Malrotation in Congenital Diaphragmatic Hernia: Is It Really a Problem?

INTRODUCTION: Congenital diaphragmatic hernia (CDH) has been associated with other congenital malformations, such as intestinal rotation abnormalities. However, there is no standard evaluation and treatment of intestinal rotation during initial CDH repair. This study evaluates intestinal malrotation in CDH patients. MATERIALS AND METHODS: All patients with a CDH treated in a high-volume center between 2000 and 2015 were retrospectively evaluated. Demographics, gastrointestinal characteristics, surgical treatment, and abdominal outcomes (acute surgery, small bowel obstruction, and volvulus) were described. RESULTS: A total of 197 CDH patients were surgically repaired. In 76 (39%) patients, a malrotation was described at initial CDH repair, in 39 (20%) patients, a normal rotation, but 82 patients (42%) had no report on intestinal rotation. During follow-up (range: 2-16 years), 12 additional malrotations were diagnosed, leading to a prevalence of at least 45% (n = 88). These were mostly diagnosed due to acute small bowel obstruction, of which three had a volvulus. Of the 12 later diagnosed malrotations, 58% required acute surgery, compared to 3% of the 76 initially diagnosed patients (p < 0.001). CONCLUSION: Malrotation is associated with CDH, with a prevalence of at least 45% and in 21% a normal intestinal rotation. "Missed" malrotations have a higher risk on need for acute surgery later in life.

Clinical and pathological comparisons of adnexal torsion between pregnant and non-pregnant women.

AIM: We evaluated and compared the clinical and pathological differences between pregnant and non-pregnant women with adnexal torsion. METHODS: We retrospectively reviewed 239 women with adnexal torsion from January 2006 to December 2015 in a tertiary hospital. The clinical and pathological differences between pregnant and non-pregnant women who underwent surgery for adnexal torsion were analyzed. RESULTS: The most common pathologies were corpus luteum cysts in pregnant women and dermoid cysts in non-pregnant women. Eight of the pregnant women (24.2%) had a history of exogenous ovarian stimulation, and their episodes were only caused by corpus luteum or a stimulated ovary. In pregnant women, 72.7% of the torsion occurred before the 14th week of gestation. CONCLUSION: The common pathology causing adnexal torsion was different, depending on the pregnancy status. Exogenous ovarian stimulation increases the risk of adnexal torsion, and the majority of episodes occurred in the first trimester in pregnant women.

Femoral Version and Tibial Torsion are Not Associated With Hip or Knee Arthritis in a Large Osteological Collection.

BACKGROUND: Tibial torsion and femoral anteversion are common rotational abnormalities in children, and their courses are most often benign and self-resolving. Although neither usually requires surgical treatment, the decision to perform a derotational osteotomy is usually based on the degree of functional impairment. Neither condition is thought to influence the development of osteoarthritis of the hip or knee; however, to date there have been no large scale studies confirming this. METHODS: Tibial torsion and femoral version in 1158 cadaveric tibiae and femora were measured using a camera setup based on previously described radiographic landmarks. Any specimens with obvious traumatic, rheumatic, or metabolic abnormalities were excluded. Degenerative joint disease of the hip and knee were each graded from 0 to 6. Correlations between tibial torsion, femoral version, age, race, and sex with osteoarthritis of the hip and knee joints were evaluated with multiple regression analysis. RESULTS: The mean and SDs of tibial torsion and femoral anteversion were 7.9±8.8 and 11.4±12.0 degrees, respectively. African Americans had significantly increased tibial torsion (5.1±8.7 vs. 9.2±8.5 degrees, P<0.0005) and greater femoral anteversion (14.1±11.9 vs. 10.2±11.8 degrees, P<0.0005) compared with whites. The average grades for hip and knee osteoarthritis were 3.1±1.4 and 2.7±1.4. Using multiple regression analysis neither tibial torsion nor femoral version were independent predictors of hip or knee arthritis (P>0.05 for all). DISCUSSION: This study confirmed previously reported differences in the rotational profiles between races and sexes. However, neither tibial torsion nor femoral anteversion had a significant influence on the development of arthritis of the hip or knee. CLINICAL RELEVANCE: These results support the practice of treating tibial torsion and femoral anteversion based on the symptomatology of the patient. Parents of asymptomatic children can be reassured that long-term consequences are unlikely.

