Congenital segmental dilatation of the colon in a newborn: a radiologic perspective of an unusual entity.

A newborn with congenital segmental dilatation of the intestine affecting the colon is presented. This rare condition, unrelated to Hirschsprung's disease, may affect any portion of the bowel and is characterized by focal dilatation of a segment of bowel flanked by normal proximal and distal bowel. While reported in the surgical literature, congenital segmental dilatation of the intestine has not been reported in the pediatric radiology literature even though pediatric radiologists may be the first to encounter imaging suggesting the diagnosis. We therefore present the characteristic imaging findings, including abdominal radiographs and images from a contrast enema, and discuss the clinical presentation, pathology findings, associations, treatment, and prognosis of congenital segmental dilatation of the intestine to increase awareness of this unusual diagnosis.

Diffuse Lamellar Keratitis in a Patient Undergoing Collagen Corneal Cross-Linking 18 Years After Laser In Situ Keratomileusis Surgery.

PURPOSE: To report a case of diffuse lamellar keratitis (DLK) after corneal collagen cross-linking in an eye with a remote history of laser in situ keratomileusis (LASIK) surgery. METHODS: This is a case report and literature review. RESULTS: This report describes the development of unilateral stage IV DLK in a patient who underwent bilateral corneal cross-linking for corneal ectasia 18 years after LASIK surgery. The patient was treated with high-dose topical steroids that were tapered over 1 month and multiple flap lifts. The ultimate best-corrected visual outcome was 20/60. CONCLUSIONS: DLK is a potential sight-threatening complication of refractive surgery that can occur at any time in the postoperative period, even years after the procedure. Undergoing a subsequent corneal procedure that may disrupt or promote inflammation within the surgical flap-stromal interface, such as corneal collagen cross-linking, is a recognized risk factor for the development of DLK. This case suggests that patients with any history of LASIK surgery undergoing corneal cross-linking or other lamellar corneal surgeries may benefit from closer follow-up (eg, daily) than patients with no history of LASIK.

CT-guided nephrostomy-An expedient tool for complex clinical scenarios.

INTRODUCTION & OBJECTIVES: Percutaneous nephrostomy [1] has emerged as a pivotal approach in the therapeutic management of the obstructed urinary tract. A consecutive incorporation of ultrasonic and radiographic guidance, the approach experienced an almost ubiquitious distribution while most centers currently applying either one or both of these tools jointly. However, success of ultrasound-guidance is limited in obese patients and non-dilated uropathy. In turn, fluoroscopy usually requires an opacification of the urinary collecting system by intravenous or antegrade contrast media injection, which might be harmful for already impaired renal function, raise intrapelvic pressure and augment the risk of sepsis and hemorrhage. CT-guided PCN aids in overcoming these limitations. In the current study, we present the experience of a tertiary referral center with this technique. MATERIALS & METHODS: Epidemiological and clinical data of all patients treated with a CT-guided PCN of native kidneys at the University Hospital Frankfurt between October 2003 and October 2013 were retrospectively collected from the patient charts. Procedural parameters including radiological aspects, technical and therapeutic success, complication and mortality rate have been analyzed statistically. RESULTS: In total, 140 PCN procedures have been performed in 77 patients with a median age of 69 (± 13). The median body mass index was 27 with 66.6% of patients being overweight or obese. Charlson comorbidity index was 7 ranging 0-16. Indications for PCNs were obstructive uropathy (62.9), urine extravasation (22.9%), urinary tract fistulas (11.4%) and technical reasons (2.8%). In 68.8% of patients, initial diagnosis was malignancy. 56.4% of kidneys were non-dilated before puncture. In 78.4% prone position, otherwise supine oblique position (17.3%) or supine position (4.3%) was used. 71.4% of PCNs were carried out solely under local anesthesia. Technical success has been achieved in 90% with a complication rate of 3.6% (all grade minor B) and was not significantly different between dilated and non-dilated kidneys. 42.9% of fistulas and 64.3% of urinary tract leakages, healed after PCN placement. 30 days mortality rate was 5.2% without being directly associated with the PCN procedure itself. CONCLUSION: CT-guided PCN is a feasible approach associated with low morbidity. It is particularly useful in complex clinical scenarios e.g. critically ill, newly operated or obese patients as well as non-dilated kidneys. Moreover, it represents a minimally-invasive option for treating leakages and fistulas of the urinary tract.

[Granulomatous lobular mastitis associated with mammary duct ectasia: a clinicopathologic study of 32 cases with review of literature].

