Significance of Chilaiditi sign and Chilaiditi syndrome.

Chilaiditi sign and syndrome are uncommon conditions and often misdiagnosed. They are clinically significant, however, because they can result in a range of complications, including bowel volvulus, perforation and obstruction. When patients are symptomatic, treatment is usually conservative and surgery is rarely indicated unless there is a suspicion of ischaemia, or if conservative management does not resolve other signs and symptoms. This article describes Chilaiditi sign and syndrome, and presents four case studies to illustrate the relevant signs and symptoms.

Contrast Intravasation During Hysterosalpingography.

Hysterosalpingography is an imaging method to evaluate the endometrial and uterine morphology and fallopian tube patency. Contrast intravasation implies backflow of injected contrast into the adjoining vessels mostly the veins and may be related to factors altering endometrial vascularity and permeability. Radiologists and gynaecologists should be well acquainted with the technique of hysterosalpingography, its interpretation, and intravasation of contrast agents for safer procedure and to minimize the associated complications.

Iatrogenic watering-can perineum and osteomyelitis of pubic ramus as a complication of post-urethral calculus removal UTI.

We present a case of a 55-year-old male with a history of urethroscopic calculus removal who later developed urinary tract infection (UTI), complicated by periurethral abscess formation with osteomyelitis of the inferior pubic ramus and a urethrocutaneous fistula after surgical drainage of the abscess. UTI with periurethral abscess and urethrocutaneous fistula (watering-can perineum) is a rare complication of UTI. A periurethral abscess with pubic osteomyelitis has not been previously reported.

A Classic Case of Tuberous Sclerosis with Multisystem Involvement Including Giant Bilateral Renal Angiomyolipomas Presenting as Massive Hematuria.

BACKGROUND: Tuberous Sclerosis (TSC) also known as Bourneville disease is a neurocutaneous syndrome having an autosomal dominant inheritance pattern, though the condition has a high rate of spontaneous mutation. It is the second most common neurocutaneous syndrome after neurofibromatosis. This disease demonstrates a widespread potential for hamartomatous growths in multiple organ systems. CASE REPORT: We report a case of a 36-year-old female with TSC presenting as massive hematuria with underlying giant bilateral renal angiomyolipomas (AML) with estimated total tumor burden of more than 8 kg which is to the best of our knowledge the highest ever reported. The patient also had lymphangioleiomyomatosis and lesions in the brain, skin, teeth and bones. CONCLUSIONS: TSC has a wide variety of clinical and radiologic manifestations. It should be suspected when some of the common radiological manifestations are found, including CNS involvement, renal and hepatic AMLs and LAM, even if clinical signs are not obvious. Renal AMLs in setting of TSC may reach giant proportions and may present with massive hematuria.

Congenital Epulis: A Rare Benign Jaw Tumour in a 2-Day-Old Male Baby.

BACKGROUND: Congenital epulis is a rare benign jaw tumor. It is a reactive or degenerative lesion having a mesenchymal origin; presenting as an obvious mass arising from the gingival mucosa of the maxilla or mandible, presenting in neonates. Its etiology, histopathogenesis and natural history are still not well established. It is seen usually in the female gender and mostly involves the maxillary alveolar ridge. MATERIAL/MEHODS: We report a case of a 2.7 kg male baby born with growth on his mandibular ridge which was excised and was proved to be epulis on histopathology. RESULTS: Congenital epulis is often misdiagnosed before surgery because of its rarity and a lack of awareness of the condition by clinicians. It is important for the attending pediatricians, pediatric surgeon to be aware of this rare but benign congenital tumor. CONCLUSIONS: Congenital epulis is often misdiagnosed before surgery because of its rarity and a lack of awareness of the condition by clinicians. As the clinical presentation of this congenital tumor can be distressing due to its size and aggressive appearance, it is important for the attending pediatricians, pediatric surgeon to be aware of this rare but benign congenital tumor.

Antenatal Diagnosis of Jeune Syndrome (Asphyxiating Thoracic Dysplasia) with Micromelia and Facial Dysmorphism on Second-Trimester Ultrasound.

BACKGROUND: Jeune syndrome is a rare congenital malformation with a reported incidence of 1 in 100,000-130,000 live births. Thoracic hypoplasia is the most striking abnormality of this disorder. Here we report a case of Jeune syndrome with marked thoracic hypoplasia, micromelia and facial dysmorphism, which was diagnosed on a second-trimester antenatal real-time three-dimensional ultrasound. CASE REPORT: A 24-year-old primigravida came for routine anomaly scan at 19 weeks of gestation. Transabdominal grey scale and real time 3D ultrasound (US) was done with GE Logiq P5 with curvilinear array transducers (4C and 4D3C-L). US findings were consistent with the diagnosis of Jeune syndrome (Asphyxiating thoracic dysplasia). CONCLUSIONS: Jeune syndrome is an extremely rare congenital disorder with a spectrum of abnormalities of which thoracic hypoplasia is the most striking. It can be diagnosed on early antenatal US by its characteristic skeletal and morphological features which can guide further management of pregnancy in form of termination or preparation for surgical correction of the deformity.

Membranous IVC Obstruction Presenting with Antegrade/Retrograde Respiratory Flow in the Intrahepatic Segment in Doppler Imaging and Prostatic and Urethral Congestion.

