Anurag Krishna

Accessory limbs associated with spina bifida – a second look

Abstract An accessory limb associated with spina bifida was already reported by the authors. We had then described it as a result of a very early splitting of the limb bud arising from the paraxial mesoderm. We have subsequently seen three other such cases, which are described in this report as well as a review of five other cases in the literature. It is proposed that the growth of the accessory limb occurs from a mesodermal blastema that is a result of de-differentiation from Schwann cells.

Laparoscopic repair of a congenital diaphragmatic hernia

Abstract.The standard treatment of congenital diaphragmatic hernia is by open operation. We have successfully performed a laparoscopic repair in an asymptomatic 2-year-old child. We recommend this as a safe alternative treatment modality in diaphragmatic hernias presenting past infancy. The role of laparoscopic correction in the newborn period, however, remains to be determined.

Isolated extrahepatic bile duct injury: diagnosis and surgical management

Isolated injuries of the extrahepatic bile ducts due to blunt trauma are rare. The diagnosis in these cases is often delayed and there are no firm guidelines in regard to the treatment. We present a 3-year-old child with partial disruption of the confluence of the left and right hepatic ducts. A hepatic scintiscan was used to confirm the diagnosis and identify the site of the bile leak. The available literature has been reviewed briefly to highlight the clinical features that should arouse suspicion of such injuries and the various surgical options are discussed.

Scapular bronchogenic cyst

We report a rare case of a 3-year-old male child with scapular bronchogenic cyst. The cyst was excised because of associated pain and discharge from the swelling. Till date, 64 cases of cutaneous bronchogenic cyst have been reported in the literature. Only 12 of these patients had lesion located in periscapular area. The treatment is surgical as it can undergo malignant transformation.

Posterior urethral valves after infancy–urodynamic consequences

Abstract This study describes a subset of patients with posterior urethral valves (PUV) who presented late in childhood. The objective was to identify factors that lead to back-pressure effects on the upper tracts, which persist in spite of adequate valve ablation in some patients, and seek factors that may preserve the upper tracts despite untreated obstruction in other patients. Six children with PUV diagnosed after infancy were evaluated. The pre-operative work-up included renal biochemistry, ultrasonography, voiding cystourethrography, and uroflowmetry. Detailed urodynamic studies, including uroflowmetry and slow-fill cystometry, were performed in all cases 6 months after surgery. Adequacy of valve fulguration was confirmed by urethroscopy. Three of the six patients had normal upper tracts; in these, there was marked improvement in peak urine flow rates after fulguration and bladder pressures were normal. The other three patients had bilateral hydroureteronephrosis, and two had chronic renal failure. This group had markedly decreased functional bladder capacity with loss of compliance at low bladder volumes and significant residual urine volumes in spite of adequate valve fulguration, suggesting myogenic detrusor failure. We conclude that in patients with PUV presenting beyond the age of 5 years, upper-tract deterioration may accompany high storage pressures in the bladder. In some boys with long-standing obstruction the upper tracts may escape damage; in our series this was associated with normal bladder dynamics and appeared unrelated to the severity or duration of outflow obstruction.

Congenital H-type urethroanal fistula.

Abstract A case of congenital urethroanal fistula with a normal anterior urethra in a male child is reported. The fistula was demonstrated between the prostatic urethra and anorectum. This anomaly is usually associated with an atretic anterior urethra and has been variously described as a variant of a urethral duplication by some authors, and of an anorectal malformation (ARM) by others. We conclude that its rightful classification is as a variant of ARM in which the fistula is a result of persistence of the cloacal duct and corresponds to the anorecto-vestibular fistula with a normal anus (perineal canal) in a female.

Laparoscopic repair of paraesophageal hiatus hernia in infancy

Paraesophageal hiatus hernia (PEHH) is an uncommon type of diaphragmatic hernia in the pediatric age group. Two patients aged 5-months and 8-months presented with respiratory symptoms and underwent a laparoscopic repair. Preoperative assessment included chest x-ray and CT scan. We suggest that laparoscopic repair of PEHH in infants is safe and preferred mode of the treatment.

Massive pancreatico-pleural effusion: An often unrecognised entity

Massive haemorrhagic pleural effusion secondary to pancreatitis in a five year old girl is described. The diagnosis was established on the basis of an exudative pleural effusion with an amylase level above 4,000 IU/I. Management of effusion was by intercostal tube drainage and antibiotics.

Leopard Syndrome : A Tropical Rarity

This is a case report of a classical presentation of the Leopard syndrome from the Indian subcontinent. The male patient had progressively increasing generalised multiple lentiginosis, ocular hypertelorism, pulmonary stenosis, short stature, cryptorchidism, and pectus excavatum. The case is documented for its rarity in this part of the world and typical clinical presentation.