OBJECTIVE: To study the clinicopathologic features of granulomatous lobular mastitis and mammary duct ectasia. METHODS: The clinicopathologic data from August 2005 to May 2013 of 32 cases of granulomatous lobular mastitis and mammary duct ectasia were retrospectively reviewed. RESULTS: The age of patients ranged from 26 to 45 years. Two patients had no history of delivery. Fourteen patients had no history of lactation or lactational disorder in the lesional side. Most of the remaining patients had history of breast feeding. Gross examination showed that the lesions were poorly circumscribed and varied from 3 to 12 cm in greatest dimension. Tiny abscess cavities, ranging from 0.1 to 0.5 cm in diameter and containing light yellowish to greyish secretion, were demonstrated. Histologic examination showed granuloma formation and ductal dilatation. Eleven patients had received antibiotic treatment. Twelve cases were complicated by sinus formation related to skin incision and drainage. The duration of follow-up ranged from 5 to 90 months. Three cases showed ipsilateral recurrence and 3 cases had similar pathology in the contralateral breast. Four patients defaulted follow-up. CONCLUSIONS: Granulomatous lobular mastitis is associated with mammary duct ectasia. Accurate pathologic diagnosis is prudent for clinical management and control of local recurrence.

Surgical considerations regarding giant dilations of the perivascular spaces.

OBJECT: Dilations of brain perivascular spaces (PVSs), also known as Virchow-Robin spaces, are routinely identified on magnetic resonance imaging studies of the brain and recognized as benign normal variants. Giant dilations occur only rarely and can be easily misdiagnosed as central nervous system tumors. The relevant surgical literature was reviewed to help establish indications for surgical intervention in these typically benign lesions. METHODS: Giant dilations of the PVSs in 12 patients who had undergone surgery for several different indications were identified. Both clinical and radiographic presentations of these patients were reviewed along with the surgical procedures. CONCLUSIONS: Dilations of the PVSs can become giant lesions that may necessitate surgical intervention to relieve mass effect or hydrocephalus. The relationship of these lesions to neurological symptoms such as tremor and seizures remains unclear.

Surgical management of chronic unremitting constipation and fecal incontinence associated with megarectum: A preliminary report.

BACKGROUND/PURPOSE: Functional constipation with associated fecal incontinence responds poorly to medical management once megarectum has developed. The authors describe resecting the dilated rectum and inserting a cecostomy button for antegrade enemas in this difficult condition. METHODS: Four children, ages 9 to 15 years, with a history of unremitting constipation and fecal incontinence were referred for evaluation after not responding to medical management. All patients had exhibited normal lumbosacral magnetic resonance images (MRI) and open rectal biopsies; however, all 4 patients had a megarectum on contrast enema. In addition, anorectal manometry was consistent with functional fecal retention. The dilated rectum was resected by anastamosing the nondilated sigmoid colon to the distal rectum, and a standard gastrostomy button was inserted into the cecum for antegrade enemas. Mean follow-up was 35 months (range, 8 to 60 months). RESULTS: Constipation and incontinence resolved within 6 months in all patients, and all children remained continent without the aid of cathartic agents. There were no postoperative episodes of fecal impaction. The only complication was antibiotic-associated diarrhea in 1 patient. Cecostomy buttons were removed at 1 year postplacement in all 4 patients with continued success. Three patients underwent repeat anorectal manometry; all 3 had normal rectal sensory threshold volumes and anorectal inhibitory reflexes. Barium studies also were obtained in the 3 patients without evidence of recurrent rectal dilation. CONCLUSIONS: Refractory constipation and incontinence associated with megarectum may be amenable to surgical intervention in selected patients. The authors' limited experience suggests that proctectomy and button cecostomy is an effective treatment option that improves the quality of life in these patients. Furthermore, proctectomy alone may be curative.

Clinical and pathophysiologic correlates of 1064-nm Nd:Yag laser treatment of reticular veins and venulectasias.

BACKGROUND: The goal of sclerotherapy, laser therapy, and intense pulsed-light therapy is to produce long-term, cosmetically significant elimination of disfiguring leg veins. This study examines the histologic and clinical effects of using a 1064-nm Nd:YAG laser system on lower extremity vessels. DESIGN: A single treatment using the following parameters: wavelength, 1064 nm (multiple synchronized pulsing); spot size, 6 mm; pulse duration, 14 milliseconds (single pulse); and fluence, 130 J/cm(2). SETTING: Private dermatology practice. PATIENTS: Thirteen women (mean age, 38.5 years) with blue venulectasia, 0.5 to 1.5 mm in diameter (class 2), and reticular veins, 1.5 to 3.0 mm in diameter (class 3), on the thighs. MAIN OUTCOME MEASURES: Examination of treated and untreated areas by 2 masked observers using macrophotography (1, 2, 3, and 6 months after treatment), Doppler, and optical chromatographic changes. Findings from three 2-mm punch biopsies from treated (immediately and 4 weeks after treatment) and untreated sites. Routine histologic examination; special stains (for elastic and connective tissue and for mucopolysaccharides); and immunohistochemical analysis for expression of the heat shock protein hsp70, tie2 (an endothelial cell-specific receptor tyrosine kinase), and transforming growth factors beta1 and beta2. RESULTS: Eight patients (62%) manifested 75% to 100% clearing of treated vessel surface area. Treated areas revealed perivascular hemorrhage, thrombi, fragmentation and homogenization of elastic fibers, and eosinophilia of vessel walls. Expression of hsp70 and transforming growth factor beta was increased in treated vessels. CONCLUSIONS: Our data confirm the effectiveness of 1064-nm Nd:YAG laser treatment in clearing dilated lower extremity veins, probably by heat-induced vessel damage and subsequent fibrosis. Maintenance of clearing was achieved for up to 6 months. However, the presence of recanalized thrombi in some of the specimens suggests the potential for long-term vessel reappearance.