BACKGROUND: Obstruction of the inferior vena cava (IVC) is infrequent, membranous obstruction of the IVC (MOIVC) being one of its rare causes. Early diagnosis is important, as it can lead to hepatic congestion, cirrhosis and Budd-Chiari syndrome (BCS) and can predispose to development of hepatocellular carcinoma (HCC) in severe cases. CASE REPORT: We report a case of membranous IVC obstruction at the junction of hepatic and suprahepatic segments in a young male with extensive collateralization and venous aneurysms. Unique findings involved antegrade and retrograde flow during respiration in the upper part of intrahepatic IVC proximal to a large collateral vein as well as prostatic and urethral congestion leading to intermittent urinary hesitancy, which have not yet been described in such cases. CONCLUSIONS: MOIVC is a rare cause of IVC obstruction with typical radiological features. Early diagnosis and management is required due to risk of cirrhosis and HCC. Antegrade and retrograde flow may be seen in incomplete MOIVC above the level of a large collateral vein and it may lead to prostatic and urethral congestion.

Congestive Myelopathy due to Intradural Spinal AVM Supplied by Artery of Adamkiewicz: Case Report with Brief Literature Review and Analysis of the Foix-Alajouanine Syndrome Definition.

BACKGROUND: Spinal arteriovenous malformations (AVMs) can lead to development of congestive myelopathy (Foix-Alajouanine syndrome). Spinal AVMs are rare and so is this syndrome. Diagnosis is often missed due to its rarity and confusing definitions of the Foix-Alajouanine syndrome. CASE REPORT: We report a case of a 47-year-old male patient suffering from this rare syndrome with an AVM arising from the artery of Adamkiewicz, which is another rarity. Our patient was treated by embolization of the lesion with 20% glue, after which he showed mild improvement of symptoms. We also present a brief review of literature on spinal AVMs and elucidate the evolution of the term Foix-Alajouanine syndrome. CONCLUSIONS: Use of the term "Foix-Alajouanine syndrome" should be restricted to patients with progressive subacute to chronic neurological symptoms due to congestive myelopathy caused by intradural spinal AVMs. CT angiography should supplement DSA as preliminary Imaging modality. Patients may be treated with surgery or endovascular procedures.

Torsion of the greater omentum secondary to omental lymphangioma in a child: a case report.

BACKGROUND: Omental cyst and omental torsion both are uncommon but important causes of acute abdomen with a difficult clinical diagnosis due to nonspecific features. Here we report a case of an eight year old child with acute abdominal pain referred for USG and CT scan which revealed two cysts in greater omentum leading to secondary omental torsion. CASE REPORT: An eight year old male child presented to casualty with severe pain abdomen since 1 day. There was no history of vomiting or altered bowel habits. The patient was febrile with tachycardia on arrival. On examination rigidity and tenderness all over abdomen were present. Serum amylase was within normal range. USG and CECT abdomen were done subsequently. USG showed two well defined cystic lesions in lower abdomen with presence of some internal echogenic debris and calcified foci in their dependent part. There was also presence of omentum with a whirl of blood vessels seen along anterior abdominal wall leading to these lesions suggesting torsion. On colour Doppler the presence of blood flow within the whirl of vessels was seen. Mild amount of free fluid was also seen in the peritoneal cavity. On CECT abdomen the findings of omental cysts and torsion of greater omentum with free fluid in abdomen were confirmed. The cysts measured 60×55 and 65×55mm on CT. The patient was taken for an emergency laparotomy for indication of acute generalized peritonitis. Two large omental cysts were found in the pelvic cavity along with torsed greater omentum along with 150 ml of hemorrhagic fluid in peritoneal cavity. The cysts and twisted necrotic part of the greater omentum were excised at surgery. No postoperative complications were observed. Histopathologic examination was suggestive of lymphangioma of omentum. CONCLUSIONS: Lymphangioma of the omentum is an not very uncommon however acute presentation with omental torsion and infarction is an unusual entity. Optimal utilization of preoperative imaging with USG, Doppler and contrast enhanced CT scan can provide correct diagnosis.

Giant Cell Tumor of Bone in Skeletally Immature Patients - A Clinical Perspective.

INTRODUCTION: Giant cell tumors of skeleton are very rare in pediatric and adolescent population. Here we report two cases-one a fifteen year old child with swelling distal humerus and another a case of a thirteen year old child with pain and swelling proximal tibia. CASE REPORT: A fifteen year old child presented to department of orthopedics of our institute with complaint of difficulty in moving upper limb and swelling distal humerus. Another patient who was a 13 years old male had painful ambulation and swelling in upper tibia. MRI followed by core needle biopsy was done in both the patients confirming the mass to be giant cell tumor which is quite rare in this age group. First patient was managed by wide excision and total elbow replacement and second one by curettage, cementation and augmentation with plate-screw construct. CONCLUSION: Giant cell tumour of skeleton is highly uncommon in pediatric age group. It should be considered as one of the differential diagnosis of epiphyseo metaphyseal lesions in pediatric population in spite of its rarity.

Postpartum pituitary apoplexy with isolated oculomotor nerve palsy: A rare medical emergency.

Pituitary apoplexy is a clinical syndrome characterized by sudden onset headache, visual deficits, ophthalmoplegia, altered mental status, and hormonal dysfunction due to an expanding mass within the sella turcica resulting from hemorrhage or infarction of pituitary gland. We report a case of pituitary apoplexy that developed in postpartum period following postpartum hemorrhage and presented with isolated third cranial nerve palsy.