Biomedical subjects

T Wester

Publications and source records attributed to T Wester.

At least 19 recordsLinked to original sources

PubMed2006

Postoperative obstructive symptoms are common after Rehbein's procedure for Hirschsprung's disease.

Rehbein's procedure for Hirschsprung's disease has been questioned because several centimetres of aganglionic bowel are left behind, considered to result in obstructive symptoms. However, the reported outcome is similar to that after operations with other techniques. The purpose of this study was to review our experience of Rehbein's procedure during an 8-year period, focusing particularly on obstructive symptoms. We retrospectively studied 44 patients operated with Rehbein's procedure for biopsy-verified Hirschsprung's disease from October 1993 to October 2001. Three patients with total colonic aganglionosis and two patients who underwent total colectomy as a result of severe enterocolitis finally had a distal ileo-rectal anastomosis. Four (9 %) patients had a sphincteromyectomy before the Rehbein procedure. The patients' bowel function was evaluated by a questionnaire and an interview carried out 27 to 123 (mean 82) months after the operation. Two patients could not be traced and one patient, with total colonic aganglionosis, had an ileostomy at follow-up. Nineteen (46 %) of the 41 patients were treated with oral laxatives or enemas (12 patients), required repeated Botox injections (4 patients), or had undergone sphincteromyectomy (9 patients) due to obstructive symptoms. Eleven (27 %) of the 41 evaluated patients had soiling at least once a week at follow-up. Eleven (29 %) of 38 patients with a colorectal anastomosis had good bowel function without additional therapy. In conclusion, Rehbein's procedure for the treatment of Hirschsprung's disease is often complicated by obstructive problems.

Child↗

PubMed2004

Early outcome of transanal endorectal pull-through with a short muscle cuff during the neonatal period.

BACKGROUND/PURPOSE: Transanal pull-through has been advocated recently for classic Hirschsprung's disease. The procedure leaves no scars, is associated with less postoperative pain and discomfort, and shortens hospital stay. Long-term functional outcome of patients having transanal pull-through is unclear, but short-term function is reported to be very similar to that after open or laparoscopic procedures. One-stage neonatal repair of Hirschsprung's disease has been suggested to be associated with less cost and demand of resources without jeopardizing functional outcome. The aim of this study was to ascertain the feasibility and safety of transanal pull-through in the neonatal period. METHODS: Case notes of 40 consecutive patients who had undergone transanal endorectal pull-through for Hirschsprung's disease between January 2000 and February 2003 were reviewed. The patients were divided in to 3 groups: patients with neonatal primary pull-through (group I, 15 patients), patients operated on beyond the neonatal period (group II, 11 patients), and patients with a previous colostomy (group III, 14 patients). All colostomies except one were taken down and pulled through concomitantly with the transanal procedure. The case notes were evaluated for hospital stay, time to full oral feedings, operative and postoperative complications, need for postoperative dilatations, and occurrence of postoperative enterocolitis. RESULTS: There was no difference in median hospital stay (group I, 5 days; group II, 4; group III, 5) and median time to full oral feedings (group I, 4 days; group II, 2; group III, 3) between groups. Two patients (group III) had immediate postoperative prolapse of the pulled-through colon that was reduced without further sequels; 1 (group III) had infection of the stoma closure wound. Perianal skin rash that usually resolved within 6 weeks occurred more often in neonatal patients (group I, 10 of 15; group II: 4 of 11; group III: 8 of 14). Anastomotic dilatation regimen was required more often in neonatal cases (group I, 6 of 15; group II, 1 of 11; group III, 2 of 14). Enterocolitis requiring hospital care occurred in 2 patients (group I), 5 further patients (group II, 1; group III, 4) were treated as outpatients for symptoms suggesting mild enterocolitis or bacterial overgrowth. The median follow-up was 6 months. CONCLUSIONS: Transanal endorectal pull-through in neonatal patients is as feasible and safe as in older children or in those with a levelling colostomy. However, temporary postoperative skin rash occurs more frequently in neonatal patients, and postoperative dilatations are required more often than in older children.

