalocele. CONCLUSIONS: A large lipoencephalocele with bony outgrowth in an adult patient is a rare presentation of atreic occipital encephalocele.

Complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) in a young woman-a rare variant of Herlyn-Werner-Wunderlich syndrome.

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is an unusual and special type of Müllerian duct anomaly. Patients usually present in adolescence, soon after menarche, with pain and pelvic mass and rarely with infertility in adulthood. Majority of the cases of OHVIRA have been reported in association with uterus didelphys and the presentation of uterus didelphys with OHVIRA is known as Herlyn-Werner-Wunderlich syndrome. A complete septate uterus with OHVIRA is exceedingly unusual. Less than 30 cases of complete septate uterus with OHVIRA have been reported to date, to the best of our knowledge. We present a rare case of incidentally detected complete septate uterus with OHVIRA in a young woman who presented with acute pain in the right iliac fossa owing to acute appendicitis.

Neglected vaginal foreign body leading to vaginolith, vesicovaginal fistula and vesical calculus formation in an adolescent girl.

Vaginal foreign body insertion is not an uncommon clinical entity; however, long-standing neglected vaginal foreign body causing vaginolith, vesicovaginal fistula and vesical calculus formation is unusual. We present a case of a neglected vaginal foreign body (plastic cap of a nail colour) leading to vesicovaginal fistula, vaginolith and vesical calculus formation in a 12-year-old female child presenting with continuous dribbling of urine per vagina. Diagnosis was confirmed on ultrasonography, non-contrast CT scan and MRI of the pelvis. The MRI demonstrated the exact size and site of the urinary bladder wall defect, besides the foreign body and the vesical calculus. The foreign body along with vaginolith and the vesical calculus were removed via suprapubic approach under general anaesthesia; the fistula was repaired by suturing the urinary bladder and vagina wall defect.

Sonographic prediction of scar dehiscence in women with previous cesarean section.

PURPOSE: To estimate the risk of uterine dehiscence/rupture in women with previous cesarean section (CS) by comparing the thickness of lower uterine segment (LUS) and myometrium with trans-abdominal (TAS) and trans-vaginal sonography (TVS). METHOD: In this case-control study, in 100 pregnant women posted for elective CS (with or without previous CS; group 1 and group 2 respectively), the thickness of LUS and myometrium was measured sonographically (TAS and TVS). Intra-operatively, LUS was graded (grades I-IV), and its thickness was measured with calipers. The primary outcome of the study was correlation between echographic measurements (TAS and TVS) and features of LUS (grades I-IV) at the time of CS. Secondary outcomes were correlation between myometrial thickness, number of previous CS, and inter-delivery interval with LUS (grades I-IV). RESULTS: Sonographic measurements of LUS and myometrium were significantly different between the two groups (both TAS and TVS p value = 0.000 each). However, the number of previous CS (p = 0.440) and inter-delivery interval (p = 0.062) had no statistically significant correlation with thickness of LUS. CONCLUSIONS: Sonographic evaluation of LUS scar and myometrial thickness (both with TAS and TVS) is a safe, reliable, and non-invasive method for predicting the risk of scar dehiscence/rupture. Specific guidelines for TOLAC, after sonographic assessment of women with previous CS, are need of the hour.

Iatrogenic uterine perforation with abdominal extrusion of fetal parts: a rare radiological diagnosis.

BACKGROUND: Failure to detect uterine perforation during surgical abortion may result in adverse patient outcome besides having medicolegal implications. This rare case of uterine perforation was diagnosed seven days after abortion and underscores the importance of remaining vigilant for this complication during and after the procedure. CASE: A female underwent surgical abortion at sixteen weeks gestation and was discharged after the procedure, assuming no complication. She presented with abdominal pain seven days after the event. Ultrasound and CT revealed uterine perforation with abdominal expulsion of fetal parts. CONCLUSION: A patient complaining of abdominal pain following recent abortion related instrumentation should alert the clinician regarding possibility of perforation. Secondary signs on ultrasound may reveal the diagnosis even if rent is not identified. CT is valuable in emergent situations.