Two Cases of Interrupted Inferior Vena Cava with Azygos / Hemiazygos Continuation.

Interrupted inferior vena cava (IVC) is a rare disease, occurring either in isolation or in association with asplenia or polysplenia syndromes. Infrahepatic part of the IVC is absent representing the failure of fusion of the vitelline and subcardinal embryological portions of the IVC. It is replaced by an enlarged azygos or hemiazygos vein continuing into the thorax, either into the superior vena cava or into the brachiocephalic veins. We present two cases of interrupted IVC, one occurring in isolation with hemiazygos continuation and discovered incidentally, and the second one is a child with azygos continuation, associated with polysplenia syndrome. Key Words: Inferior vena cava, Polysplenia, Azygous vein, Hemiazygos vein.

Hereditary Spastic Paraplegia: Role of MRI.

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Hyperglycemia Presenting with Hemichorea-Hemiballismus and T-1 Hyperintensity on MRI Brain.

Hemichorea-hemiballismus, secondary to hyperglycemia, is a rare but easily treatable condition that is usually associated with type II diabetes mellitus. This is a case of a 68- year lady, with long-standing, poorly controlled diabetes mellitus, who presented with disabling right-sided hemichorea-hemiballismus. The T1-weighted magnetic resonance imaging revealed hyperintensity in the basal ganglia. The abnormal movements subsided within a few days after achieving euglycaemia with insulin therapy. This case highlights the importance of treatment of hyperglycaemia in a diabetic patient presenting with acute or sub-acute abnormal movement disorder. Key Words: Hemichorea, Hemiballismus, Hyperglycemia, T-1 hyperintensity.

Branchio-oto-renal syndrome.

The association of branchial arch anomalies (branchial cysts, branchial fistulas), hearing loss and renal anomalies constitutes the branchio-oto-renal (BOR) syndrome also known as Melnick Fraser syndrome. We present a case of this rare disorder in a girl child who presented with profound deafness, preauricular pits, branchial sinuses and renal hypoplasia.

Grebe syndrome: a rare association with congenital heart disease.

Grebe syndrome is a very rare form of short-limbed dwarfism. It is a genetic condition, passed by autosomal recessive inheritance. It is characterized by marked acromesomelic shortening of all the four limbs. There are no other associated anomalies. The affected baby has normal intelligence and normal life span. We present here a case of Grebe syndrome along with congenital heart disease.

Primary synovial osteochondromatosis of the surapatellar pouch of knee. Correlation of imaging features with surgical findings.

A 31 years old female presented with swelling and pain above the right knee for three years. On examination, there was a tender swelling over the right knee more pronounced over the suprapatellar region. Plain X-ray, US, CT scan and MRI of the knee were suggestive of Primary synovial osteochondromatosis (PSC) of the suprapatellar pouch. Patient underwent total synovectomy and the diagnosis of synovial osteochondromatosis was confirmed histopathologically. Recognizing the imaging appearances of PSC is important to improve patient management.

Pictorial review: Imaging features of unusual patterns and complications of hydatid disease.

Hydatid disease is a worldwide zoonosis produced by the larval stage of the Echinococcus tapeworm. We demonstrate rare locations and unusual complications of this entity during past 6 years. Rare locations during our observation included lumbar spine, sacral spine, spleen, ovary, abdominal wall, diaphragm, pelvis and right kidney. Unusual complications included formation of bronchopulmonary fistula, complete collapse of left lung secondary to hilar location of Hydatid cyst and hydatiduria.

Rosai Dorfman disease--a rare entity report of two cases with nodal and extranodal involvement.

Rosai Dorfman Disease (RDD) also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML)is a very rare variety of reactive histiocytoses. It commonly involves cervical lymph nodes; although involvement of other lymph node regions, skin and other organ involvement can occur. It has a good prognosis so there is a need to differentiate it from other lympho proliferative disorders of poorer prognosis. We present two cases of this rare disorder illustrating its clinical spectrum; a 22 years old woman with involvement of submandibular lymph nodes, nasal septum and sub-glottic region and a 45 years old male with prominent skin involvement.

Frequency of Otitis Media with Effusion in recurrent upper respiratory tract infection in children.