Abnormalities, Multiple↗

PubMed2003

[Transanal endorectal pull-through with short muscular cuff in the treatment of Hirschsprung disease. Preliminary study with 37 patients].

INTRODUCTION: Totally transanal operation for classic Hirschsprung's disease has gained increasing popularity during the last few years. The procedure leaves no scars, is associated with less postoperative pain and discomfort and shortens hospital stay. The most commonly used technique for transanal pull-through is long endorectal dissection leaving a long muscular cuff, which is usually split posteriorly. We present our preliminary results following transanal endorectal operation with a short unsplit muscular cuff. MATERIALS AND METHODS: Thirty-seven patients underwent short-cuff transanal endorectal operation for Hirchsprung's disease between years 2000 and 2002. Patients' hospital records were analysed retrospectively. The collected data included age at the operation, associated conditions, hospital stay and time to full enteral feeds, occurrence of pre- and postoperative enterocolitis and preoperative stoma, operative complications, need for postoperative anal dilatations, postoperative perianal skin problems and preliminary data on bowel function. RESULTS: The median age at operation was 5 months (range 0-60 months), 13 patients were operated in the neonatal period. Six patients had Down's syndrome, 1 had cartilage-hair hypoplasia and one Ondine's syndrome. Six patients had preoperative enterocolitis. Thirteen patients had undergone levelling stoma formation for unremitting obstruction or enterocolitis, two of these had a long segment aganglionosis. The proximal ganglionic stoma was concomitantly pulled-through and anastomosed to the anus in all 13 patients with a stoma. Complications related to surgery occurred in 3 patients, 2 had prolapse of the pulled-through bowel (reduced immediately without later sequels) and 1 infection in the stoma closure wound. Median postoperative hospital stay was 4 days (range 2-21 days) and median time to full enteral feeds 3 (range 1-14) days. The median follow-up time was 6 months (range 1-22 months). Postoperative enterocolitis occurred in 1 patient, further 3 patients had symptoms suggesting mild enterocolitis. Nine patients required anal dilatations, two of those for a period of 3 weeks. Twenty-two patients had perianal skin rash, which resolved usually within 6 weeks. Early postoperative bowel function was characterised by frequent bowel movements in most patients. These usually resolved within few months; of the 17 patients with a follow-up for longer than 6 months only 2 have more than 3 bowel movements per day. At last follow-up frank soiling occurred in 2 patients, one patient needs oral laxatives for constipation. CONCLUSIONS: Transanal endorectal pull-through with a short cuff is a safe operation with low incidence of operative and postoperative complications. Hospital stay and time to full enteral feeds is significantly shorter than following conventional procedures; this associates with lower hospital costs. Long-term functional outcome of patients having totally transanal pull-through is unclear but short tern function is very similar than after procedures where transanal mucosectomy is combined with rectosigmoid dissection through laparotomy.

Child, Preschool↗

PubMed2000

[Treatment of patients with acute head injury in Vestfold 1987-96].

BACKGROUND: In Norway, patients with severe head injuries are transported to a regional, neurosurgical department for surgery, but some are operated on by surgeons without neurosurgical training in local hospitals. MATERIAL AND METHODS: Data were retrospectively collected from the records of all patients (n = 161) hospitalised alive with a severe head injury occurring within Vestfold County (1987-96). RESULTS: Overall lethality was 27%. 54 patients (34%) underwent decompressive surgery. 31 patients were operated in the local hospital, by 13 different surgeons. 30 of these patients had extracerebral haematomas. These patients had a significantly worse outcome than the 23 patients operated in a regional neurosurgical department. Patients with an epidural haematoma had a better outcome than patients with an acute subdural haematoma. We retrospectively classified ten operations in the local hospital as inadequate. INTERPRETATION: In Norway and countries with a similar hospital system, local hospitals should establish guidelines for safe and swift transport of head injury patients to the nearest neurosurgical department, and should not try to perform neurosurgical decompression in such patients.