Antenatally diagnosed right-sided stomach (dextrogastria): A rare rotational anomaly.

AIM: Antenatal detection of right-sided stomach (dextrogastria) is rare, and its significance in regards to intestinal rotation is unclear. We aimed to review all cases of antenatally-diagnosed dextrogastria in our regional fetal medicine unit over 10years. METHODS: A retrospective case-note review of patients identified from a prospectively-maintained database was performed. RESULTS: Twenty cases of antenatally-diagnosed dextrogastria were identified from 2004 to 2014. There were 8 terminations and 1 intra-uterine death. One patient has no post-natal information obtainable. Ten infants were live-born, and 2 died secondary to cardiac disease in the neonatal period. All had significant cardiac/vascular anomaly on postnatal assessment, including the 3 neonates in whom dextrogastria was the only antenatal finding. Two neonates developed bilious vomiting and underwent Ladd's procedure. Operative findings were dextrogastria/malrotation in both. A third child had gastro-oesophageal reflux, and contrast demonstrated stable duodenal/midgut position. This child has not developed symptoms attributable to malrotation and not undergone surgery. All 3 of these infants had asplenia or polysplenia and were managed with antibiotic prophylaxis/immunisation. Five children in the series were not investigated for malrotation and have not come to surgical attention (one is known to be asplenic). CONCLUSION: Antenatally-detected dextrogastria, even if apparently isolated, was always associated with postnatal significant cardiovascular anomaly, splenic abnormality or situs inversus. This may be important for antenatal counselling. We currently recommend postnatal echocardiography and splenic assessment, but reserve GI investigation/intervention for symptomatic malrotation owing to potential significant cardiac comorbidity.

Prenatal and postnatal ultrasonographic findings of the torsioned ovarian cyst: a case report and brief literature review.

Cystic and solid tumors of the ovary are rare during the newborn period and infancy. We present the case of a term female infant born to a mother of 28 years of age and found to have a cystic abdominal mass through prenatal sonographic evaluation in the third trimester. The complex cyst was also demonstrated by postnatal abdominal ultrasonography. Laparotomy revealed a large cystic mass with a torsed right ovary. Pathologic examination of cyst revealed hemorrhagic necrosis with ovarian torsion.

Esophageal atresia and malrotation: what association?

INTRODUCTION: Esophageal atresia/tracheo-esophageal fistula (EA/TEF) has an incidence of approximately 1:3,500. The incidence of malrotation is thought to be 1:200-500. We attempted to define the incidence of a combination and discuss the implications. METHODS: This was a retrospective review of all patients admitted to a single institution with a diagnosis of EA or EA/TEF or TEF between April 1981 and January 2013. Patients were included if the position of the duodeno-jejunal flexure (DJF) was determined by upper GI contrast study (UGIS), surgery or post-mortem. RESULTS: Case notes were reviewed for 235 patients. In the EA type A group, 3/28 (11 %; 95 % CI 3.7-27.2 %) had malrotation, significantly higher than the reported incidence of malrotation in the general population (p = 0.0008). All three patients in this group were symptomatic with one patient found to have a volvulus at emergency surgery. In the type C group, 6/196 (3 %, 95 % CI 1.4-6.5 %) had malrotation, significantly higher than the incidence reported for the general population (p = 0.0033) but not significantly different to that of the type A group (p = 0.0878). There were no patients with malrotation identified in any other EA/TEF type. In total, 9/235 (3.8 %; 95 % CI 2.0-7.2 %) patients with EA had malrotation, significantly higher than the 5/1,050 (0.48 %) reported for the general population (p = 0.0002). CONCLUSION: There is a high incidence of malrotation in patients with pure EA. In the type A group an attempt to identify the DJF position at gastrostomy siting and/or performance of UGIS in the neonatal period should be undertaken. There should also be a low threshold for UGIS in all EA/TEF patients.

Penile torsion repair in children following a ladder step: simpler steps are usually sufficient.