OBJECTIVE: To determine the frequency of Otitis Media with Effusion (OME) in children with recurrent upper respiratory tract infections. STUDY DESIGN: A descriptive study. PLACE AND DURATION OF STUDY: Combined Military Hospital, Gujranwala, from September 2003 to September 2005. PATIENTS AND METHODS: Children of either gender suffering from more than 2 episodes of upper respiratory tract infection in the last 12 weeks were studied. The inclusion criteria were mild intermittent ear pain, fullness or "popping" of the ear drum; poor response to normal speech, inattentiveness, behavioural changes; irritability, sleep disturbances; balance problems and poor school performance. Diagnosis of OME was confirmed by pneumatic otoscopy while tuning-fork tests, tympanometry, pure tone audiometry were used as adjunct investigations. Results were described in percentage and 95% confidence interval was determined. RESULTS: Out of the 350 referred cases, pneumatic otoscopy by otolaryngologists diagnosed OME in 147 (42%, 95% CI=37.9-47.1). Out of these, 112 (76%) had retracted tympanic membrane, 25 (17%) had bulging of tympanic membrane and 10/147 (6.08%) cases showed dullness of tympanic membrane with no additional features. Tympanometry confirmed fluid behind the tympanic membrane in 145/147 (98.6%) cases. Tuning-fork tests showed negative Rinne's test in 122 (83%). Pure tone audiometry diagnosed deafness in 138/147 (93.8%) cases. CONCLUSION: OME with hearing loss is common in children having recurrent upper respiratory tract infection and nonspecific ear-related symptoms.

Klippel-Feil syndrome with situs inversus--a rare association.

Klippel-Feil Syndrome (KFS) is a congenital anomaly characterized by a defect in the formation or segmentation of the cervical vertebrae. The clinical triad consists of short neck, low posterior hairline and limited neck movement. Multiple congenital anomalies have been associated with this disease. This is a case of KFS in a young girl along with situs inversus, which is an extremely rare association. Various systemic associations occurring in this multi-system disorder are also discussed.

Multifocal osseous tuberculosis involving hand and feet.

A rare case of multifocal cystic tuberculosis of hands and feet is presented in an adolescent female. The presence of multiple lytic areas mimicked secondary metastases and biopsy remained the mainstay for final diagnosis.

Brodie's abscess--an uncommon cause of leg pain.

A rare case of Brodie's abscess of distal left tibia is presented in a child which was initially missed on clinical grounds alone. Differentiation from different bone neoplasms was done on radiological grounds. The patient was managed surgically with high dose intravenous antibiotics. Brodie's abscess is very rarely encountered in our reporting of X-rays.

Hematometrocolpos associated with common cloacal anomaly.

A rare case of hematometrocolpos is presented in a 14 years old female with known common cloacal malformation. It presented as a slowly growing tender pelvic mass. Ultrasound and CT scan of the pelvis revealed vagina and uterus filled with blood. A diagnosis of hematometrocolpos was made. Per-operatively multiple adhesions were found in the pelvis necessitating the removal of blood-filled uterus.

Klippel Trenauny Syndrome.

Klippel Trenauny Syndrome is a rare congenital syndrome characterized by port wine stain (capillary malformation), limb or hemihypertrophy and other vascular malformations. We present a case of this rare disorder in a young boy at an early stage of this disease.

Isolated malignant peripheral nerve sheath tumor of retroperitoneum.

A case of an isolated Malignant Peripheral Nerve Sheath Tumor (MPNST) of the retroperitoneum without neurofibromatosis is presented. The tumor was located deep in the retroperitoneum with metastasis to the ribs. It was surgically removed. Patient was further managed with radiotherapy and chemotherapy. MPNST in such a location is very unusual.

Mayer Rokitansky Kuster Hauser syndrome with urogenital sinus anomaly.

Mayer Rokitansky Kuster Hauser (MRKH) syndrome is a rare disorder, characterized by the congenital absence of uterus and associated renal tract anomalies. The case presented with primary amenorrhea and primary infertility, despite development of normal female secondary sexual characteristics. CT scan revealed absent uterus, a solitary left sided pelvic kidney and a vesicovaginal communication that, on cystoscopy, revealed urogenital sinus anomaly manifesting as a common channel formed due to absent anterior wall of vagina and posterior wall of urethra. The urogenital sinus anomaly in MRKH syndrome has not been reported earlier.