Adolescent↗

PubMed2000

Expression of leukaemia inhibitory factor during the development of the human enteric nervous system.

Leukaemia inhibitory factor (LIF) is a neuropoietic cytokine, which promotes the development of enteric neurons in vitro, particularly when administered together with neurotrophin-3 (NT-3). The purpose of this study was to map the LIF immunoreactivity in the human enteric nervous system in foetuses, children, adults, and in patients with Hirschsprung's disease. Normal bowel specimens were obtained at postmortem examination of 13 foetuses, at 13-31 weeks of gestation, and at surgery in five children and two adults. Bowel resected in seven patients with Hirschsprung's disease was also investigated. Immunohistochemical analysis was performed on material fixed in formalin and embedded in paraffin. The specimens were exposed to antibodies raised against LIF. The ABC-complex method was used to visualise binding of antibodies to the corresponding antigen. LIF immunoreactivity was disclosed in the myenteric and submucous ganglion cells at 13-31 weeks of gestation, in childhood cases, and adults. LIF-immunoreactive ganglion cells were absent in aganglionic bowel, where the ganglia in the intermuscular layer were replaced by hypertrophic nerve bundles. These morphological findings indicate that LIF may play a role in the development of the enteric nervous system.

Adult↗

PubMed1999

Accessory wandering spleen associated with short pancreas. A pediatric case report.

We present an extremely rare case of a 5-year-old girl with short pancreas associated with three accessory spleens, one of which was a large wandering spleen. The diagnosis was established by US and CT. A large accessory wandering spleen is considered to be at risk of torsion and infarction as well as susceptible to abdominal trauma. The authors discuss the difficulty of establishing the diagnosis and underline the importance of US and CT as pre-operative diagnostic tools.

Abdominal Pain↗

PubMed1999

Decompressive surgery in acute head injuries: where should it be performed?

BACKGROUND: In Norway, most patients with severe head injuries are transported to, and operated in, the neurosurgical unit of the regional university hospital. However, some patients are still occasionally operated on in county central hospitals by orthopedic or general surgeons who do not have neurosurgical expertise. The aim was to analyze this surgical activity outside the neurosurgical units. METHODS: Data were collected from two sources: a nation-wide survey and the records of all patients with a severe head injury occurring within Vestfold county (1987-1996). RESULTS: The Norwegian county central hospitals perform each only 2.5 to 3 surgical evacuations of intracranial hematomas per year. In Vestfold county, a total of 161 patients were hospitalized alive with an acute severe head injury. One third of the patients (54 patients) underwent decompressive surgery, mostly evacuations of intracranial hematomas. The patients operated on in the central hospital had a significantly worse outcome than the patients who were transferred to and operated on in the neurosurgical unit of the regional hospital. Only patients with extracerebral hematomas were operated on in the central hospital. Patients with an extradural (epidural) hematoma had a better outcome than patients with an acute subdural hematoma. Based on the surgery records and preoperative and postoperative computed tomographic scans, one third of the operations (10 operations) in the central hospital were classified retrospectively as inadequate, because the hematoma was not evacuated or found or because the surgeons did not achieve control of the perioperative bleeding. The overall mortality rate was 29.8%. CONCLUSION: The present study indicates that, in Norway and countries with a similar hospital system, it must be difficult for general and orthopedic surgeons to achieve and maintain the skills required for emergency operations in patients with acute severe head injuries. Thus, it is probably to the patients' benefit to improve the general hospitals' competency and speed in the detection of candidates for surgical decompression, and stress the importance of these patients being transferred without unnecessary delay to a neurosurgical unit.

Acute Disease↗

PubMed1999

Interstitial cells of Cajal in the human fetal small bowel as shown by c-kit immunohistochemistry.