OBJECTIVE: To evaluate the correction of different degrees of penile torsion following a ladder step so that simpler steps are used whenever possible. This can avoid the morbidity and complications of complex procedures. PATIENTS AND METHODS: Cases of congenital and acquired penile torsion were repaired on a ladder step basis irrespective of the degree of torsion, starting with degloving and skin realignment, then a dorsal dartos flap and finally corporopexy. The torsion is checked with artificial erection after each step, and if corrected completely then the next step(s) is omitted. RESULTS: Twenty-five cases of penile torsion (30-180°) were repaired over a 4-year period. Three cases were corrected by degloving only, 12 by degloving and skin realignment, five by a dartos flap and four required a corporopexy. Postoperative complications included five cases of penile edema, one case of hematoma and one case of dorsal skin gangrene. Residual torsion of <15° occurred in three cases. No cases required redo surgery. CONCLUSION: A ladder step approach is a good option for penile torsion repair, starting with simpler techniques until complete correction is achieved. There is no need to plan a complex procedure in advance.

Polyorchidism: a torted right-sided supernumerary testis.

Polyorchidism is a rare congenital anomaly defined by the presence of more than two histologically proven testes. The commonest variant is triorchidism, the supernumerary testis being commonly reported on the left side. Most cases of polyorchidism are found incidentally in association with undescended testis, hydrocele, hernia or torsion. We report a right-sided triorchidism in a 15-year-old boy found at time of groin exploration for an irreducible right inguinal hernia.

Acute appendicitis presenting as acute gastritis in an adult patient with undiagnosed congenital gut malrotation: a case report.

BACKGROUND: We report a case of an atypical presentation of acute appendicitis in an adult due to an undiagnosed congenital gut malrotation. OBJECTIVE: The obvious benefit of observation with serial examinations followed by the use of computed tomography (CT) is discussed in aiding in the diagnosis for atypical presentations of appendicitis. CASE REPORT: A 45-year-old man who presented with epigastric pain and vomiting was diagnosed with acute left-sided appendicitis on CT scan. The patient's appendix was located in the left lower quadrant of the abdomen due to undiagnosed congenital gut malrotation, thus resulting in an atypical presentation of a common illness. CONCLUSION: Observation with serial examinations, followed by CT scanning if indicated, is an invaluable strategy to use in diagnosing atypical presentations of common pathology.

Dorsal dartos flap rotation versus suturing tunica albuginea to the pubic periosteum for correction of penile torsion: a prospective randomized study.

PURPOSE: To compare the results of dorsal dartos flap rotation versus suturing tunica albuginea to the pubic periosteum for correction of congenital penile torsion. PATIENTS AND METHODS: Seventeen patients with congenital penile torsion were evaluated and managed from 2006 to 2010. On a random basis, 9 patients underwent surgical repair using a dorsal dartos flap rotation technique (group 1), and 8 patients by suturing tunica albuginea to the pubic periosteum (group 2). All were followed up every 6 months. RESULTS: The mean age in group 1 was 52.3 ± 24.8 months, while in group 2 it was 53.4 ± 33.6 months. Mean follow up was 22.3 ± 10.2 and 21.5 ± 7.2 months in group 1 and group 2 respectively. Torsion was corrected in 6 (66.7%) patients of group 1; 3 (33.3%) had residual torsion < 10°. In group 2, correction was achieved in 6 (75.0%) patients and 2 (25.0%) had residual torsion < 10°. The mean operative time was significantly less for group 1 than for group 2: 45.3 ± 7.1 and 74.5 ± 5.3 min, respectively (P < 0.0001). CONCLUSIONS: In short-term prepubertal follow up, both dorsal dartos flap rotation and suturing tunica albuginea to the pubic periosteum are effective for correction of congenital penile torsion. The former technique is easier to perform as it requires much less dissection.

Right paraduodenal hernia leading to bowel strangulation.

Up to half of all internal hernias are caused by paraduodenal hernia, a rare congenital midgut malrotation that accounts for less than 1% of all intestinal obstructions. The diagnosis may arise from an incidental finding on abdominal imaging or the patient may present with abdominal pain, vomiting, and obstipation. Early recognition and management of this disease entity are keys because serious complications such as bowel ischemia and infarction may result from a delay in diagnosis. We present a case involving a 14-year-old boy with gangrenous small bowel secondary to right paraduodenal hernia.

