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Omphalocele, the congenital anomaly of the anterior abdominal wall in the newborn, is a serious condition with a grave prognosis. Sac rupture, occurring in 25% of cases in Ibadan is associated with a high mortality. Although its embryogenesis is now better understood, its underlying aetiology remains obscure. Prematurity and low birth weight have no causal relationship to the condition, but a preponderance among the lower socio-economic groups in Ibadan is observed. Thirty-three cases seen over a 5-year period at the University College Hospital, Ibadan, Nigeria, constitute an unusually high incidence, suggesting that the condition may be aetiologically related to umbilical hernia which is known to be commoner in African than in Caucasian infants. Overall treatment results remain unsatisfactory for all types and mortality is still high. In those centres, particularly in the developing countries with scarce resources, conservative management should be the primary treatment modality for all intact sacs but staged procedures utilizing prosthetic materials should be reserved for the large ruptured sac.

With the present-day development and understanding of anesthetic methods, fluid and electrolyte therapy, antibiotic medications and pediatric care, many congenital anomalies once uniformly fatal are now being successfully treated by emergency operations in the neonatal period. The eight most common of these which demand emergency operation in the immediate postnatal period are esophageal atresia and tracheoesophageal fistula, diaphragmatic hernia with dislocation of the abdominal viscera into the chest, malrotation of the intestine with obstruction, intestinal atresia, meconium ileus, imperforate anus, omphalocele and myelomeningocele.

Although infants born with any of these serious problems often are born prematurely and often have more than one congenital anomaly, survival rates in the surgical treatment of these conditions are steadily improving. Early diagnosis and prompt treatment are the most important factors in the continued improvement of these survival rates.

A case is presented of an amnionic rupture sequence which led to massive fetal ventral herniation and lordoscoliosis. Characteristic ultrasonographic findings of an omphalocele, fetal attachment to the placenta, and the absence of free-floating umbilical cord were observed.

Isolated absence of the ductus arteriosus is extremely rare condition although the ductus arteriosus may be hypoplastic or aplastic in association with other aortic arch anomalies. Authors described a case of isolated agenesis of the ductus arteriosus documented by postmortem examination of a newborn infant who died of pneumonia following operation for a large omphalocele. The heart showed ventricular septal defect. However, no other cardiovascular anomalies were associated in this case. There were three vessels that were taking off from the aorta consisted of the right brachiocephalic artery, left common carotid-artery and left subclavian artery. The anteriorly located pulmonary artery was divided into the right and left pulmonary arteries. There was no connection of vessel between the pulmonary artery and the aorta.

One hundred consecutive cases of confirmed anterior abdominal wall defect, identified prenatally in the Oxford Prenatal Diagnosis Unit over 11 years, were studied. Fifty nine per cent of cases were suspected omphaloceles and 41% suspected gastroschisis. Fifty four per cent of omphaloceles were accompanied by other defects compared with 5% of those with gastroschisis. Overall, 29% of fetuses with omphalocele had an abnormal karyotype, and of those with another abnormality identified on scan (excluding four cases with no karyotype performed), 54% had an abnormal karyotype. Of the 27 cases with suspected isolated omphalocele, 14 were live born, all of whom have survived. If the 11 whose parents opted for termination of pregnancy are excluded, survival to birth was 88%. Six of the suspected isolated omphaloceles have Beckwith Wiedemann syndrome (BWS). Eight (57%) of the live born babies with omphaloceles had major problems up to the age of 2, but only one (7%) has long term major problems. This child has BWS and is deaf.
 Of the 39 cases of suspected isolated gastroschisis, 33 (85%) pregnancies resulted in live birth and one in neonatal death after surgery. Survival rate (excluding terminated pregnancies) was 97%. Gastroschisis was associated with a younger maternal age than omphalocele (p<0.001) and lower birthweight centile (p<0.01).
 Fifteen per cent of the gastroschisis babies had major problems up to the age of 2 years and 12% long term developmental problems. Ninety three per cent of the omphalocele babies and 88% of those who had gastroschisis have no long term problems. Over the study period there have been major changes in scanning equipment and expertise. Since 1991 no woman with a suspected isolated lesion has opted for termination of pregnancy.



Introduction

Iatrogenic damage to the seminal tract is one of the causes of obstructive azoospermia, which can be an indication for reconstruction surgery. We present a case of obstructive azoospermia as an unusual complication after neonatal herniorrhaphy of an omphalocele.