BACKGROUND: Interstitial cells of Cajal (ICCs) express the tyrosine kinase receptor c-kit, which is required for their development and spontaneous pacemaker activity in the bowel. From murine models it has been proposed that ICCs do not develop until after birth, but more recent findings indicate that c-kit is expressed early in the embryonic period. The temporal development of ICCs in the human gut remains unknown. AIM: To investigate ICCs in the human fetal small bowel using c-kit immunohistochemistry. SUBJECTS: Small bowel specimens were obtained at post mortem examination of 16 fetuses and nine neonates, eight of whom were premature, born at gestational ages of 13 to 41 weeks, without gastrointestinal disorders. METHODS: Immunohistochemical analysis was performed on material fixed in formalin and embedded in paraffin. The specimens were exposed to antibodies raised against c-kit (an ICC marker) and neurone specific enolase (a general neuronal marker). The ABC complex method was used to visualise binding of antibodies to the corresponding antigens. RESULTS: c-kit immunoreactive cells were visualised from 13 weeks of gestation. The immunoreactivity was mainly localised in association with the myenteric plexus. From about 17-18 weeks of gestation, the ICCs formed a layer along the myenteric plexus, whereas this layer appeared to be disrupted at 13-16 weeks of gestation. CONCLUSIONS ICCs are c-kit immunoreactive at least from a gestational age of 13 weeks in the human fetal small intestine. From 17-18 weeks of gestation until birth, they form a continuous layer around the myenteric ganglia.

Embryonic and Fetal Development↗

PubMed1999

Notable postnatal alterations in the myenteric plexus of normal human bowel.

BACKGROUND: Nitric oxide is the most important transmitter in non-adrenergic non-cholinergic nerves in the human gastrointestinal tract. Impaired nitrergic innervation has been described in Hirschsprung's disease, hypertrophic pyloric stenosis, and intestinal neuronal dysplasia (IND). Recent findings indicate that hyperganglionosis, one of the major criteria of IND, is age dependent. However, information is scanty regarding the neurone density in normal human bowel in the paediatric age group. AIMS: To determine neurone density, morphology, and nitric oxide synthase distribution of the normal myenteric plexus at different ages during infancy and childhood. METHODS: Specimens were obtained from small bowel and colon in 20 children, aged one day to 15 years, at postmortem examination. Whole mount preparations were made of the myenteric plexus, which were subsequently stained using NADPH diaphorase histochemistry (identical to nitric oxide synthase) and cuprolinic blue (a general neuronal marker). The morphology of the myenteric plexus was described and the neurone density estimated. RESULTS: The myenteric plexus meshwork becomes less dense during the first years of life. The density of ganglion cells in the myenteric plexus decreases significantly with age during the first three to four years of life. The NADPH diaphorase positive (nitrergic) subpopulation represents about 34% of all neurones in the myenteric plexus. CONCLUSIONS: The notable decrease in neurone density in the myenteric plexus during the first years of life indicates that development is still an ongoing process in the postnatal enteric nervous system. Applied to the clinical situation, this implies that interpretation of enteric nervous system pathology is dependent on the age of the patient.

Adolescent↗

PubMed1999

Expression of bcl-2 in enteric neurons in normal human bowel and Hirschsprung disease.

