Man Mohan Harjai

Repair of a giant omphalocele by a modified technique

Abstract Large omphaloceles that contain centrally herniated liver pose challenges to surgical closure, the most significant being the space limitation of the abdominal cavity. In addition, the “pedicled” nature of the liver on the inferior vena cava creates a predisposition to acute hepatic vascular outflow obstruction as the liver is reduced into the abdominal cavity. In such cases, the alternatives include conservative treatment or staged silo reduction. The worst complication of silastic silo (SS) placement is tension and infection of the fascia with disruption of the suture line. Once infection or premature disruption occurs, closure of the defect is difficult or impossible.This case report details a different management technique for a newborn with a giant omphalocele and presents an interesting variation of the usual SS technique that may be helpful in the management of some cases, especially in an emergency. The thick silk sutures applied in the present case absorbed the tension and the silastic sheet prevented the risks of infection and adhesions.

Congenital urethrocutaneous fistula

Abstract Congenital urethral fistula is an extremely rare but easily manageable anomaly that may be confused with hypospadias. This is a case description of a congenital fistula of the anterior urethra. Awareness of the entity will avoid complications.

Fecal Incontinence after Posterior Sagittal Anorectoplasty – Follow up of 2 years

After an anorectal malformation (ARM) is repaired, the goal is fecal continence of the patient. Toilet training is not complete in children below 4 years of age. Manometric and radiological studies need cooperation of the child, and are therefore of little value during the critical preschool years. In this present study, we used only clinical criteria to assess the child for constipation and incontinence after definitive operation. We included all patients of ARM wef 01 April 1998 to 31 March 2000. Only 2 children had crossed 4 years of age at the time of this assessment and therefore it was not possible to assess total continence postoperatively. We found that the incidence of incontinence was less in low anomalies and more in high or intermediate anomalies, while the incidence of constipation was higher in low anomalies and less in high and intermediate anomalies. 31% of all patients born with anorectal malformations and subjected to posterior sagittal anorectoplasty (PSARP) approach were totally continent, 38% suffered with soiling of faeces while 31% had problems of constipation. The higher incidence of constipation as well as incontinence in our study is because of a short follow-up and secondly, these problems are known to improve with passage of time. The purpose of this article is to highlight the problems of bowel control even after the definitive operation and still much more is required to improve the quality of life of these unfortunate children.

Nuss Procedure for Pectus Excavatum – An early Experience

The technique of minimally invasive repair of pectus excavatum is a new operation that allows for repair of this deformity without any cartilage resection or sternal osteotomy. The procedure has revolutionized the management of pectus excavatum. Six cases of pectus excavatum were referred to our centre for surgical correction. However, based on Hallers CT index 2 merited surgery. Our results suggest that the minimally invasive repair of pectus excavatum is an exciting operation with minimal morbidity and excellent results. The innovative incorporation of thoracoscopic techniques and small but important modifications to the techniques will make this operation very effective and safe.

CRUSH SYNDROME REVISITED

Dear Editor, In wake of devastating disaster of Gujarat earthquake and casualties received at Command Hospital Pune, where about 7-8 patients (5%) developed renal shut-down following crush syndrome, we draw attention of all our colleagues towards this entity. Crush syndrome constitutes the systemic changes seen after crush injuries that are caused by either mechanical crush or extreme physical exertion. The pressure or entrapment causes necrosis of muscles and during revascularisation there is leakiness of the sacrolemmal membrane to cardiotoxic or nephrotoxic cations and metablites (i.e. potassium, phosphate, myoglobin and urate) of the sarcoplasma. The rapid and massive uptake by damaged muscles of extracellular fluid, sodium and calcium leads to profound hypovolaemic and hypocalcaemic shock. Thus the causes of death in crush syndrome are severe hypovolaemic shock, hyperkalaemia, hypocalcaemia, metabolic acidosis and acute myoglobinuric renal failure [1]. The myoglobinuria refers to an abnormal pathologic state in which an excessive amount of myoglobin is found in the urine, imparting a cola-like colour, usually in association with myonecrosis and a clinical picture of weakness, myalgias and oedema. Acute renal failure due to tubular obstruction by myoglobin plugs and urate is the most serious complication of myoglobinuria, which can be prevented by prompt and aggressive treatment [2]. The laboratory test in this entity reveals marked elevation of creatinine kinase (CK) levels and histopathological examination of the excised muscle shows features of acute rhabdomyolysis. Treatment consists of early massive volume replacement followed by forced alkaline solute (mannitol) diuresis. The fluids should be started preferably at site of disaster for a substantial salvage of lives, limbs and kidney function. The mannitol-alkaline regimen ameliorates the acidosis associated with shock and hyperkalemia, and protects against the nephrotoxicity of myoglobin and urate by alkalization of the urine [1]. If renal failure develops, haemodialysis should be started. Early fasciotomy and debridement with use of hyperbaric oxygen and medical treatment substantially increases the survival of disaster victims [3]. Overall early diagnosis and treatment improve prognosis in crush syndrome, but outcome of such limbs is poor and Volkmanns ischaemic contracture often follows [4].

