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Biomedical subjects

M D Stringer

Publications and source records attributed to M D Stringer.

At least 91 records · Page 5Linked to original sources

Improved outcome for children with hepatoblastoma.

Between 1981 and 1993, 41 children were treated for hepatoblastoma. Clinical, radiological and pathological data were reviewed retrospectively, focusing on surgical aspects of treatment and outcome. Fourteen children underwent primary resection of the hepatic tumour. One infant with severe congenital anomalies received only palliative treatment. Of 26 with irresectable disease, pulsed cytotoxic chemotherapy (cisplatin and doxorubicin) enabled subsequent surgical excision in 22 and one child with persistent extensive intrahepatic disease was successfully treated by liver transplantation. Thus, with a policy of selective preoperative chemotherapy, 90 per cent of hepatoblastomas were resectable. There were no perioperative deaths from haemorrhage but one child died from an intraoperative tumour embolus. A total of 28 survivors, 27 of whom are disease-free, were followed for a median of 5 years. The cumulative probability of survival in patients treated with intent to cure was 67 per cent. Analysis of survival data suggested a favourable outcome for those with a pure fetal histological tumour subtype. These results demonstrate significant progress in the treatment of hepatoblastoma.

Child↗

Biliary amylase and congenital choledochal dilatation.

The relationship between levels of biliary amylase measured at operation and clinical features was studied in a series of 55 children with congenital biliary dilatation (choledochal cyst) who presented between 1976 and 1993. There were 36 cystic and 19 fusiforms dilatations in the series. The most common modes of presentation were painless jaundice (n = 23) and pancreatitis (n = 22). Five infants presented with abnormal antenatal ultrasound examinations. Children with pancreatitis were older than those with painless jaundice (4.2 versus 1.5 years; P = .005), and a higher proportion had raised levels of biliary amylase (100% versus 44%; P < .0001). There was no difference in the age at presentation (P = .32), clinical mode of presentation (P = .3), or the level of biliary amylase (P = .25) between cystic and fusiform dilatations. A correlation was found between age at surgery and biliary amylase in the cystic (rs = 0.55; P = .001) but not in the fusiform group (P = .22). All infants with antenatal diagnoses were cystic dilatations. Choledochal cystic dilatations that were diagnosed antenatally did not have significant amylase reflux, suggesting that the aetiology of this subgroup is truly congenital. Children who present at a later age with pancreatitis invariably have high levels of biliary amylase, which is presumed to occur because of a common channel and reflux of biliary and pancreatic secretions.

Adolescent↗

Prenatal diagnosis of esophageal atresia.

The prenatal sonographic detection of esophageal atresia (EA) has been possible for more than a decade and relies on the finding of a small or absent fetal stomach bubble associated with maternal polyhydramnios. The aims of this study were to assess the accuracy of this technique and to determine whether the outcome of prenatally diagnosed EA differs from its postnatal counterpart. All fetal sonograms performed between January 1989 and October 1993 demonstrating a small or absent fetal gastric bubble were reviewed together with all neonates with EA treated during the same period. Eighty-seven fetuses with a small (n = 53) or absent stomach bubble (n = 34) were identified, representing 1.4% of all fetal sonographic surveys. Esophageal atresia was present in 15; in 13 of these, the maternal amniotic fluid volume was increased. The positive predictive value of an absent stomach bubble and polyhydramnios was 56%, and the sensitivity of prenatal sonography in the diagnosis of EA was 42%. One neonate with EA had the prenatal diagnosis established at another institution, yielding a total of 16 cases of prenatally diagnosed EA for analysis. Seven (44%) of these had trisomy 18. Of the remaining nine, two had isolated EA, two had laryngeal atresia and EA, and there were two late-gestational unexplained fetal deaths. Only four (25%) survived through the neonatal period. The prognosis of the fetus with EA is radically different from that of the neonate with EA.

Esophageal Atresia↗

Fetal diaphragmatic hernia without visceral herniation.