Case presentation

A 30-year-old Japanese man was diagnosed with obstructive azoospermia. He had undergone herniorrhaphy of an omphalocele immediately after birth. Reconstruction surgery of both seminal tracts was performed to pursue the possibility of naturally achieved pregnancy. Intra-operative findings demonstrated that both vasa deferentia were interrupted at the internal inguinal rings, although the abdominal end of the right vas leading to the seminal vesicle was found in the abdominal cavity. The discharge from the stump of the testicular end had no sperm, although the right epididymal tubules were dilated with motile sperm. Therefore, we performed right-sided vasovasostomy in the internal inguinal ring and ipsilateral epididymovasostomy simultaneously.

Conclusion

To the best of our knowledge, this is the first report describing obstructive azoospermia as an unusual complication of herniorrhaphy of an omphalocele. It is important to pay attention to the existence of seminal tracts in such surgery as well as in inguinal herniorrhaphy.

Fetal ultrasound combined with semiquantitative measurements of alpha-fetoprotein in maternal serum was used for early detection of neural tube defects and omphalocele in 10 147 pregnancies. The accurate assessment of gestational age, obtained by ultrasound, facilitated evaluation of alpha-fetoprotein concentrations in selecting cases for amniocentesis. The advantage of screening with two independent methods is suggested by the finding that eight out of 10 cases with malformations (spina bifida, encephalocele, anencephalus, omphalocele) were detected when both methods were used. Screening by routine ultrasound alone detected only four malformations and by measurement of alpha-fetoprotein alone only seven. The results suggest that, in a low risk population, ultrasound should be combined with the measurement of alpha-fetoprotein in screening for neural tube defects. Measurement of alpha-fetoprotein is indispensable in detection of the small neural tube defects, where the fetus would survive with severe sequelae. The semi-quantitative analysis of alpha-fetoprotein that may be used in combination with ultrasound examination is of negligible cost.

Introduction. A rare case of histologically proven placental mesenchymal dysplasia (PMD) with fetal omphalocele in a 22-year-old patient is reported. Material and Methods. Antenatal ultrasound of this patient showed hydropic placenta with a live fetus of 17 weeks period of gestation associated with omphalocele. Cordocentesis detected the diploid karyotype of the fetus. Patient, when prognosticated, choose to terminate the pregnancy in view of high incidence of fetal and placental anomalies. Subsequent histopathological examination of placenta established the diagnosis to be placental mesenchymal dysplasia. Conclusion. On clinical and ultrasonic grounds, suspicion of P.M.D. arises when hydropic placenta with a live fetus presents in second trimester of pregnancy. Cordocentesis can detect the diploid karyotype of the fetus in such cases. As this condition is prognostically better than triploid partial mole, continuation of pregnancy can sometimes be considered after through antenatal screening and patient counseling. However, a definite diagnosis of P.M.D. is made only on placental histology by absence of trophoblast hyperplasia and trophoblastic inclusions.

Introduction

Uterine rupture is a life-threatening condition both to mothers and fetuses. Its early diagnosis and treatment may save their lives. Previous myomectomy is a high risk factor for uterine rupture. Intestinal adhesion due to previous myomectomy may also prevent early diagnosis of uterine rupture.

Case presentation

A 38-year-old primiparous non-laboring Japanese woman with a history of myomectomy was admitted in her 34th week due to lower abdominal pain. Although the pain was slight and her vital signs were stable, computed tomography revealed massive fluid collection in her abdominal cavity, which led us to perform a laparotomy. Uterine rupture had occurred at the site of the previous myomectomy; however, the small intestine was adhered tightly to the rupture, thus masking it. The baby was delivered through a low uterine segment transverse incision. The ruptured uterine wall was reconstructed.

Conclusion

Intestinal adhesion due to a prior myomectomy occluded a uterine rupture, possibly masking its symptoms and signs, which may have prevented early diagnosis.

Both taking folic acid-containing vitamins around conception and consuming food fortified with folic acid have been reported to reduce omphalocele rates. Genetic factors are etiologically important in omphalocele as well; our pilot study showed a relationship with the folate metabolic enzyme gene methylenetetrahydrofolate reductase (MTHFR). We studied 169 non-aneuploid omphalocele cases and 761 unaffected, matched controls from all New York State births occurring between 1998 and 2005 to look for associations with single nucleotide polymorphisms (SNPs) known to be important in folate, vitamin B12, or choline metabolism. In the total study population, variants in the transcobalamin receptor gene (TCblR), rs2232775 (Q8R), and the MTHFR gene, rs1801131 (1298A>C), were significantly associated with omphalocele. In African-Americans significant associations were found with SNPs in genes for the vitamin B12 transporter (TCN2) and the vitamin B12 receptor (TCblR). A SNP in the homocysteine-related gene, betaine-homocysteine S-methyltransferase (BHMT), rs3733890 (R239Q), was significantly associated with omphalocele in both African-Americans and Asians. Only the TCblR association in the total population remained statistically significant if Bonferroni correction was applied. The finding that transcobalamin receptor (TCblR) and transporter (TCN2) SNPs and a BHMT SNP were associated with omphalocele suggests that disruption of methylation reactions, in which folate, vitamin B12, and homocysteine play critical parts, may be a risk factor for omphalocele. Our data, if confirmed, suggest that supplements containing both folic acid and vitamin B12 may be beneficial in preventing omphaloceles.