OBJECTIVE: The bcl-2 protein has the functional role of blocking apoptosis, ie, programmed cell death. This protein is widely expressed in the developing central and peripheral nervous systems. The purpose of this study was to map bcl-2 expression in the human enteric nervous system, as this has not previously been done. METHODS: Rectal specimens were obtained at autopsy of 13 fetuses at 13 to 31 weeks of gestation. Normal colon was also obtained from 5 children and 2 adults, and, in addition, ganglionic and aganglionic bowel resected in 11 patients with Hirschsprung disease was examined. Specimens were fixed in formalin, embedded in paraffin, and analyzed with immunohistochemical methods, using antibodies raised against bcl-2 and neuron-specific enolase (NSE). RESULTS: The bcl-2 protein was expressed in myenteric and submucous ganglion cells in fetuses, children, and adults. Nerve fibers of the enteric plexuses that were bcl-2 immunoreactive were few compared with the number of NSE-immunoreactive nerve fibers. In aganglionic bowel no bcl-2-or NSE-immunoreactive ganglion cells were revealed. Results of NSE immunohistochemistry showed clearly stained hypertrophic nerve bundles, known to be of extrinsic origin, which were only weakly bcl-2 immunoreactive. CONCLUSION: Expression of bcl-2 in enteric ganglion cells of the myenteric and submucous plexuses is displayed in the fetus and during childhood and is also retained in adult bowel. Immunohistochemical analysis of bcl-2 provides a good marker for identification of ganglion cells in Hirschsprung disease and may also be valuable for the diagnosis of disorders characterized by hypoganglionosis or hyperganglionosis.

Aged↗

PubMed1998

Morphometric aspects of the submucous plexus in whole-mount preparations of normal human distal colon.

BACKGROUND/PURPOSE: The diagnosis of intestinal neuronal dysplasia (IND) has traditionally been based on the finding of hyperplasia of the submucous plexus and increased acetylcholinesterase activity in parasympathetic nerve fibers in the lamina propria. However, recently it has been suggested that proposed diagnostic criteria relating to nerve cell density may overlap with age-related changes and that the finding of giant ganglia (ganglia containing more than seven ganglion cells) is the most relevant diagnostic parameter of IND. Ganglion cell counting is usually performed on conventional histological sections, whereas the topology of whole ganglia has been poorly studied. The aim of this study was to define the number of ganglion cells per ganglion and the ganglion cell density (the number of ganglion cells per surface area) in submucous whole-mount preparations of normal human colon. METHODS: Specimens from distal colon were obtained during postmortem examination from 14 patients who died of nongastrointestinal disease. The submucous layer was prepared as a whole mount and stained for NADPH diaphorase (a nitrergic neurotransmitter marker) and cuprolinic blue (a general neuronal marker). Ganglion cell density was estimated by counting at least 10 mm2. The number of ganglion cells per ganglion was counted in at least 20 ganglia per case. RESULTS: Ganglion cell density (NADPH diaphorase) fell markedly during the first 5 to 6 years of life (r = -0.60, P < .05). Most ganglion cells formed ganglia of 3 to 64 cuprolinic blue staining cells. The mean number of ganglion cells per ganglion did not vary with age. CONCLUSIONS: The density of NADPH diaphorase-positive ganglion cells in the submucous plexus of human distal colon decreases markedly with age. However, the number of ganglion cells per ganglion remains constant. These findings indicate that the age of the patient has crucial importance for the histolopathologic evaluation of enteric nervous system disorders.

Adolescent↗

PubMed1998

Hirschsprung's disease: a search for etiology.

