Arié L. Bensoussan

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.

Isolated complete transection of the common bile duct due to blunt trauma in a child, and review of the literature.

A case of isolated complete transection of the common bile duct due to blunt abdominal trauma in a 3-year-old child is presented. The rarity of this injury and its initial presentation as a pancreatic pseudocyst warrant its description. This patient, the fifteenth child to be reported in the literature, was diagnosed as having a biliary injury following ultrasound-guided percutaneous drainage. Choledochal transection was documented at laparotomy and was successfully treated by proximal cholecystostomy and choledochojejunostomy with Roux-en-Y reconstruction. Delayed diagnosis is common, but this injury should always be kept in mind. Early diagnosis is feasible with the use of the HIDA scan. A review of the literature, modes of diagnosis, and techniques for surgical repair are described.

Esophageal leiomyoma in children: Two case reports and review of the literature

Leiomyoma of the esophagus is not uncommon in the adult population but is rarely seen in children; only 20 cases have been reported in the pediatric population. In this paper we describe two cases of esophageal leiomyoma in female patients aged 6 and 13 years and review previous reports. Several differences were noted between the pediatric and adult population. The mean age in children is 14 years (range, 4 to 20 years). Leiomyoma appears 1.71 times more often in females than in males. Localized lesions are found in only 9%, whereas the diffuse form predominates in 91%. The entire esophagus may be involved 35% of the time, and encroachment on the cardia or upper stomach occurs in 70%. Leiomyomas associated with familial syndromes (familial leiomyoma and Alports syndrome) occur in 22% of the cases. Major symptoms include dysphagia (86%), dyspnea (36%), vomiting (27%), retrosternal pain (27%), and coughing (22%). The initial diagnosis following contrast studies is most often achalasia. The diagnosis of leiomyoma is made only with subsequent endoscopy. Enucleation was performed in only 11% of the cases; surgical resection (including part of the stomach) was necessary in 78% with a 21% postoperative mortality. Esophageal leiomyoma should be considered in the differential diagnosis of mediastinal masses and esophageal obstruction. Accurate preoperative diagnosis is desirable in order to plan proper surgical treatment.

Injection sclerotherapy in the treatment of rectal prolapse in infants and children.

PURPOSE The aim of this study was to examine the efficacy of injection sclerotherapy as treatment for persistent rectal prolapse. METHODS The records of 28 patients with rectal prolapse treated with injection sclerotherapy over a 16-year period were reviewed. Initial management included assessment and correction of predisposing factors. If rectal prolapse persisted or if the prolapse required repeat emergency or operating room reduction, injection sclerotherapy was performed. The sclerosing agent included D50W in 21 patients (sole agent in 15, combined with ethanolamine oleate in four, and with phenol 5% in two). Phenol 5% alone was used in six patients, and 25% saline was used in one patient. Number of injections, recurrences, and complications were reviewed. RESULTS Two patients were lost to follow-up, and one patient was cured once a polyp was recognized and removed. Of the remaining 25 patients, 21 were cured. Sixteen required one injection, three required two injections, and two required three injections (64% cure rate after one injection, 84% cure rate after three injections). There were 4 of 25 failures: two went on to low anterior resection after having failed two injections each; one patient was treated with Thiersch cerclage and injection after two failed injections; and one patient did not respond after three injections but had less severe prolapses. Of those injected with D50W alone, 13 of 14 were cured with injection sclerotherapy. Nine received one injection, two received two injections, and two received three injections (64% cure rate after one injection, 93% cure rate after three injections). The only complication was excessive oozing at the injection site in one patient. He was simply observed in hospital overnight. Follow-up averaged 33 months. The only significant underlying abnormality in our patient population was spina bifida in one patient. This patient was cured with injection therapy. Cystic fibrosis was ruled out by clinical examination and sweat chloride test in all patients. Constipation was the most common condition identified with the onset of rectal prolapse (15 of 28). CONCLUSIONS Injection sclerotherapy is simple and should be considered as the first line treatment of recurrent rectal prolapse after failure of conservative measures. D50W is effective, easily available, inexpensive, and associated with few complications.