Many cases of congenital diaphragmatic hernia (CDH) are currently detected before birth. The authors hypothesized that there is a subgroup of patients with CDH who have no evidence of visceral herniation in utero and who would be expected to have less pulmonary hypoplasia and a good prognosis. Among 41 neonates with left-sided CDH treated between January 1990 and October 1993, 17 cases were diagnosed after birth. Ten of the 17 had undergone detailed fetal sonographic imaging at or after 20 weeks' gestation. After independent review of the prenatal scans of these 10 patients, one was found to have evidence of a diaphragmatic hernia and was excluded from further analysis. The other nine fetuses survived, and prosthetic repair of the diaphragmatic defect and extracorporeal membrane oxygenation (ECMO) were each required in only one patient. This contrasted with the outcome for 18 control patients with prenatally diagnosed CDH: 4 (22%) died, 13 (72%) required prosthetic repair, 9 (50%) were supported with ECMO and the duration of ventilatory support and hospital stay were significantly longer. There is a spectrum of severity among both pre and postnatally diagnosed cases of CDH. In the neonate with an isolated left-sided diaphragmatic hernia, a good prognosis is to be expected if the condition was not detectable by detailed prenatal sonography in the second half of pregnancy.

Female↗

Short bowel syndrome.

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Adaptation, Physiological↗

Choledochal cysts: lessons from a 20 year experience.

Cystic dilatation of the biliary tree is a rare congenital anomaly. To determine mode of presentation, diagnostic pitfalls, and long term outcome after surgery, 78 children (57 girls, 21 boys) with choledochal cyst treated between 1974 and 1994 were reviewed. Anatomical types were: Ic (n = 44), If (n = 28), IVa (n = 4), and V (n = 2); a common pancreaticobiliary channel was identified in 76% patients. Age at presentation ranged from 0-16 (median 2.2) years, six patients being diagnosed by prenatal ultrasonography. Of the 72 patients diagnosed postnatally, 50 (69%) presented with jaundice, associated with abdominal pain in 25 or a palpable mass in three, 13 (18%) presented with pain alone, and two (3%) with a palpable mass. The classic triad of jaundice, pain, and a right hypochondrial mass was present in only four (6%). Four children presented acutely after spontaneous perforation of a choledochal cyst, two presented with ascites and one cyst was discovered incidentally. Plasma and/or biliary amylase values were raised in 30 of 31 patients investigated for abdominal pain; seven had evidence of pancreatitis at operation. In 35 of 67 (52%) patients referred without previous surgery, symptoms had been present for more than one month, and in 14 of them for more than one year, before diagnosis. Delayed referral was due to misdiagnosis as hepatitis (n = 12), incomplete investigation of abdominal pain (n = 6), and failure to note the significance of ultrasonographic findings (n = 10). Two patients referred late died from liver failure. Of the 76 patients with type I or IV cysts, 59 underwent radical cyst excision and hepaticojejunostomy as a primary procedure and 10 as a secondary operation after previously unsuccessful surgery. Sixteen patients have been lost to follow up but most of the remainder are well after a mean period of 4.1 (0.1-13) years. Choledochal cysts are often misdiagnosed, but prognosis is excellent if radical excision is performed.

Adolescent↗

Intra-abdominal extravasation complicating parenteral nutrition in infants.

Two infants receiving total parenteral nutrition via a central venous catheter positioned in the inferior vena cava developed an acute abdomen secondary to extravasation of the infusate. The presence of an associated abdominal mass necessitated a laparotomy in one patient. Both infants recovered completely after the catheter had been removed.

Abdomen, Acute↗

Small or absent fetal stomach: prognostic significance.

