Michel Lallier

Falls from heights among children: a retrospective review.

BACKGROUND Falls are a major cause of emergency room visits and admissions in pediatric hospitals. METHODS To better understand the epidemiology of falls from height and develop prevention strategies, the authors reviewed all admissions after a fall at a single institution from 1994 to 1997. Inclusion criteria are falls from a minimum height of 10 feet. RESULTS Of 1,410 patients admitted after a fall, 64 patients including 45 boys and 19 girls with a mean age of 7.4 years (range, 1 to 18) are included in this study. Fifty (78%) children fell from 20 feet or less (two stories) and 14 (22%) from height greater than 20 feet. Patients mainly fell from balconies (n = 15), windows (n = 13), trees (n = 9), roofs (n = 6), stairs (n = 6), diving board (n = 3) and miscellaneous (n = 12). Over 60% of falls occurred in private houses and during the summer months. Fifty-five patients (86%) sustained only one system injury, two patients had no significant injury, and seven patients had multisystem injury. Major injuries included head trauma (39%), musculoskeletal (34%), abdominal (12%), maxillofacial (8%), and spine (6%). A surgical intervention was required for 43% of intracranial trauma, 39% of musculoskeletal injuries, 60% of facial trauma, and 50% of spine fractures. Mean length of stay in hospital varied according to the injured system. The overall survival rate is 98% with only one death after a fall greater than 50 feet. CONCLUSIONS Although rarely mortal, falls from height carry a significant morbidity and are costly to the health care system. To decrease the occurrence of injuries caused by falls, strategies should include awareness campaigns, parents education about the mechanisms of falls, increase parenteral supervision during playing activities, and legislative measures to ensure the safety of windows and balconies before the onset of summer.

Vascular complications after pediatric liver transplantation

From February 1986 to July 1994, 81 hepatic transplantations were performed in 73 children, with an overall patient survival rate of 83%. Forty-two patients received whole-liver grafts (WLG) and 39 had reduced-size grafts (RSG). The mean patient weight was 19.7 kg, with 29 patients weighing less than 10 kg. Seventeen vascular complications (21%) occurred in 13 children: 8 (10%) had hepatic artery thrombosis (HAT), 5 (6%) had portal vein thrombosis (PVT), 1 had both HAT and PVT (1%), and 3 (4%) had aortic conduit perforation (ACP). There was no significant difference in the incidence of HAT between RSG (5%) and WLG (14%) or between children weighing less than 10 kg (10%) and those weighing more than 10 kg (10%). The site of arterial reconstruction, end-to-end to the recipient common hepatic artery or end-to-side to the infrarenal aorta, had no significant effect on the occurrence of HAT (7% v 8%), but HAT occurred in 2 of 6 cases (33%) in which an aortic conduit was used. PVT documented in 5 cases (6%) was associated with technical complications (2), preduodenal portal vein (2), and a circulating cardiolipid antibody (1), and required thrombectomy, with no graft loss. Combined HAT and PVT was found in one patient 2 years postretransplantation for HAT. Although graft function is normal, portal hypertension persists. The aortic conduit, used in six patients, led to arterial perforation (3), HAT (2), and death (2). Of the 8 cases of HAT, 1 was diagnosed during autopsy and 7 occurred within 30 days and required retransplantation (6) or thrombectomy with rearterialization (1).(ABSTRACT TRUNCATED AT 250 WORDS)

THE OTOLARYNGOLOGIC MANIFESTATIONS OF GASTROESOPHAGEAL REFLUX: WHEN IS A PH STUDY INDICATED?

PURPOSE The aim of this study was to evaluate the pertinence of pH studies for persistent ear, nose, and throat (ENT) symptoms and their eventual relationship to gastroesophageal reflux (GER). METHODS Retrospective analysis was performed of age, reason for referral, pH study, treatment, and follow-up of patients with ENT symptoms suspected to have GER. RESULTS Of 3,000 esophageal pH studies performed over 16 years, 105 children were referred for ENT symptoms by an otorhinolaryngologist to rule out GER. Mean age was 33 months; 65% were boys. Reasons for referral included (number and mean age): stridor (n = 31, 8 months), laryngomalacia (n = 18, 13 months), recurrent otitis (n = 12, 42 months), laryngitis (n = 16, 50 months), dysphonia (n = 14, 59 months), laryngeal papillomatosis (n = 8, 62 months), sinusitis (n = 5, 56 months), and dysphagia (n = 1). Overall, 41% of study results were positive: stridor (58%), laryngomalacia (61%), laryngitis (56%) and sinusitis (40%). Patients with otitis, dysphonia and laryngeal papillomatosis had GER in 1%, 14%, and 25%, respectively. Follow-up in the three larger groups of patients showed resolution of the ENT symptoms after medical treatment of the reflux in 83% of patients with stridor and reflux, 86% with laryngitis and reflux, and 80% with laryngomalacia and reflux. Four fundoplications were performed: one neurologically impaired patient, and four nonresponders. CONCLUSIONS The authors recommend that a pH study be performed in children with stridor, laryngomalacia, laryngitis, and sinusitis when faced with failure of the usual treatment. However, a pH study does not seem as beneficial for recurrent otitis, dysphonia, or laryngeal papillomatosis.

