Evaluation of UW solution for preservation of small intestinal transplants in the rat.

Simple cold preservation was evaluated in the rat model of small intestinal transplantation. Lewis rats received a syngeneic heterotopic graft of the jejunum either immediately (SI) or after preservation for 24 hr in Euro-Collins (SPE24), for 48 hr in EC (SPE48), for 24 hr in University Wisconsin solution (SPW24), or for 48 hr in UW (SPW48). The survival rates of SI, SPE24, SPE48, SPW24, and SPW48 were 100%, 78%, 0%, 100%, and 33%, respectively. Physiologic and pharmacologic properties of the grafts and native intestine were evaluated in vitro between 8 and 12 days after transplantation. Smooth muscle in all specimens contracted in response to cholinergic agonists, phenylephrine, and substance P, and was relaxed by isoproterenol. Excitatory innervation was present in 100%, 100%, 100%, and 67% of SI, SPE24, SPW24, and SPW48, respectively, while inhibitory innervation in each group was 50%, 29%, 60%, and 0%. Thus, smooth muscle function was preserved in all groups, but neural activity was impaired by some of the storage conditions. Preservation was best in SPW24, which had physiologic responses similar to those of SI. The rat jejunum can, therefore, be preserved in good condition for up to 24 hr before transplantation using simple cold storage in UW solution.

Small bowel transplantation. The effect of intraportal donor-specific transfusion 24 hours pretransplant and low-dose cyclosporine.

We tested the synergy of donor-specific transfusion (DST) and cyclosporine (CsA) in small bowel transplantation by comparing the systemic versus portal route of DST administration in a fully allogeneic rat model. The protocol is relevant to cadaveric transplantation by conditioning only 24 hr before allografting. A 1 ml intraportal DST day -1 and low-dose CsA significantly prolonged survival (MST 53.7 +/- 17.5 days) when compared with systemic DST day -1 and low-dose CsA (MST 18.4 +/- 5.6 days). This suggests that intraportal DST can be beneficial in cadaveric SBT, as only a 24-hr induction period is necessary. We speculate that antigen trapping in the liver and interaction of the DST with Kupffer cells is central to the portal DST effect.

A new prognostic classification for esophageal atresia.

BACKGROUND: Waterston's risk categories have been extensively used in the past for the classification and management of neonates with esophageal atresia. Advances in neonatal care have affected the prognostic usefulness of the Waterston classification. The purpose of this study is to formulate a new classification of risk factors that would more accurately predict outcome. METHODS: We retrospectively reviewed 95 consecutive cases of esophageal atresia and/or tracheoesophageal fistula treated at the Montreal Children's Hospital. Fifteen patients were in the Waterston class A, with a 6.7% mortality; 35 patients, in class B with a 5.7% mortality; and 45 patients, in class C with a 26.7% mortality. Logistic regression analysis of the influence of each risk factor (weight, pulmonary status, and severity of associated anomalies) on mortality was performed. RESULTS: Birth weight was not found to independently influence mortality. Only severe pulmonary dysfunction with preoperative ventilator dependence and severe associated anomalies had a prognostic influence. We therefore revised the classification to include only significant factors. The new high-risk class II consists of patients with either life-threatening anomalies or both major anomalies and ventilator dependence; the low-risk class I includes all other patients. These criteria radically changed the stratification of both the number of cases and the mortality among classes: the 82 patients in class I had a 7.3% mortality; the 13 patients in class II had a 69.2% mortality. Logistic regression analysis confirmed the validity of this new classification by showing displacement of individual variables by the revised classification but not by Waterston's. CONCLUSIONS: This new "Montreal classification" can simplify and improve the stratification of patients with esophageal atresia. It also reflects the more favorable outcome of low birth weight neonates.

Diaphragmatic pacing in children with congenital central alveolar hypoventilation syndrome.

BACKGROUND: The purposes of this study were to study congenital central alveolar hypoventilation syndrome (CCAHS), to report the experience at our institution with this disorder, and to describe the surgical technique of diaphragmatic pacing. Three patients are in our diaphragmatic pacing program. They all have CCAHS, documented by means of lack of respiratory drive to hypercapnia and normal peripheral nerve and muscle studies. Two patients have associated Hirschsprung's disease. METHODS: The ages of patients at insertion of bilateral phrenic nerve pacemakers were 1, 2, and 5 years. A bilateral axillary thoracotomy in the third interspace was used in two patients, and anterior thoracotomy was used in one for insertion of electrodes on the phrenic nerves. Counterincisions in both flanks were used in all patients for insertion of the receivers in subcutaneous pockets. RESULTS: In all patients pacing was commenced within 1 week of the surgical procedure, because no primary pacemaker failures occurred. One patient has experienced no failure of the equipment and no infectious complications at 4 years. A second patient has had the pacemakers for 6 months without complications. The third patient underwent placement of bilateral pacemakers in 1984. In this patient a 10-year experience has resulted in subcutaneous implant failure on two separate occasions; also the presence of a staphylococcal empyema necessitated the temporary removal of one phrenic nerve electrode for 6 months, with successful reinsertion. All patients now receive mechanical assisted ventilation when sleeping and phrenic nerve pacing when awake. CONCLUSIONS: Pediatric surgeons should be aware of CCAHS because it may be treated with surgically implanted electrodes that allow for pacing of the diaphragm. The technique has an acceptable complication rate, and it can greatly decrease the impact of the disease on the lifestyle and activity of the patient. CCAHS also may be associated with Hirschsprung's disease.