Objective. To assess the efficacy of a new suture technique in controlling severe resistant uterine atonic postpartum hemorrhage. Patients and Methods. This is a retrospective observational study that included thirteen women with uterine atony and postpartum bleeding that did not react to usual medical management. All these women underwent compressing vertical suture technique in which the anterior and posterior walls of the uterus were attached so as to compress the uterus. The suture is transfixed at the uterine fundus, thus eliminating the risk of sutures sliding off at the uterine fundus (safety pin suture). Results. safety pin uterine compression suture was a sufficient procedure to stop the bleeding immediately in 92.2% of the women. None of the women developed complications related to the procedure. Conclusion. A new safety pin suture is a simple and effective procedure to control bleeding in patients with treatment-resistant, life-threatening atonic postpartum hemorrhage with the advantage of eliminating the risk of the sutures sliding off at the uterine fundus.

Summary

Objectives

To present our experience with diagnosis and surgical management of penile fracture

Patients and methods

We present six cases of penile fracture managed at our unit between 2003 and 2008. The diagnosis was based on clinical presentation and physical examination. The treatment was surgical in all cases with Subcoronal circumferential degloving incision, evacuation of hematoma and reconstruction of the rupture with absorbable suture.

Results

The clinical diagnosis of penile fracture was accurate in all six cases. All patients had a successful outcome, with preservation of erectile function

Conclusion

Penile fracture is a clinical diagnosis and immediate surgical repair offers complete recovery of sexual function

Objective

Tracheoesophageal fistula (TEF) is an uncommon and potentially life-threatening complication of blunt chest trauma. The objectives of this report are to describe our surgical experience in three patients with huge TEF and to evaluate the short-term results of surgical management in this potentially life-threatening complication of blunt chest trauma.

Methods

Three patients with huge TEF (5.2-7.0 cm in diameter) after blunt chest trauma were kept in supine position, then the neck was excided and esophagus was free, double breasted suture and clench and mutilation were performed successively over the same level of superior aperture of thorax. The thoracic esophagus was then located in situ at the membrane portion where the trachea was incomplete. Meanwhile, the stomach was then freed through a middle abdominal incision and pulled through the posterior tunnel of the sternum to the neck. Last, an anastomosis between esophagus and tubular stomach was performed over left neck.

Results

All three patients recovered well after the operation. They were able to take liquids and then solids beginning ten days after the procedure. One year post-operation, they were able to resume normal activity.

Conclusions

The surgical management of patients with huge TEF by esophageal exclusion (cervical gastroesophagostomy) and use of esophagus segment in situ as replacement of the posterior membranous wall of the trachea is feasible.

The surgeon who is faced with the management of a neonate with a prenatally ruptured omphalocele or a gastroschisis has a challenging problem. These anomalles are some of the most serious, lifethreatening neonatal surgical emergencies.

Pneumoperitoneum in the newborn is an acute surgical emergency requiring immediate surgical intervention to ensure survival. It refers to radiological evidence of rupture of an air-containing viscus with resultant soiling of the peritoneal cavity. A female baby was born preterm at 29 weeks with birth weight of 650 grams. She developed abdominal distension on day 6, and abdominal radiography revealed presence of free air in the peritoneum. She proceeded for a laparotomy, and intraoperative findings revealed blood in the peritoneum with an area of inflammation and a small perforation. About 5 cm of the inflamed bowel was resected, and an end to end anastomosis performed. The histopathology of the specimen was consistent with Meckel's diverticulum. Symptomatic Meckel's diverticulum is usually seen in the first two years of life, and perforation is a rare presentation. Perforated Meckel's diverticulum in a premature newborn is very rare, and a review of literature reveals only one other reported case. Newborn Meckel's perforation cases often mimic necrotizing enterocolitis, although many present without any feature of peritonitis. Establishing a preoperative diagnosis of perforated Meckel's is difficult and may not be essential as the treatment remains the same. However, prompt surgical intervention confers a good prognosis in neonates with isolated perforated Meckel's diverticulum.

