Biomedical subjects
Publications and source records attributed to V Sripathi.
The MURCS association i.e. MUllerian duct aplasia, Renal aplasia, Cervicothoracic Somite dysplasia is a rare developmental disorder. The authors report a case of MURCS association with supernumerary ribs in a 7-month-old infant who presented with failure to thrive.
During evaluation of chronic abdominal pain, a 9-year-old male was found to have a horseshoe kidney (HSK) with multicystic dysplasia (MCD) of the left-sided component. Attached to the MCD was a very large, tortuous ureter occupying almost the whole left side of the abdomen. This ureter on dissection was found to end blindly adjacent to the bladder. MCD of one-half of a HSK is an unusual lesion. Its association with a large megaureter with juxtavesical atresia is a unique event. In HSKs, controversy exists about the need to remove a small dysplastic segment. If this segment is associated with a large ureter, as in our case, removal is mandatory in order to avoid pain and infection.
A 3-year-old male with a right-sided Wilms' tumor presented with tender hepatomegaly and bilateral lower-limb edema. Ultrasound and echocardiography showed a massive tumor thrombus completely occluding the inferior vena cava, right atrial cavity, and extending retrogradely into the middle hepatic vein. Two courses of preoperative chemotherapy (vincristine, actinomycin D, adriamycin) caused minimal shrinkage of the thrombus. The tumor and thrombus were successfully removed with the patient under cardiopulmonary bypass and deep hypothermic circulatory arrest followed by multiagent chemotherapy (vincristine, actinomycin D, adriamycin, cyclophosphamide). The child is alive and well with no evidence of disease 15 months later. Occlusion of the hepatic vein by a tumor thrombus in Wilms' tumor is a very rare event. It was completely removed by the right atrial route under direct vision in this child.
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BACKGROUND: Hydronephrosis secondary to pelviureteric junction (PUJ) obstruction is common in infancy and childhood. Pyeloplasty has until recently been the accepted method of management, but alternative endo-urological techniques have evolved in the last decade. METHODS: Published results of conventional pyeloplasty for primary PUJ obstruction in children were compared with published results of endo-urological procedures. RESULTS: Sixty-six pyeloplasties were performed in 61 children in a 6-year period. During a similar period, 63 primary endo-urological procedures were reported in the literature. The success rate after pyeloplasty was 95.5% compared with 65% after endo-urology. CONCLUSIONS: Conventional pyeloplasty is superior to endo-urology and should remain the gold standard for the treatment of primary PUJ obstruction in children.
OBJECTIVE: To review the results of gender reversal in six patients with 46XX congenital virilizing adrenal hyperplasia (CVAH). PATIENTS AND METHODS: Fifty-one patients with 46XX CVAH were seen in an 8 year period; 45 were managed by conventional feminizing genitoplasty, but six underwent gender reversal and were managed as males. The clinical decision for gender reversal was made after appropriate counselling and was based primarily on parental choice, this being influenced significantly by a delayed diagnosis in four patients. Surgical management consisted of gonadectomy, excision of Müllerian structures and staged hypospadias repair/ chordee correction in four patients, and circumcision in two completely masculinized children. RESULTS: All six boys are well adjusted to their gender of rearing, with ages ranging from 3 years to 16.5 years (mean 8.5) at the time of review. Two children have normal penises and four have a satisfactory result after two-stage repair of hypospadias/chordee. CONCLUSION: Most patients with 46XX CVAH are preferably raised as females and require a feminizing genitoplasty. However, the clinical decision may be influenced by many factors, including delay in diagnosis, social bias and the premium on male rearing in certain communities. When male rearing is chosen, early gonadectomy and excision of Müllerian structures, together with staged hypospadias repair, gives satisfactory results.
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The tenth occurrence of complete unilateral agenesis of the diaphragm (McKusick 22240) in siblings is reported in this study. It is likely that parents of children with this type of congenital diaphragmatic hernia have a significantly increased recurrence risk for future offspring. Genetic counselling and antenatal ultrasound should be considered for subsequent pregnancies in families with a previous child with complete agenesis of the diaphragm.
Twenty-three children with primary obstructive megaureters presented between 1978 and 1988 to the Princess Margaret Hospital for Children in Perth. Twenty-eight ureters were treated. Urinary infections were the presenting feature in 14 children. The obstructive segment was transvesically excised. Histopathologic examination of the distal, intramural ureter showed fibromuscular disarray with a relative increase in fibrous tissue and reduction of musculature in all specimens. Twenty-two ureters were tapered by excision and all 28 were reimplanted using an antireflux technique. Seventeen children were followed for an average of 3 years. Seven children showed renal growth, reduction in ureteric size by greater than 2 cm, improvement in glomerular filtration rate by more than 10%, no obstruction on reflux, and no infections in postoperative period. Four children showed all the above but suffered one or more infections after the operation. Of the remaining 6 children, 3 had postoperative obstruction and 3 had vesicoureteric reflux.
Lap belts are a feature of the middle seat occupant in the rear seat of most Australian family cars. Without the provision of a child harness, they are inadequate to restrain children under eight years of age. We report a motor vehicle accident involving a seven year old child wearing an adult lap belt which resulted in an ileal stricture and a potentially unstable distraction injury of the lumbar spine.