Single donor transplantation of fresh and cryopreserved pancreatic fragments into the liver parenchyma in syngeneic rats.

In a series of experiments on syngeneic rat islet (pancreatic fragments) transplantation we demonstrate that direct hepatic transplantation is successful to alleviate streptozotocin induced diabetes with tissue from a single donor. The experimental groups were: recipients of fresh, and cryopreserved pancreatic fragments. The fresh graft was prepared by collagenase digestion. Cryopreserved fragments were further treated by a standard freeze-thaw protocol which consists of slow cooling at 0.3 degrees C/min to -75 degrees C followed by transfer to -196 degrees C, in the presence of 1.4 M Me2SO, and storage at this temperature for one day or 1 wk, and then warming them back to room temperature at a rate of 35 degrees C/min. Streptozotocin-induced diabetes in rats can be reversed by injection of isolated pancreatic fragments from a single donor directly into the liver. No significant difference was observed between the recipients receiving fresh or cryopreserved tissue for 1 day or 1 wk. It is possible that elaborate purification itself is not conducive to successful alleviation of diabetes. This would corroborate the hypothesis that trophic factors are present in impure fragments. The direct infusion of islet fragments into the liver could allow for percutaneous administration in human transplantation.

Laryngotracheoesophageal cleft. An easily missed malformation.

Laryngotracheoesophageal cleft is a rare congenital anomaly characterized by a midline defect of variable length between the posterior larynx and trachea and the anterior wall of the esophagus. This report concerns the clinical course and follow-up of three patients with this anomaly and a review of the literature. Only one of these patients had a successful repair on the first attempt. The difficulty in making the diagnosis is stressed. This anomaly is associated with a high morbidity and mortality.

Preservation of canine small bowel by freezing.

A total of 133 canine small bowel segments were isolated in 51 dogs according to a previously described technique that permits an in vivo freeze-thaw experiment to be carried out after perfusion with various cryoprotective agents. All control segments (33) survived. One hundred segments were frozen with cold intra-arterial helium and ambient cold nitrogen gas after perfusion with dimethyl sulfoxide (DMSO), inositol, or glycerol in 5% and 10% concentrations, alone, or combined, and with chlorpromazine and hydrocortisone added. Inositol had no cryoprotective effect. Approximately one half of segments frozen after protection with DMSO and with glycerol alone or combined with inositol survived the freeze-thaw injury and were intact on long-term follow-up.

Diaphragmatic pacemaker failure in congenital central hypoventilation syndrome: a tale of two twiddlers.

Two patients with congenital central hypoventilation syndrome (CCHS) experienced phrenic nerve pacer failure due to deliberate manipulation of the internal receiver implant ("twiddling"). The patients, aged 7 and 12 years, presented with repeated episodes of pacer failure associated with local pain over a period of 18 months. They had progressively coiled the pacing wires to the point of breakage, which only became apparent at surgery. The breaks were not recognized radiologically, although in retrospect progressive twisting of the wires was evident on serial chest radiographs. Both patients required replacement of the internal receivers under general anesthesia. We recommend that the chest radiograph that is undertaken to investigate the cause of pacer dysfunction include the internal receiver. A plain chest radiograph that demonstrates progressive coiling of the subcutaneous pacing wire should raise suspicion of pacer wire breakage regardless of the patient's age.

Experience with peritoneo-venous shunting for congenital chylous ascites in infants and children.

The management of chylous ascites presenting in association with primary lymphedema of the limbs and possibly lymphatic malformation in the lungs is difficult when the increasing abdominal distension causes respiratory distress. Laparotomy may be useful in traumatic chylous ascites or in intestinal lymphangiectasia localized to a segment of the bowel. It would seem that when conservative management such as diuretics, diet, and repeated abdominal paracentesis do not improve the respiratory distress, a peritoneo-venous shunt is logical. This report of two patients with severe chylous ascites and generalized lymphatic malformations causing or accentuating respiratory distress, stresses the possibility that peritoneo-venous shunts may not have the same long term function as seen in cirrhotic ascites. Long-term follow-up on the use of these shunts specifically for chylous ascites is not available.

