Frank M. Robertson

Renal cell carcinoma in association with tuberous sclerosis in children

Tuberous sclerosis, a genetically transmitted multisystem neurocutaneous disorder, is associated with renal lesions in 50% of cases. The expected lesions are angiomyolipomas or renal cysts; renal cell carcinoma has been encountered in adults who have tuberous sclerosis, but is very rare in children. The authors report the case of a 5-year-old girl with tuberous sclerosis for whom atypical computed tomography findings led to the diagnosis of renal carcinoma at an early age. This experience suggests that children with tuberous sclerosis may need earlier screening.

Effects of nitric oxide synthase inhibition on regional blood flow in a porcine model of endotoxic shock.

The results of early studies suggest that nitric oxide (NO) synthesis inhibition may be therapeutic in sepsis, but recent data indicate that NO inhibition may be harmful. This study investigates the effects of NO synthesis inhibition with N-nitro-L-arginine methyl ester (NAME) on regional blood flow following endotoxemia. Anesthetized, instrumented swine were randomly divided into four groups. Controls received normal saline resuscitation (NSR) at 1 cc/kg/min beginning at T0. The lipopolysaccharide group (LPS) received NSR and Escherichia coli LPS, 200 micrograms/kg at T0. The LPS+NAME group received NSR and LPS at T0, plus NAME (50 micrograms/kg/min) starting at T1. The NAME group received only NSR and NAME. Hemodynamic data, regional blood flow, and gastric intramucosal pH (pHi) were measured hourly. LPS increased renal and carotid blood flow consistent with a hyperdynamic state. Mesenteric blood flow was decreased. Treatment of endotoxic animals with NAME decreased renal and carotid blood flow. Mesenteric blood flow and gastric pHi were improved by NAME. NO inhibition in endotoxic shock results in decreased carotid and renal blood flow, by decreasing cardiac output. Mesenteric blood flow and perfusion were improved; however, this requires further study for validation.

Fetoscopic cord ligation to prevent neurological injury in monozygous twins

SIR—The shared placental circulation of monochorionic twins predisposes a surviving twin to devastating neurological injury in the event of co-twin demise. We report use of fetoscopic cord ligation specifically to prevent neurological injury in monozygous twins in which severe congenital heart disease incompatible with life in one twin threatened the normal co-twin. A 32-year-old woman with one previous child was referred for evaluation of a 24-week twin gestation. Ultrasound revealed a single posteriorly placed placenta, same-sex twins, and a two-layered membrane consistent with monochorionic twins. Twin A was structurally normal, but fetal echocardiography of twin B revealed hypoplastic left heart syndrome associated with the unusual finding of severe right ventricular dysfunction, marked left ventricular endocardial fibroelastosis, severe tricuspid regurgitation, and early signs of non-immune hydrops indicating imminent fetal demise. Under combined epidural and general anaesthesia (providing maternal and fetal anaesthesia and uterine relaxation for fetal surgery), two 3 mm ports were placed under ultrasound guidance through 2-0 silk pledgeted purse-string sutures in the myometrium incorporating the amnion to prevent postoperative amniotic fluid leakage. A 2-0 vicryl suture was used for umbilical cord ligation under fetoscopic and ultrasonographic control, after which twin B became bradycardic and died. Trocars were removed and the pursestring sutures were tied, and the abdomen closed in layers. Follow-up sonograms revealed no central nervous system changes in the surviving twin. 8 weeks after the procedure the mother spontaneously ruptured her membranes and vaginally delivered a 1535 g neurologically intact baby girl with Apgars of 9 at 1 min and 9 at 5 min. Postnatal ultrasound revealed only an old unilateral grade I intraventricular haemorrhage. At 6 months computed tomography of the brain was normal and at 1 year of age she is neurologically intact and has met all developmental milestones. Necropsy on twin B confirmed the echocardiographic findings. Neurological injury in a surviving co-twin of a monochorionic gestation complicated by intrauterine demise has been estimated to occur in up to 46% of fetuses. The nature of the neurological injury is devastating with multicystic leucoencephalomalacia the most common pathology. The pathophysiology of the neurological injury in the surviving co-twin is thought to be acute hypotension and ischaemia occurring at the time of co-twin demise. If co-twin demise can be anticipated then fetoscopic cord ligation may prevent such injury. Although uterine exposure by laparotomy was necessary, it allowed precise mapping of the amniotic sacs and purse-string closure of the trocar sites. Although an entirely percutaneous technique has been reported, closure of the trocar sites is not possible and amniotic fluid leakage occurs in up to 30% of patients. Unlike previous cases, the indication for fetoscopic cord ligation in our case was specifically to prevent neurological injury and death of the normal co-twin.

Transanastomotic feeding tubes in repair of esophageal atresia

To avoid the need for a gastrostomy and parenteral nutrition during the 7- to 10-day healing period after esophageal anastomosis, the authors modified their technique for esophageal atresia repair to include placement of a transanastomotic feeding tube. A SILASTIC transanastomotic feeding tube and early enteral nutrition was used for 19 of 23 consecutively treated patients after repair of esophageal atresia and tracheoesophageal fistula. One of the 19 patients had recurrent fistula and another had an anastomotic leak. Five patients had significant gastroesophageal reflux (noted on barium esophagram), and four had strictures that required dilatation. Parenteral nutrition was necessary for only two patients. The authors conclude that transanastomotic feeding tubes and early enteral nutrition are safe and effective, reduce costs, and do not appear to increase the incidence of anastomotic leaks, strictures, or gastroesophageal reflux.

Recurrence of immune thrombocytopenic purpura after splenectomy

Immune thrombocytopenic purpura (ITP) frequently leads to splenectomy. Accessory spleens maybe found in a variety of locations, and may not be readily apparent. Retained accessory splenic tissue can lead to recurrent ITP, which, this report (involving multiple relapses in a single patient) demonstrates.

Inguinal-scrotal suppuration following treatment of perforated appendicitis

Suppuration of a congenital hernia or communicating hydrocele following generalized peritonitis is rare. The lack of reported cases in the recent literature may indicate a decreasing incidence of this unusual complication. We report a case of suppuration of a communicating hydrocele with testicular loss 2 days after treatment of perforated appendicitis in a 10-year-old boy.