PURPOSE: To investigate the outcome of fetuses with a small or absent stomach. MATERIALS AND METHODS: We retrospectively reviewed sonograms in 87 fetuses with a small or absent stomach. Gestational age, amniotic fluid volume, stomach size, morphologic defects, and karyotypes were correlated with clinical follow-up or autopsy findings. RESULTS: Eight pregnancies were excluded because of oligohydramnios due to ruptured membranes. Seventy-nine fetuses with an absent (n = 27) or small (n = 52) stomach were included in the study group. Gestational ages ranged from 18 to 39 weeks (mean, 27 weeks). An abnormal outcome (structural abnormalities, intrauterine fetal or postnatal death) occurred in 23 (85%) of 27 fetuses with an absent stomach and 27 (52%) of 52 fetuses with a small stomach (combined, 63%). Karyotype was abnormal in eight (38%) of 21 fetuses with an absent stomach and two (4%) of 46 fetuses with a small stomach. CONCLUSION: An absent or small fetal stomach after 18 weeks gestation is associated with a guarded prognosis.

Abnormalities, Multiple↗

Patterns of portal vein occlusion and their aetiological significance.

Clinical and angiographic findings in 53 patients with portal vein occlusion were reviewed to determine the relationship between the pattern of venous occlusion and its aetiology. Five major patterns were identified. Group 1 comprised three children with idiopathic intrahepatic portal vein occlusion alone (hepatoportal sclerosis). Those in group 2 (25 patients), with occlusion of the main portal vein, accounted for almost half the total number and for most of those with a probable congenital aetiology. The ten patients in group 3 had angiographic occlusion and collateralization of the main portal and superior mesenteric veins. Intra-abdominal sepsis, other than that from pancreatic disease, was associated with this pattern of venous occlusion in which the splenic vein is spared. No patients with isolated portal and splenic vein occlusion (group 4) were recorded. Widespread thrombosis throughout the portal venous system (group 5; 15 patients) had a multifactorial aetiology but was typically observed in hypercoagulable states that were also associated with hepatic vein occlusion or other deep vein thromboses.

Adolescent↗

Separation of brachio-thoraco-omphalo-ischiopagus bipus conjoined twins.

Separation of 3-year-old brachio-thoraco-omphalo-ishiopagus bipus conjoined twin girls is reported. Detailed preoperative assessment and a multidisciplinary approach were essential prerequistes to surgery. The difficulties of managing the extensive body wall defect resulting from separation are discussed. Despite the use of subcutaneous and intraperitoneal tissue expanders, a large area of prosthetic material was required to reconstruct the abdominal wall. Skin cover was achieved using a combination of meshed allogeneic skin, homograft split skin, and autologous cultured keratinocytes. One twin died in the early postoperative period, but the other has recovered well and is awaiting further rehabilitative treatment.

Abdominal Muscles↗

Meconium ileus due to extensive intestinal aganglionosis.

Seven full-term infants with aganglionosis extending into the small bowel presented with clinical, radiological, and operative features of meconium ileus. Misdiagnosis resulted in inappropriate treatment. The correct diagnosis was eventually established by rectal suction biopsy, mostly after either recurrent intestinal obstruction or stomal dysfunction, and after cystic fibrosis had been excluded. For two patients, the results of rectal suction biopsies were initially misleading. Two infants died. Extensive intestinal aganglionosis should be considered a rare possibility in all infants with meconium ileus. In such cases, histological examination of the appendix may avoid this potential pitfall.

Biopsy, Needle↗

Inguinal hernias associated with bladder exstrophy.

OBJECTIVE: To determine the incidence and complications of inguinal hernias in patients with bladder exstrophy. PATIENTS AND METHODS: A retrospective review of inguinal hernias and their management in 70 consecutive patients (50 boys and 20 girls) with bladder exstrophy managed by staged reconstruction. Follow-up data were available for 69 patients. RESULTS: During a mean follow-up period of 5.9 years (range 0.3-14), 42 (86%) boys and three (15%) girls developed inguinal hernias. These were bilateral in 35 (78%) cases. All but three of these patients presented during infancy. In girls, no instance of incarceration or recurrence was noted. In contrast, 14 (29%) boys developed an incarcerated hernia and seven (17%) developed recurrent inguinal hernias. All recurrences occurred in boys under 2 years of age, were associated with an indirect sac and were unrelated to the seniority of the surgeon performing the initial herniotomy. No recurrences occurred in eight boys undergoing inguinal herniotomy at the time of bladder closure. CONCLUSIONS: Boys with classical bladder exstrophy managed by staged reconstruction have a much higher incidence of inguinal hernias than previously recognized. Most are bilateral, present in early infancy and are prone to incarceration. Bilateral groin exploration and meticulous inguinal herniotomy at the time of bladder closure may reduce morbidity.