Correction of penile torsion by mobilization of urethral plate and urethra.

OBJECTIVE: To assess the feasibility of correction of torsion by mobilization of the urethral plate with the corpus spongiosum and the proximal urethra. PATIENTS AND METHODS: Of 27 cases of congenital penile torsion, 18 had hypospadias, seven were chordee without hypospadias, and two were isolated penile torsion. Age of patients varied from 2 to 26 years (mean 6 years, 8 months). Correction of torsion was performed: (1) penile skin de-gloving; (2) mobilization of the urethral plate with the corpus spongiosum up to the corona; (3) mobilization of the proximal urethra up to the perineum; and (4) mobilization of the hypoplastic urethra/urethral plate into the glans. Tubularized incised plate urethroplasty with spongioplasty was done in cases of hypospadias, as compared to spongioplasty alone in cases of chordee without hypospadias. RESULTS: Degree of torsion varied from 45 to 180 degrees (mean 68.70); 74% of the patients had left and 26% had right penile torsion. Correction of torsion was possible by penile de-gloving (4%), mobilization of urethral plate and spongiosum (26%), mobilization of proximal urethra (22%), and mobilization of urethral plate/hypoplastic urethra with spongiosum into glans (48%). CONCLUSIONS: Extended urethral mobilization corrected penile torsion in almost all cases. The technique is simple, safe, reproducible and effective for correction of both torsion and chordee.

Antenatal diagnosis of intestinal malrotation on fetal MRI.

We report a case of intestinal malrotation without any associated GI tract complications diagnosed antenatally by fetal MRI. Antenatal US revealed a midline stomach. Subsequent fetal MRI confirmed the midline stomach and, in addition, revealed all loops of small bowel to the right of the midline and all large bowel to the left. All these features were consistent with intestinal malrotation. There was no abnormal bowel wall thickening, bowel dilatation, ascites or polyhydramnios. To our knowledge, this is a unique case of intestinal malrotation without associated GI tract complications diagnosed antenatally on fetal MRI.

Congenital short bowel syndrome: a case report and review of the literature.

Congenital short bowel syndrome (SBS) is a rare condition of the newborn, with several reports demonstrating high mortality. A six-week-old boy presented with chronic diarrhea and failure to thrive. An upper gastrointestinal endoscopy showed a straight duodenum, and multiple small bowel biopsies were histologically normal. An upper gastrointestinal series showed malrotation. At laparotomy, the small bowel was 50 cm in length, confirming the diagnosis of congenital SBS. Parenteral nutrition was initiated and enteral feeding with an amino acid-based formula containing long-chain fatty acids was introduced early and gradually advanced. At the last follow-up examination at 24 months, he was thriving on a regular diet, with normal growth and development. Long-term survival of children with congenital SBS is now possible if enteral feeds are introduced early to promote intestinal adaptation, with subsequent weaning off parenteral nutrition.

Torsion of the penis in adults: prevalence and surgical correction.

INTRODUCTION: Torsion of the penis is a condition where the penis rotates around its longitudinal axis, whether congenital or acquired. Extreme degrees may provoke a cosmetic complaint. AIM: We describe surgical correction of congenital torsion of the penis in adults, and its prevalence among a special patient group. MAIN OUTCOME MEASURES: Success and ease of surgical repair. METHODS: Sixteen cases with congenital torsion were operated upon, by counter-rotation, using a dartos flap in eight cases, and skin realignment in the other eight. The prevalence of congenital torsion was examined in 12,307 patients attending two andrology clinics. RESULTS: Full correction was achieved in all cases. Skin realignment was easier and faster than dartos flap, and was equally effective. Congenital torsion was present in 11.993% of the epidemiologic study group, mild in 80%, moderate in 15%, and severe in 5%. Only 2.2% was bothered by the condition. CONCLUSION: Torsion of the penis is not uncommon but rarely provokes a complaint. Surgical repair by degloving and skin realignment is effective and easy. Dartos flap technique may be utilized if the former is inadequate.