Pyloric stenosis associated with malrotation.

Three cases of pyloric stenosis associated with malrotation are presented. In one case, the diagnosis was made synchronously, and in two cases, pyloric stenosis was noted following correction of malrotation. All three patients underwent pyloromyotomy and Ladd's procedure. These cases differ from the few reported hereditary cases of pyloric stenosis associated with malrotation, which have congenitally shortened bowel, functional obstruction, and poor prognosis. The unusual finding of pyloric stenosis and malrotation occurring together in the first patient was diagnosed with upper gastrointestinal series, obtained because of a high index of suspicion despite a palpable pyloric tumor and positive ultrasound examination. The second and third cases illustrate that vomiting following Ladd's procedure may be due to pyloric stenosis. When clinical findings do not support the presumed diagnosis, further investigation prior to operation is recommended so that rare concommitant pathology can be corrected.

Hematoma of bowel and cellulitis of the abdominal wall complicating herniography.

Two complications of herniography are presented: a hematoma of bowel causing intestinal obstruction, and a cellulitis of the abdominal wall causing septicemia. A technical error probably caused the hematoma and gastroenteritis played a role in the cellulitis. We have now narrowed our indications for herniography. We think it should be reserved for more difficult diagnostic problems such as recurrent inguinal hernia, recurrent hydrocele or eventration of the diaphragm.

Hereditary sacrococcygeal teratomas.

Hereditary presacral teratoma and its associated anomalies have been described in six kindreds. We report on a similar family where the father presents with a presumed meningocele and his two children with sacrococcygeal teratomas, associated with anterior meningocele in one.

Small intestinal transplantation in the rat: effect on physiological properties of smooth muscle and nerves.

The effect of transplantation on the physiological and pharmacologic properties of small intestine was evaluated in a syngeneic rat model. We examined the intrinsic contractile properties of the smooth muscle, the neural control of intestinal motility, and the sensitivity of the muscle and nerve endings to biologically active compounds in vitro, comparing transplanted tissue to controls. Both graft and control tissue contracted in a dose-dependent manner to cholinergic agonists, 5-hydroxytryptamine, and substance P and relaxed in response to noradrenaline. Contractile properties of smooth muscle and sensitivity to drugs were not altered by transplantation. Excitatory innervation was also similar in all specimens, but the inhibitory response was altered by transplantation. In the control intestine inhibition became maximal above 30 Hz, while in the graft maximal inhibition was obtained at 5 Hz. These findings imply an absence of extrinsic adrenergic inhibitory innervation in the graft. Intrinsic nonadrenergic inhibitory nerves and the excitatory innervation were intact after transplantation.

Congenital anal fistula with normal anus.

Three patients with a congenital anovestibular/perineal fistula were treated at the Montreal Children's Hospital. Two females (one of East Indian and the other of Japanese origin) had anovestibular fistulae that became symptomatic in the first few months of life. The third patient, a boy of Korean descent, presented at 9 months of age with a chronically draining perineal fistula. During surgery, a small fistula tract was easily dissected out and excised. Microscopic examination showed a well-preserved rectal mucosa throughout the tract. Most male patients described to date had anourethral fistulae, often accompanied by other major anomalies such as esophageal atresia or renal malformations. We believe our patient is the first male to be described with a congenital perineal fistula; this suggests that some fistula-in-ano in male infants may be due to a congenital sinus that secondarily becomes infected and drains to the skin.

Function of smooth muscle and nerve after small intestinal transplantation in the rat: effect of storing donor bowel in Eurocollins.

Lewis rats received syngeneic heterotopic grafts of jejunum immediately, or after preservation for 24 or 48 hours in Eurocollins solution. Most recipients of tissue stored for 0 or 24 hours remained healthy. Longer storage caused death of the recipients. We evaluated the effect of tissue preservation by comparing grafts stored for 0 and 24 hours with control jejunum at successive intervals up to ten days after transplantation. The physiology and pharmacology of smooth muscle and nerve endings were studied in vitro. Circular and longitudinal muscle in all specimens contracted in response to cholinergic agonists, phenylephrine, and substance P, and was relaxed by isoproterenol and noradrenaline. 5-hydroxytryptamine caused contraction of longitudinal muscle in all cases, and of both muscle layers in grafts that had been stored prior to transplantation. In all grafts, the excitatory innervation was similar to control, while the extrinsic adrenergic inhibition was absent. Prior storage caused an additional loss of intrinsic nonadrenergic inhibition, but this recovered within eight days. These findings indicate that Eurocollins solution might be a useful vehicle for the preservation of donor intestine prior to transplantation, as long as the period of storage does not exceed 24 hours.

Spontaneous deep vein thrombosis in childhood and adolescence.