Perineal hemangioma, anorectal malformation, and genital anomaly: A new association?

Two patients presented as full-term baby girls with anorectal and genital malformations with extensive perineal hemangiomas. The first patient had a vestibular anus with a perineal hemangioma involving the bladder, rectal, and vaginal walls. Skin ulcerations required a transverse loop colostomy for wound care. The vulva, urethral opening, and clitoris were deviated to the left, labia minora were absent, and the labia majora were abnormal. The second patient had an anus displaced anteriorly and deviated to the right. The external anal sphincter was hypertrophic on the left and atrophic on the right. Rectal examination showed agenesis of the right levator ani and a dentate line located at the skin level. She had a large perineal, sacral, vaginal, pararectal and retroperitoneal hemangioma and developed extensive skin ulcerations. She had only a hemiclitoris located to the left of the midline, near absence of labia minora, and hypertrophied labia majora. The urethra was displaced to the left and opened in the vestibule. Both patients had a spinal malformation (one with tethered cord and one with spina bifida) and a normal karyotype. Steroids and interferon allowed near-complete resolution of hemangiomas in both patients. The authors were impressed by the similarity of these two cases and could not find any previous description of this association.

Biliary tract complications in pediatric orthotopic liver transplantation

Biliary tract complications are reported in 15% to 30% of orthotopic liver transplantations (OLTs). Since 1986, 53 OLTs were done in 48 children with a mean age and weight of 5.3 years and 18.9 kg, respectively. Twenty-seven transplantations (51%) were reduced liver grafts (RLG) and 26 (49%) were whole liver grafts (WLG). Since 1988, 70% of transplantations have been RLG. Choledochocholedochostomy (mean weight, 25 kg) with a T-tube (CC) or choledochojejunostomy (CJ) (mean weight, 14.5 kg) were done in 24 (45%) and 29 (55%) cases, respectively. The overall mortality was 19% but none of the deaths were related to biliary problems. There were 13 biliary tract complications (24.5%) in 11 patients including 7 leaks, 5 obstructions, and 1 intrahepatic biloma. Leaks leading to bile peritonitis were managed with simple suture and drainage and were related to the T-tube (4), to the Roux-en-Y loop (2), and to the transection margin of a RLG (1). Obstruction was documented in 5 cases with none associated with hepatic artery thrombosis (HAT). Stenosis after CC reconstruction (2) required conversion to CJ. Two patients had revision of CJ because of kinking of the common bile duct after a left lateral segment graft and an anastomotic stricture 46 months after OLT. The last patient developed a vanishing bile duct syndrome 4 months posttransplant and is awaiting retransplantation. One patient had multiple episodes of cholangitis after HAT and was retransplanted. Neither the type of grafts (RLG 25.9% v WLG 23.1%) nor the type of biliary reconstruction (CC 25% v CJ 24%) influenced the rate of biliary complications.(ABSTRACT TRUNCATED AT 250 WORDS)

Hepatocyte transplantation in the Long Evans Cinnamon rat model of Wilson's disease.

Wilsons disease (WD), caused by a mutation in the P-type copper transporting ATPase (Atp7b) gene, results in excessive accumulation of copper in the liver. Long Evans Cinnamon rats (LEC) bear a mutation in the atp7b gene and share clinical characteristics of human WD. To explore hepatocyte transplantation (HT) as therapy for metabolic liver diseases, 8-week-old LEC rats (n = 12) were transplanted by intrasplenic injection of hepatocytes from donor Long Evans (LE) rats. Immunosuppression was maintained with intraperitoneal tacrolimus. The success of HT was monitored at 24 weeks of life. Serum aminotransferases and bilirubin peaked at 14–21 weeks in both HT rats and nontransplanted controls, but at 24 weeks, survival was 97% in LEC-HT versus 63% in controls. All transplanted rats showed restored biliary copper excretion and reduced liver iron concentration associated with increased ceruloplasmin oxidase activity. Liver tissue expressed atp7b mRNA (11.9 ± 13.6%) indicative of engraftment of normal cells in 7 of 12 HT rats, associated with a reduced liver copper concentration compared to untreated LEC rats. Periportal islets of normal appearing hepatocytes, recognized by atp7b antibody, were observed in transplanted livers while lobular host cells showed persistent pleomorphic changes and inflammatory infiltrates. In conclusion, transplantation of normal hepatocytes prevented fulminant hepatitis, reduces chronic inflammation, and improved 6-month survival in LEC rats. Engraftment of transplanted cells, which express atp7b mRNA, repopulated the recipient liver with normal functional capacity.