Background

Principles for the treatment of tibial intercondylar eminence fracture are early reduction and stable fixation. Numerous ways to treatment of this fracture have been invented. We designed a simple, low-invasive, and arthroscopic surgical strategy for tibial intercondylar eminence fracture utilizing the Meniscal Viper Repair System used for arthroscopic meniscal suture.

Methods

We studied 5 patients, who underwent arthroscopic suture fixation that we modified. The present technique utilized the Meniscal Viper Repair System for arthroscopic suture of the meniscus. With one handling, a high-strength ultra-high molecular weight polyethylene(UHMWPE) suture can be passed through the anterior cruciate ligament (ACL) and the loops for suture retrieval placed at both sides of ACL. Surgical results were evaluated by the presence or absence of bone union on plain radiographs, postoperative range of motion of the knee joint, the side-to-side differences measured by Telos SE, and Lysholm scores.

Results

The reduced position achieved after surgery was maintained and good function was obtained in all cases. The mean distance of tibia anterior displacement and assessment by Lysholm score showed good surgical results.

Conclusion

This method simplified the conventional arthroscopic suture fixation and increased its precision, and was applicable to Type II fractures that could be reduced, as well as surgically indicated Types III and IV. The present series suggested that our surgical approach was a useful surgical intervention for tibial intercondylar eminence fracture.

Introduction

Bleeding from pancreatic pseudocyst’s rupture into adjacent organs is a rare, but potentially fatal, complication of chronic pancreatitis requiring quick management. Timing of the rupture is unpredictable; early diagnosis and correct management is essential in preventing the bleeding.

Case presentation

We describe the case of a 53 years old male patient successfully treated with emergency surgery for massive hematemesis due to a rupture of a bleeding pseudocyst into the stomach. Patient underwent emergency laparotomy and suture of the bleeding vessel. At 5 years follow-up patient is in healthy condition.

Conclusion

This case shows to surgeons that pancreatic pseudocyst cannot be managed strictly with one rule and prompt surgical treatment is mandatory in case of haemodinamic instability.

Purpose

Treatment of acute subcutaneous Achilles tendon rupture remains challenging. Whereas the results of conservative and operative treatment are inconsistent, early mobilisation treatment seems to be beneficial. Besides suture repair, operative treatment using adhesives reveals promising results. Our hypothesis was that a gluing technique provides initial stability comparable to sutures.

Methods

In a biomechanical study, 18 fresh frozen sheep Achilles tendons were used to compare the biomechanical properties of suture repair using PDS® II and Bunnell’s technique to tendon gluing using BioGlue® and Tissucol®. Load to failure testing was performed.

Results

Ultimate failure loads of sutures (146.2 ± 30.8 N) are significantly superior to the techniques using BioGlue® (38.4 ± 18.3 N; p <0.0001) or Tissucol® (4.7 ± 2.5 N; p <0.0001). Interestingly, no significant differences in stiffness were found between the application of BioGlue® and PDS® II.

Conclusions

Suture repair provides significantly superior biomechanical properties compared to the use of both tested adhesives BioGlue® and Tissucol®. Based on the presented data we recommend the use of suture material for open Achilles tendon repair.

Diaphragmatic rupture is a life-threatening condition. Diaphragmatic injuries are quite uncommon and often result from either blunt or penetrating trauma. Diaphragmatic ruptures are usually associated with abdominal trauma however, it can occur in isolation. Acute traumatic rupture of the diaphragm may go unnoticed and there is often a delay between the injury and the diagnosis. A comprehensive literature search was performed using the terms "delayed presentation of post traumatic diaphragmatic rupture" and "delayed diaphragmatic rupture". The diagnostic and management challenges encountered are discussed, together with strategies for dealing with them. We have focussed on mechanism of injury, duration, presentation and site of injury, visceral herniation, investigations and different approaches for repair. We intend to stress on the importance of delay in presentation of diaphragmatic rupture and to provide a review on the available investigations and treatment methods. The enclosed case report also emphasizes on the delayed presentation, diagnostic challenges and the advantages of laparoscopic repair of delayed diaphragmatic rupture.

Objects:

Progressive skin necrosis of giant occipital encephalocoele is an extremely rare complication found in neonates. Infection and ulceration of the necrosed skin may lead to meningitis or sepsis. We present here a neonate with giant occipital encephalocoele showing progressive necrosis during the first day of his life.

Methods:

A newborn baby was found to have a huge mass in the occipital region, which was covered by normal pink-purplish skin. During the last hours of the first day of his life, the sac started becoming ulcerated accompanied with a rapid color change in the skin, gradually turning darker and then black. The neonate was taken up for urgent excision and repair of the encephalocele. Two years after the operation, he appears to be well-developed without any neurological problems.