Deep vein thrombophlebitis (DVT), unrelated to intravenous catheters, is unusual in children. During the period between 1970 to 1984, 15 cases of spontaneous DVT were seen at The Montreal Children's Hospital. The female to male ratio was 2:1. Age ranged from 10 to 17 years. Venogram was positive in the 14 patients in whom it was done. Significant factors were related to the DVT in 12 patients: oral contraceptives, pelvic fracture, Ewing's sarcoma and chemotherapy, brain tumor, nephrotic syndrome, and ulcerative colitis (2, one of whom developed the DVT 3 months before the onset of gastrointestinal symptoms). The sites of thrombophlebitis encountered were left iliofemoral vein, right iliofemoral, right tibial, and left subclavian. Three patients developed pulmonary emboli, which were recurrent in two and required iliac vein ligation or inferior vena cava clipping. Treatment usually consisted of intravenous heparin followed by oral anticoagulants for 3 to 6 months. Two patients received streptokinase with good results. All the patients recovered. With a limited follow-up (6 months to 5 years), we have not seen any postphlebitic sequelae.

Splenic cysts: aspiration, sclerosis, or resection.

Percutaneous aspiration and tetracycline sclerosis is a safe but temporary therapy of large splenic cysts in children. Between 1985 and 1987, three girls with splenic cysts were seen. Their ages ranged from 5 to 14 years, and the cysts were from 8 to 16 cm in diameter. Despite their large size, all were asymptomatic and were discovered upon physical examination or ultrasound for unrelated conditions. All cysts were avascular by scan and had irregular crenated or smooth walls by ultrasound. Further investigation excluded infectious or parasitic causes. Each cyst was aspirated for diagnosis, and a pigtail catheter was inserted for drainage and sclerotherapy. All needle aspirations resulted in cyst collapse, but in one patient the pigtail catheter insertion was unsuccessful, and in the other two cases, multiple attempts of tetracycline sclerosis failed to obliterate the cysts. There were no other complications. Surgery for the recurrent splenic cysts was performed 3 months to 2 years following the percutaneous procedures. The two patients operated on with 3 months of aspiration underwent successful partial splenectomy and have normal splenic function by ultrasound scan, and absence of RBCs. The third patient had progression of the cystic disease throughout the spleen, and required splenectomy. Pathology confirmed multiseptate congenital mesothelial cysts in the first two patients and massive lymphangiomatosis in the third. In all three cases, percutaneous therapy was safe but did not result in long-term control. In one patient, the cystic disease progressed following sclerotherapy and may have influenced the need for complete splenectomy. Prior manipulation did not adversely affect the dissection and mobilization of the spleens.(ABSTRACT TRUNCATED AT 250 WORDS)

Hirschsprung's disease, imperforate anus, and Down's syndrome: a case report.

Patients with trisomy 21 have a higher incidence of several gastrointestinal anomalies. However, the coexistence of imperforate anus, Hirschsprung's disease, and trisomy 21 had not been reported previously. This report describes the case of an infant girl born with trisomy 21 and imperforate anus, without a fistula, who presented with bowel obstruction 3 months after anoplasty. The obstruction was attributable to Hirschsprung's disease. This was managed by a leveling colostomy in the descending colon, followed by an endorectal pull-through after 4 weeks. She has a normal stooling pattern 11 months after colostomy closure. Hirschsprung's disease should be suspected in infants with trisomy 21 who have constipation after repair of imperforate anus. The authors believe that the endorectal pull-through is the safest technique to use for Hirschsprung's disease after a previous anoplasty.

Hydrometrocolpos in neonate due to distal vaginal atresia.

Hydrocolpos is the result of vaginal obstruction and can become an emergency in the newborn period. The treatment of imperforate hymen is well defined, but the treatment of vaginal atresia is more complex. We encountered two cases of hydrocolpos secondary to distal vaginal atresia, that were operated on in the first days of life. One baby had distal atresia without persistance of urogenital sinus. Surgery combining abdominal perineal approaches and a posterior vaginoplasty was carried out. The second baby had hydrocolpos with persistance of urogenital sinus. Drainage through the sinus was unsuccessful because the baby developed sepsis by trapping urine in the uterus. Finally an abdominoperineal vaginal pull-through was successfully done. The embryology and literature are reviewed. The classification, indications and surgical technique are discussed.

Sequential sac ligation for giant omphalocele.

The authors describe a technique for the management of giant omphalocele in which the sac is not resected, but used to sequentially reduce the contents into the abdominal cavity. Three patients were treated in this fashion. Delayed primary fascial closure was achieved in two. The third patient did not have complete reduction after 3 weeks, and temporary placement of a silastic sheet was required. The sac remained intact in all three cases, and neither infection nor injury to the abdominal contents occurred. This technique has the advantages of maintaining an intact membrane and avoiding the initial operative placement of a prosthetic silo in these sometimes critically ill neonates.