CONGENITAL TRIPLE ATRESIA OF THE ESOPHAGUS, DUODENUM AND RECTUM – A DIAGNOSTIC DILEMMA

The early disturbance of organogenesis that results in esophageal atresia (EA) also affects other systems. The VACTERL association is a well known combination of defects for esophageal atresia and ano-rectal malformation. The associated anomalies often significantly alter treatment and affect the survivaL The association of esophageal atresia with tracho-esophageal fistula and anorectal malformation is common and is seen in 10% of cases while its association with duodenal atresia is not so common. Here is a case of triple atresia involving the esophagus, duodenum and anorectum.

Congenital scaphoid megalourethra associated with posterior urethral valves

Abstract Megalourethra is a very rare mesenchymal congenital anomaly and its association with posterior urethral valves is still rarer. We hereby present such a rare combination and discuss its etiopatho-genesis

An Unusual Herniation Through Omentoplasty Defect

Omental transfer is an old known procedure to revascularize the ischaemic limb. Its efficacy is doubtful but still practised in various parts of the country [1, 2, 3]. Both free and pedicled omental grafts have been used in the management of rest pain and non healing ulcers associated with Buergers disease. Sometimes the transgression of natural anatomical planes is associated with bizarre complications. Here, we present an unusual case of herniation through the defect made for omental transfer. We found that the passage of the omentum through musculoaponeurotic plane was responsible for the hernia. To our knowledge, such complication has not been previously reported.

The Malone Antegrade Continence Enema Procedure, An Indian Perspective

Eight patients with bowel incontinence underwent an open Malone Antegrade Continence Enema (MACE) procedure between May 1997 to May 2000. Indications for the procedure included high anorectal malformation in 3, bowel dysmotility in 1. spinal dysraphism in 3 and presacral teratoma in 1. Age at presentation varied between 06 to 12 years. All but one patient claimed excellent results. Complications of the procedure included stomal stenosis in 1, stomal leak in 2 and inadequate emptying of effluent in 1. Patient selection was the key to success. All patients except one, were children who were literate and had access to a western toilet Whereas, for the vast majority in the Indian rural setting, the procedure may not be beneficial but for a select group of bowel incontinence children, this procedure may bring a dawn of hope.

CONGENITAL ANOMALY PRESENTING AS LUMP ABDOMEN IN ADOLESCENT GIRL

The close embryological proximity of the mullerian, wolffian and metanephric systems increases the potential for a common ipsilateral embryological error around the fourth week of gestation. Genital anomalies are four times as common in females as in males with unilateral renal agenesis. Uterine anomalies associated with congenital renal agenesis and skeletal abnormalities represent an uncommon pathology that often presents important diagnostic and therapeutic problem [1]. The Mayer-Rokitansky-Kuster-Hauser syndrome is an eponym often applied to individuals with mullerian duct anomalies in association with a solitary kidney. We present a rare variant where left sided renal agenesis was associated with a large ipsilateral ovarian cyst and a bicornuate uterus. The left sided cornua was not communicating with the cervix hence there was ipsilateral hemihematometra. We could not find a report of this particular triad inspite of an extensive search of literature.