In 1967, Okamoto et al suggested that the absence of ganglion cells in Hirschsprung's disease (HD) was attributable to failure of migration of neural crest cells. The earlier the arrest of migration, the longer the aganglionic segment. Since then, this hypothesis generally has been accepted. However, subsequent experiments using mouse models of intestinal aganglionosis indicate that nerve cells may reach the correct position but then fail to develop or survive. An alternative hypothesis has been proposed that the aganglionosis may be caused by failure of differentiation as a result of microenvironmental changes after the migration has occurred. Extracellular matrix proteins are recognized as important microenvironmental factors. It has been shown that enteric neurogenesis is dependent on extracellular matrices, which provide a migration pathway for neural crest-derived cells and promote the maturation of settled neural crest-derived cells. Altered distributions of extracellular matrices have been shown in human HD cases and murine HD models, suggesting the role of extracellular matrices in the pathogenesis of HD. Recent studies suggest that intestinal smooth muscle cells, target cells of enteric neurons, play an important role in guiding and influencing its own innervation. Normal maturation was inhibited in neurons cultured with smooth muscle cells of aganglionic colon in comparison to normal colon. Furthermore, it was demonstrated that levels of neurotrophic factors, crucial in the development and survival of enteric neurons, are decreased in circular muscle layers of aganglionic colon in comparison to normoganglionic colon. The smooth muscle cells of the aganglionic colon may represent an unfavorable microenvironment for neuronal development compared with the normally innervated region. Recently, markedly increased immunoreactivity of major histocompatibility complex (MHC) class II antigens and ICAM-1 was demonstrated in aganglionic bowel, suggesting the immunological mechanisms may be involved in the etiology of HD. Genetic factors have been implicated in the etiology of this condition because HD is known to occur in families and in association with some chromosomal abnormalities. Recent expansion of molecular genetics identified multiple susceptibility genes of HD, including the RET gene, the glial cell line-derived neurotrophic factor gene, the endothelin-B receptor gene, and endothelin-3 gene. Of these, inactivating mutations of the RET gene are the most frequent, occurring in 50% of familial and 15% to 20% of sporadic cases of HD. To date, despite extensive research, the exact etiology of this condition remains poorly understood. The present report describes the authors' current understanding of and recent progress in the etiology of HD.

Animals↗

PubMed1998

Intestinal neuronal dysplasia.

Intestinal neuronal dysplasia (IND) is a disorder of the enteric nervous system that was first described by Meier-Ruge in 1971. IND may be associated with Hirschsprung's disease or may occur as an isolated disorder. The incidence of isolated IND varies from 0.3% to 40% of suction rectal biopsies. The uncertainty regarding the incidence of IND has resulted from considerable confusion concerning essential diagnostic criteria. The diagnostic difficulties reflect the wide variability in the literature in terms of diagnostic criteria, biopsy procedures, staining techniques, and patient age. The recent application of histochemical and immunohistochemical techniques indicates that IND is a distinct histopathologic entity. The majority of patients with IND can be treated conservatively with laxatives and enemas. If bowel symptoms persist after at least 6 months of conservative treatment, internal sphincter myectomy should be considered.

Hirschsprung Disease↗

PubMed1998

NADPH diaphorase-containing nerve fibers and neurons in the myenteric plexus are resistant to postmortem changes: studies in Hirschsprung's disease and normal autopsy material.

OBJECTIVE: Nitric oxide is considered to be the most important messenger of inhibitory nonadrenergic, noncholinergic nerves in the enteric nervous system. Histochemical studies have shown that nitric oxide synthase is identical to reduced nicotinamide adenine dinucleotide phosphate (NADPH) diaphorase. Histochemical staining with NADPH diaphorase has been widely used to study nitrergic innervation of the gastrointestinal tract, but fresh tissue is considered a prerequisite for satisfactory results. The purposes of this study were to evaluate whether whole-mount specimens of human bowel obtained after death are suitable for histochemical staining with NADPH diaphorase and to compare the staining properties with those of specimens of resected bowel from patients with Hirschsprung's disease METHODS: Whole-mount preparations of the myenteric plexus were examined using NADPH diaphorase histochemical staining of bowel specimens obtained at autopsy from 18 pediatric subjects (31 specimens). Fresh tissue was also obtained from the colon of four patients with Hirschsprung's disease. The staining properties of postmortem specimens were assessed in relation to the postmortem time before fixation (<12 hours, 13-24 hours, or 25-48 hours) and were compared with those of specimens of ganglionic bowel from patients with Hirschsprung's disease. Specimens of aganglionic bowel were also stained and examined. RESULTS: Strong NADPH diaphorase staining was achieved in 26 of the 31 postmortem bowel specimens, including all specimens from patients who underwent autopsy 25 to 48 hours after death. Staining properties were similar to those obtained in ganglionic bowel specimens from patients with Hirschsprung's disease. In aganglionic bowel the normal myenteric plexus meshwork was absent and was replaced by weakly staining nerve fibers. CONCLUSION: Histochemical staining with NADPH diaphorase is a robust technique suitable for use with whole-mount preparations to demonstrate nitrergic innervation in motility disorders such as Hirshsprung's disease. The technique may be used with both fresh tissue and specimens obtained up to 48 hours after death.