Partial splenectomy for benign cystic lesions of the spleen

Cystic lesions of the spleen are uncommon, about 600 cases being reported in the world literature. This report concerns three pediatric patients treated by partial splenectomy for benign cyst. In the world literature most of the cystic lesions of the spleen are treated by splenectomy and more recently by partial splenectomy. The infected lesions are treated by splenectomy or by incision and drainage only. Partial splenectomy has evident advantage over splenectomy. Although in the world literature we have not found a single case of infected splenic benign cyst treated by partial splenectomy, we believe that this procedure also has an advantage over drainage as the infected and necrotic part of the cyst is removed, thereby reducing morbidity and further possible complications.

Obstructive neonatal respiratory distress: infected pyriform sinus cyst

Infected lateral cervical cysts in newborn are rare. We present the case of a baby born at 41 weeks of gestation. At day 3, persistent cyanosis was noted, and a mass appeared in the left cervical region next to the sternocleidomastoid muscle. No cutaneous sinus was visible. Ultrasound imaging showed no sign of blood flow within the mass and no septae. The mass extended down to the aortic arch and pushed the trachea to the right. A cervical lymphangioma was first suspected. Puncture of the mass evacuated 80 mL of pus, and a drain was put in place. Opacification through the drain showed a tract originating from the left pyriform fossa. Preoperative laryngoscopy and catheterization of the fistula tract confirmed the diagnosis. The cyst was totally excised up to the sinus with the assistance of a guidewire inserted orally through a rigid laryngoscope. This is a rare case of an infected pyriform sinus cyst in the neonatal period.

Acute adenitis in children: clinical course and factors predictive of surgical drainage.

Objectives:  To describe clinical course of children hospitalized for a first episode of acute unilateral infectious adenitis and to identify factors predictive of surgical lymph node drainage.

Surgical and metabolic aspects of liver transplantation for tyrosinemia.

Tyrosinemia represents a very small percentage of patients undergoing liver transplantation world-wide. This disease is endemic within our referral area however, one-third of the liver transplantations at our institution are done for this disease. Since 1986, 16 patients with tyrosinemia and 34 patients with various other indications (non-tyrosinemic) have undergone a total of 55 liver transplantation. The survival rate for tyrosinemic patients is 87%, compared to 75% for non-tyrosinemic patients. Liver transplantation for hereditary tyrosinemia and other metabolic disorders without portal hypertension or previous portohepatic operations is notably easier to perform. Intraoperative blood loss was less, length of hospital stay was shorter and incidence of infections was lower in tyrosinemic than in non-tyrosinemic patients. Less than 10% of tyrosinemic patients had foci of hepatocellular carcinoma at the time of transplantation. For this reason, and while most patients with tyrosinemia will eventually require liver transplantation, our results do not support systematic early transplantation.

Neurogenic tumors and VIP-induced diarrhea

The authors report a case of cervical ganglioneuroblastoma associated with intractable watery diarrhea and hypokalemia. The probable physiopathologic mechanism of the diarrhea and its relation to the vasoactive intestinal peptide secretion (VIP) by the tumor are discussed.

Accessory liver lobe with mesothelial inclusion cysts in an omphalocele: a new malformative association.

Mesothelial inclusion cysts represent benign lesions that have been reported in a wide variety of locations. Peritoneal cysts are observed and visceral involvement has been described, notably of intraperitoneal organs such as the spleen and the testis. We report the cases of 2 neonates who underwent surgical management of an omphalocele. The hernial sac contained an accessory liver lobe, displaying in both cases multilocular mesothelial inclusion cysts. The hobnail cells lining the cysts exhibited calretinin and cytokeratin immunohistochemical reactivity, as well as focal D2–40 reactivity. One of the cases occurred in the setting of a Beckwith-Wiedemann syndrome (MIM 130650), an overgrowth disorder notably associated with omphalocele and hepatic anomalies and tumors. However, no hepatic mesothelial cyst has been described in this syndrome. In the 2nd case the omphalocele and the hepatic cysts were the sole lesions observed in the neonate. To the best of our knowledge, these 2 cases represent the first description of such an association.