Antenatal finding of intestinal obstruction caused by isolated segmental jejunal dilatation: a case report.

The authors report a case of segmental jejunal dilatation that was discovered antenatally and manifested clinically as lower obstruction in a newborn boy. The antenatal ultrasound findings at 29, 30, and 31 weeks' gestation showed a constant hypoechogenic image (6 x 3 cm in diameter) localized to the right flank of the fetal abdomen, thought to be situated in the right colon. A prenatal diagnosis of incomplete right colonic stenosis was considered. In the immediate postnatal period, there were clinical manifestations of intestinal obstruction; however, results of a contrast enema and rectal biopsies were normal. An upper gastrointestinal contrast study showed a dilated jejunal loop situated approximately 15 cm from the ligament of Treitz. During surgery, a large dilated jejunal loop (7 cm in diameter, 15 cm in length) was found and resected. Histopathologic examination confirmed the diagnosis of segmental jejunal dilatation. This is the first antenatal illustration of this rare pathology, and it supports the hypothesis of a congenital origin for this anomaly.

Contemporary management of meconium ileus.

Meconium ileus was noted as an early manifestation of cystic fibrosis in 60 neonates between 1972 and 1991. There were 20 girls and 40 boys. A family history of cystic fibrosis was present in six children. Twenty-five neonates had uncomplicated meconium ileus due to inspissated meconium within the terminal ileum. Thirty-five neonates presented with 56 complications of meconium ileus, including volvulus (n = 22), atresia (n = 20), perforation (n = 6), and giant cystic meconium peritonitis (n = 8). Clinical presentation included abdominal distension, bilious vomiting, and failure to pass meconium. In two recent cases, prenatal ultrasonography detected a mass with proximal bowel distension indicative of cystic meconium peritonitis. Mechanical bowel obstruction in the other neonates was diagnosed from plain abdominal radiographs and barium enema. Ten patients with uncomplicated meconium ileus were successfully treated with a diatrizoate meglumine (Gastrografin) enema. The remaining 15 patients required a laparotomy, with 9 treated by bowel resection and enterostomy and 6 recent cases managed with enterotomy and irrigation. Complicated cases were managed by bowel resection and anastomosis (n = 15) or enterostomy (n = 20). Survival at 1 year was 92% in patients with uncomplicated meconium ileus and 89% for those with complicated meconium ileus. The therapy of choice for uncomplicated meconium ileus is nonoperative Gastrografin enema, with enterotomy and irrigation reserved for enema failures. Complicated cases require exploration and, in the absence of giant cystic meconium peritonitis, are usually amenable to bowel resection and primary anastomosis.

Awake fibreoptic intubation for a rare cause of upper airway obstruction--an infected laryngocoele.

The anaesthetic management of a patient with an infected laryngocoele is presented. The relevance of this condition to the anaesthetist is discussed.

[Congenital segmental dilatation of the colon]. / Dilatación segmentaria congénita del colon.

Segmental dilatation of the colon is a rare congenital entity that cause chronic constipation in children. A new case is presented and another 9 former cases described in the literature are reviewed. We think that this entity may be diagnosed before the operation, if it is kept in mind. So, the barium enema and rectal biopsy are of paramount importance. The resection of the dilated ++ segment is the treatment of choice.

[New surgical technic in megarectum]. / Nouvelle technique chirurgicale pour mégarectum.

The sphinctero-myectomy as a surgical treatment of megarectum has been generally advocated, but its results are not always satisfactory. In this paper, a new surgical treatment will be presented. Ten cases with idiopathic chronic constipation presenting megarectum in barium enema underwent this operation. After anal dilatation, incision was made in width of 2 cm at 6 o'clock on the dentate line. The blunt split between internal and external sphincter muscles was advanced to 6 cm orally. The all layers of superior part of anus and lower part of rectum in length of 5 cm were clumped by two forceps in width of 1 cm. Then the all layers in outer side of forceps were cut bilaterally. The rectum on the top of the forceps was pulled through to the dentate line and was anastomosed there. The operative results were evaluated more than one year after the operation. Complete cure was noticed in 7 cases and marked improvement in 3 cases. Our procedure, named as sphinctero-myectomy and plasty can be operation of choice for chronic constipation with megarectum.

Control of bleeding during the Bentall operation.

A simple and effective method to diminish the amount of blood loss during the Bentall operation is described. This method is to connect the perigraft space between the aneurysmal wall and the aortic graft with the right atrium using a small plastic tube.