Hepatic artery pseudoaneurysm following laparoscopic cholecystectomy.

Abstract. The authors describe a hepatic pseudoaneurysm following laparoscopic cholecystectomy in a child. It arose from a hepatic artery that was ligated during surgery and was supplied by collaterals from the superior right branch. Because of the risk of hepatic infarction and recanalization of the pseudoaneurysm by new collaterals, the authors decided not to occlude the superior right branch, but to embolize the aneurysm itself with cyanoacrylate. Since the intra-arterial approach was not feasible, a transhepatic puncture was successfully performed.

Primary non-Hodgkin lymphoma of the extrahepatic biliary tract and gallbladder in a child

Lymphoma constitutes the third most frequently diagnosed malignancy after leukemia and brain tumors in children younger than 15 years of age (1). Lymphoma may be associated with bile duct obstruction in several ways: nodal enlargement secondary to lymphomatous infiltration or extranodal tumor compressing the common bile duct (2), secondary infiltration of the hepatobiliary tract in the case of disseminated lymphoma narrowing the bile duct lumen (3), and paraneoplastic bile duct paucity reported to produce cholestasis in patients treated for lymphoma (4). Primary non-Hodgkin lymphoma of the extrahepatic biliary tract has rarely been recorded. A literature review revealed only 12 reported cases of patients with lymphomatous infiltration of the extra-hepatic biliary tract, the youngest of whom was a 25-year-old woman (5–15). In these cases, the diagnosis was frequently missed at the onset of the disease. Before pathological studies confirmed the diagnosis of lymphoma it was suspected that most of the patients had adenocarcinoma or primary sclerosing cholangitis. Here, we describe an unusual case of a child with a lymphoma infiltrating the extrahepatic biliary tract without extrabiliary localization. The main challenge presented by this exceptional case was the differential diagnosis suggested by the patient’s clinical presentation and radiologic studies. We compare the patient’s evolution with previously reported cases.

Lambert-Eaton myasthenic syndrome revealing an abdominal neuroblastoma

Lambert-Eaton myasthenic syndrome is a paraneoplastic syndrome that may reveal a primitive tumor. Neuroblastoma in children and small cell lung carcinoma in adults are the leading tumors revealed or expressed by paraneoplastic phenomena. The clinical neurologic manifestations of Lambert-Eaton myasthenic syndrome are muscular weakness, sleepiness, absence of reflexes, and dysautonomia. Neurologic manifestations are explained by the induction of an autoimmune response because of the presence of antigens that are expressed by the tumor. Neurologic paraneoplastic disorders may also be the result of toxicity of drugs, coagulopathy, infection, or metabolic diseases. We describe the case of a 13-month-old child with unusual neurologic symptoms because of the presence of an abdominal neuroblastoma.

Silent tyrosinemia presenting as hepatocellular carcinoma in a 10-year-old girl.

Hepatocellular carcinoma (HCC) is rare in the pediatric population, with an incidence of 0.5 to 1.5 cases per million (1–3). Children with HCC, unlike adults, are less likely to have an underlying disease. An underlying disease is found in only 25% to 38% of children with HCC (1,3,4). HCC is seen in children with hepatitis B, biliary atresia, tyrosinemia type 1, progressive familial intrahepatic cholestasis type 1, Wilson disease, hepatitis C, neonatal iron storage disease, prolonged parenteral nutrition, glycogen storage disease type 1a, and other causes of cirrhosis (2,5–9). Tyrosinemia type 1 is an autosomal recessive inborn disease of the tyrosine pathway in which the enzyme fumarylacetoacetate hydrolase (FAH) is deficient (10). It usually presents in 1 of 2 ways: before 6 months of age with severe liver disease or after 6 months of age with renal dysfunction, mild liver disease, rickets, and/or growth failure (11). In patients with tyrosinemia and known liver disease, hepatocarcinoma is usually a long-term complication. We report the case of a 10-year-old girl who presented with HCC as the initial manifestation of tyrosinemia type 1.