Giant Fronto-parietal Encephalocele: A rare case report.

Although encephaloceles are common, but giant encephaloceles are rare with only few published short series and are mostly described in occipital location. Giant frontal encephaloceles are very rarely reported with only four published case reports in the world literature and giant encephalocele in fronto-parietal region has not been reported till date. Herein, we report a rare case of giant frontoparietal encephalocele in a six month old girl.

Unusual K-wire migration.

The authors report a case of intra-abdominal migration of a Kirschner wire from the left hip to the right lobe of the liver in a 5-year-old child. The wire was used for stabilization of the left hip after open reduction for neglected unreduced congenital dislocation of the left hip. The migrated wire was removed by laparotomy. Surprisingly, no injury was noted to any intervening abdominal structure intra-operatively. This unusual migration of a Kirschner wire into a child's liver has not been reported previously.

Infected primary intramuscular echinococcosis of thigh.

An unusual case of infected primary echinococcosis involving quadriceps muscles of the thigh and presenting as a cystic mass in a child is being reported. The diagnosis was made preoperatively with ultrasound examination and serology. After a cover of medical treatment the cyst was excised. Various clinical possibilities and management strategies are discussed.

Papillary carcinoma thyroid: FNAC diagnosis.

Thyroid carcinoma in children is currently assuming greater importance due to increase incidence in the recent times. In carcinoma thyroid, the age at the time of diagnosis is an important prognostic factor and children in particular are considered to have an excellent prognosis. We came across 3 cases of papillary carcinoma thyroid involving both the lobes and having lymph node metastasis in children, the youngest being 4 years of age at the time of diagnosis. All these cases were diagnosed on aspiration cytology.

Pancreatoblastoma (infantile pancreatic carcinoma)--a case report.

Pancreatoblastoma or infantile pancreatic carcinoma is a rare pancreatic tumor with distinct acinar and squamoid cell differentiation that generally affects infants and young children. Ultrasound and CT scan may be useful but preoperative diagnosis is often quite difficult. The outcome is generally favourable. A such case of 10 years old boy with an abdominal mass is being presented.

Aniridia and Wilm's tumor.

Aniridia (absence of iris) is a congenital, bilateral, uncommon panocular disorder. Whereas the occurrence of aniridia in the general population is 1:50000, it is present in about 1 in 70 patients with Wilm's tumor. This aniridia is sporadic and Wilm's tumor in these cases presents at an unusually early age. Aniridia was present in two cases out of 60 cases of Wilm's tumor operated at PGIMS, Rohtak. Both cases presented below two years of age. The recognition of a child with sporadic aniridia should alert to the increased risk of development of Wilm's tumor.

Perineal accessory scrotum.

A boy with perineal accessory scrotum but without any other congenital anomaly is being described. He presented with a rugose skin tag on a midline perineal mound and the diagnosis could be confirmed by the histological findings of subcutaneous smooth muscles. Other reports of perineal scrota have been reviewed and a simple classification has been proposed for such cases, based on the appearance of the accessory scrotum and the associated anomalies.

Advanced sigmoid carcinoma colon.

A 13-year-old female child diagnosed initially as having tuberculosis was subsequently found to have adenocarcinoma colon with secondaries in ovaries and omentum, and proved to be mucinous carcinoma on biopsy. Patient received chemotherapy (FAM) regimen without any relief even 2 months after surgery.

Pancreatoblastoma.

Pancreatoblastoma is an extremely rare pancreatic tumor in childhood, comprising 0.5% of pancreatic non endocrine tumors. Just over 50 cases have been reported in the literature till 1997. A 10 year-old male child presented with mass abdomen of 9 months duration. USG and CT abdomen showed a large mixed density lobulated mass in abdomen anterior to, and displacing down the portal vein and splenic veins. Exploratory laparotomy revealed a large mass, arising from body and tail of pancreas, not infiltrating the adjacent structures. Complete excision of the mass along with removal of body and tail of pancreas was done. The histopathological examination revealed pancreatoblastoma. He is well without any evidence of disease 6 months after the operation. To the best of our knowledge this is the first case of pancreatoblastoma with a large size (25 x 20 x 15 cm,) weight 2.5 kg, which was localized and where complete excision was possible. The largest size reported in the literature reviews has been around 15 cm. The literature review confirms that an aggressive surgical attack on resectable pancreatoblastoma may often be curative. The role of adjuvant chemotherapy or radiotherapy is still under discussion due to small number of patients treated as yet.

Teratomas in infancy and childhood.

A 10-year-experience with 42 cases of teratomas in paediatric age group is presented. The commonest type of teratoma was sacrococcygeal followed by ovarian and retroperitoneal teratomas. An analysis of clinical profile, malignant potential, management, prognostic factors and follow up is discussed with review of literature.

Pseudoexstrophy in a female child.

A six-year-old female patient presenting with a swelling in the infraumbilical part of the abdomen, bulging out on straining, was diagnosed to have pseudoexstrophy bladder. The urinary tract was normal. The patient had bifid clitoris. There was no other associated malformation. Surgical repair of abdominal wall defect was done successfully. A new classification of exstrophy variants is proposed.

Mesenteric leiomyoma in infancy.

Leiomyoma of the mesentery is an uncommon tumor. This has been documented in adults and children. However, there is no such case reported in an infant, which is being presented in this report.

Intrauterine intussusception--a cause for ileal atresia.

A case of ileal atresia consequent to intrauterine intussusception is reported. The baby presented with features of neonatal intestinal obstruction but signs of peritonitis were absent. The intussusception was discovered on gross examination of distal atretic ileal segment. The case was managed successfully by resection and end to back anastomosis. This case is reported to highlight intrauterine intussusception as one of the causes of ileal atresia.

Cystic hygroma of the gluteal region.

Cystic hygromas occur most commonly in the neck. Rarely are they known to involve the axilla, groin, mediastinum, retroperitoneum, pelvis, mesentery, omentum and spleen. We successfully managed a case of cystic hygroma of gluteal region in a one and half year old child who presented with a cystic, non transilluminant swelling in this region since birth. The diagnosis of cystic hygroma was made by surgery and subsequently confirmed after histopathological examination. Because of rarity of cystic hygroma in gluteal region this case in being reported.

Choledochal cyst: a 10-year experience.

A retrospective study of 11 cases of choledochal cyst over a period of 10 years is presented. There were 8 females and 3 males in a ratio of 2.67:1. The classical triad of jaundice, mass and abdominal pain was seen in none of our patients. Ultrasonography was diagnostic in 9 out of 11 (80%) patients. Surgical procedures performed were, complete excision of the cyst with Roux en Y hepaticojejunostomy (nine cases), internal drainage of the cyst (one case) and excision of the extrahepatic cyst with Roux en Y hepaticojejunostomy in one case of Type IV choledochal cyst. One patient died in the immediate postoperative period. Others have remained well upto 10 years follow-up. We recommend total cyst excision with hepaticojejunostomy as the treatment of choice.