Child↗

PubMed1997

Historical aspects of congenital diaphragmatic hernia

The first description diaphragmatic hernia appeared in 1575. In 1848, Bochdalek described congenital diaphragmatic hernia (CDH) occurring through a posterolateral defect. Successful surgical treatment of CDH in an infant was first performed in 1902, whereas the first neonate operated within 24 hours of life was reported in 1946. However, early surgery did not improve survival rates and the mortality was in the region of 50%. One reason for this was that more neonates underwent surgery who previously would have died without the repair of the CDH. Pulmonary hypoplasia and pulmonary hypertension were early recognised as important reasons for the high mortality rate. In recent years, an enormous effort has been made by research groups all over the world to describe the pathogenesis and pathophysiology of CDH, and apply these findings to clinical practice. Attempts have been made to define prognostic factors. Extracorporeal membrane oxygenation (ECMO) has produced encouraging results. Fetal surgical therapy remains an option in selected cases despite huge technical and ethical problems. Recently, several new therapeutic methods have been suggested, such as high frequency oscillatory ventilation, partial liquid ventilation, nitric oxide inhalation, surfactant therapy, and fetal tracheal ligation. However, more experience is required before the value of these approaches is clear. Despite these efforts, the mortality remains unacceptably high. The challenge for the future is to continue development of therapeutic approaches in order to improve survival of neonates with CDH.

Journal Article↗

PubMed1997

Age-related changes in innervation in hypertrophic pyloric stenosis.

BACKGROUND/PURPOSE: Recent reports have identified abnormal innervation of the circular muscle layer involving the fine intramuscular nerve fibers in hypertrophic pyloric stenous (HPS). HPS presenting after 3 months of age is rare. The aim of this study was to determine the distribution pattern of nerve fibers in the smooth muscle layers in HPS and correlate this with age at presentation. METHODS: Full-thickness pyloric muscle biopsy specimens were obtained from eight patients with HPS (five age 3 to 5 weeks, two age 3 months, and one age 7 months) and five controls with normal pylorus (age 5 days to 3 years). All specimens were stained with monoclonal antibody to the neural cell adhesion molecule (NCAM) using immunohistochemistry. RESULTS: There were many NCAM-positive nerve fibers in the circular and longitudinal muscle layers in the controls. No NCAM positive nerve fibers were seen in the circular or longitudinal muscle layers in the five cases of HPS in which the patients were less than 5 weeks old. In the two cases in which the patients were 3 months old, occasional NCAM-positive nerve fibers were seen in the circular layer, and moderate numbers of NCAM positive fibers were seen in the longitudinal muscle layers. Moderate numbers of NCAM-positive nerve fibers in the circular muscle layer and many NCAM positive nerve fibers in the longitudinal muscle layers were identified in the 7-month-old HPS patient. CONCLUSIONS: The data suggest that the pyloric muscle lacks innervation in the young HPS infant. Whereas, at 3 months of age the hypertrophied pyloric muscle is partially innervated, and at 7 months of age the pyloric muscle has practically normal innervation.

Age Factors↗

PubMed1997

Prerectal peritoneal pouch with herniating small bowel -- a unique cause of severe constipation in a child.

We report a 14-year-old boy with severe constipation with a four-year history of no spontaneous bowel movements. Defecography, with synchronously administered barium enema and barium meal, showed that the small bowel could slide down in front of the rectum obstructing defecation. He was operated and a prerectal peritoneal pouch was found, which was resected. Postoperatively he has had daily spontaneous bowel movements. We have found no similar case described in the literature.

Adolescent↗

PubMed1997

Historical aspects of congenital diaphragmatic hernia.

Female↗