Bladder Exstrophy↗

Longterm outcome after injection sclerotherapy for oesophageal varices in children with extrahepatic portal hypertension.

A consecutive series of 36 children with bleeding from oesophageal varices secondary to extrahepatic portal hypertension was successfully treated by endoscopic injection sclerotherapy and followed up over a mean period of 8.7 years after variceal obliteration. There were no deaths from portal hypertension or its treatment and morbidity related to oesophageal sclerotherapy was minimal. Endoscopic injection sclerotherapy alone proved safe and effective in controlling variceal bleeding from portal hypertension in over 80% of the children. Recurrent variceal bleeding developed in 10 (31%) patients but half of these were effectively treated by further sclerotherapy. Gastric variceal bleeding unresponsive to sclerotherapy necessitated successful portosystemic shunt surgery in four (13%) patients. Two children required splenectomy for painful splenomegaly. In most children injection sclerotherapy is the best treatment for the primary management of bleeding oesophageal varices, reserving portosystemic shunting or other surgical procedures for those with bleeding from gastrointestinal varices.

Adolescent↗

Follow-up after inguinal herniotomy or surgery for hydrocele in boys.

A retrospective review of 556 boys who had undergone inguinal herniotomy or surgery for hydrocele was carried out to assess the value of early routine postoperative follow-up. Of 386 children given an appointment, 319 (82.6 per cent) attended. The testis was no longer palpable in the normal scrotal position in 12 boys; eight were normal on further review and four required orchidopexy. Six hydroceles, all of which resolved, and four contralateral hernias were detected. Nineteen boys (4.9 per cent) were referred later with recurrent hernia (two patients), ipsilateral high testis (two), or contralateral hernia or hydrocele (15). Of 170 children not given a routine appointment, ten (5.9 per cent) were subsequently referred back with a transient hydrocele (two) or a contralateral hernia (eight). After a total of 440 appointments, eight boys (1.8 per cent) were found to have a significant abnormality. The rate of referral back to hospital was similar in both groups. Early discharge would make more efficient use of outpatient resources without significantly affecting overall outcome.

Follow-Up Studies↗

Recurrent necrotizing enterocolitis.

In the decade 1981 to 1991, 16 infants developed recurrent necrotizing enterocolitis (NEC). They comprised 12 (6%) of 196 neonates referred for further management of NEC and four others referred with major congenital anomalies. Their median gestational age was 32 weeks (range, 27 to 40), median birth weight was 1,260 g (range, 790 to 3,230), and the sex distribution was equal. Recurrent NEC occurred after a median interval of 37 days (range, 11 to 163) from the onset of the initial episode. All but one of the 16 infants were either premature (n = 10) or mature with major congenital anomalies (n = 5). Nine patients had previously undergone surgery for NEC. Medical treatment was successful in 11 patients with recurrent NEC. Two infants died, both of liver failure related to parenteral nutrition; one of whom had suffered four separate episodes of NEC and was found to have a superior mesenteric artery occlusion. There was no consistent association between recurrent NEC and the type or timing of enteral feeds or the anatomical site or method of management of the original attack. The mortality of 12.5% was similar to that for primary NEC.

Child↗

Current results of surgery for achalasia of the cardia.

Several treatment options are available in the management of achalasia of the cardia. Of a recent series of 14 children, 12 were treated by a modified Heller's myotomy combined with a floppy Nissen fundoplication. Symptoms were dramatically improved in nine during a mean follow up period of 3.9 years. Recurrent oesophageal pain was the most resistant symptom and continued to be moderately severe in three patients, two of whom obtained temporary relief by oesophageal balloon dilatation. Two patients treated by pneumatic dilatation alone have residual symptoms. These results support a primary surgical approach to the management of achalasia in children.

Adolescent↗