Conclusion:

Necrosis may have resulted from arterial or venous compromise caused by torsion of the pedicle during delivery or after birth. The high pressure inside the sac associated with the thin skin of the encephalocoele may be another predisposing factor. In view of the risk of ulceration and subsequent infection, urgent surgery of the necrotizing encephalocele is suggested.

We describe a case of subacute left ventricular free wall rupture during acute myocardial infarction in a 68-year-old man. The diagnosis was confirmed by echocardiography. The patient was supported by an intra-aortic balloon pump until the ruptured wall could be successfully repaired by suturing and gluing a pericardial patch over the defect and bypassing the left anterior descending coronary artery with a vein graft. This case demonstrates that left ventricular free wall rupture is not always fatal and that early diagnosis and institution of intra-aortic balloon pump support in such patients can allow successful bridging to definitive emergency surgical therapy.

Summary

High sugar intake has been linked to fetal anomalies in the presence and absence of insulin resistance. Using dietary data collected in the Boston University Slone Epidemiology Birth Defects Study, we examined whether high dietary glycemic index (dGI) or load (dGL) increased the risk of birth defects. Non-diabetic mothers of 1,921 cases and 704 controls were interviewed within six months after delivery (1988–1998) about pregnancy events and exposures, including a 99-item food frequency questionnaire. Case groups included amniotic bands, craniosynostosis, gastroschisis, hypospadias, small intestinal defects, anorectal defects, limb reductions, omphalocele, cleft lip and/or palate, renal agenesis, and tracheoesophageal fistula. Cubic splines were used to determine cutpoints values for high dGI and dGL in relation to the risk of each birth defect. The cutpoints were used in logistic regression models to calculate odds ratios (OR) and 95% confidence intervals (CI). Control mothers in the lowest quartile of glycemic intake were more likely to be non-Hispanic White, ≥ 30 years of age, have higher family income, have a normal body mass index, and reside in Boston. Findings were null for most case groups. The anorectal defect case group was found to have elevated risks for dGL [adjusted OR: 2.35; 95%CI: 1.1, 4.9], while estimates for dGI were elevated for the amniotic band case group [adjusted OR: 3.01; 95% CI: 1.1, 8.1]. Because this is the first paper (to our knowledge) to explore dGI and dGL in relation to a spectrum of birth defects, additional studies are needed.

Background

Nonabsorbable sutures are favorable for repairing flexor tendons. However, absorbable sutures have performed favorably in an animal model.

Methods

Two-strand sutures using the interlocking modified Kessler method with polydioxanone absorbable sutures 4-0 were used to repair completely ruptured flexor tendons in 55 fingers from 41 consecutive patients. The medical records of average 42 follow up weeks were analyzed retrospectively. The data analyzed using the chi-squared test, and Fisher's exact test was used for postoperative complications. The results were compared with those of other studies.

Results

Among the index, middle, ring, and little fingers were injured in 9, 17, 16, and 13 fingers, respectively. The injury levels varied from zone 1 to 5. Of the 55 digits in our study, there were 26 (47%) isolated flexor digitorum profundus (FDP) injuries and 29 (53%) combined FDP and with flexor digitorum superficialis injuries. Pulley repair was also conducted. Concomitant injuries of blood vessels and nerves were found in 17 patients (23 fingers); nerve injuries occurred in 5 patients (10 fingers). Two patients had ruptures (3.6%), and one patient had two adhesions (3.6%). Using the original Strickland criteria, all the patients were assessed to be excellent or good. Also, fibrosis and long-term foreign body tissue reactions such as stitch granuloma were less likely occurred in our study. Compared to the Cullen's report that used nonabsorbable sutures, there was no significant difference in the rupture or adhesion rates.

Conclusions

Therefore, this study suggests that appropriate absorbable core sutures can be used safely for flexor tendon repairs.

We report herein a rare case of complete congenital sternal cleft (absent sternum) and anterior pericardial defect in association with pectus excavatum. In neonates with absent sternum, the sternal bars can be easily approximated by simple suture, due to the flexibility of the cartilaginous thorax. There is also little danger of cardiac compression when the repair is performed early in life.

If reconstruction is delayed, the increased rigidity of the chest wall and the physiologic accommodation of the thoracic organs to the circumference of the chest render simple approximation impossible, without serious compromise of the heart and lungs.

Our patient was a 13-year-old girl, whose case was particularly unusual because of the association of sternal cleft with pectus excavatum. After surgical correction of the pectus excavatum, we were able to construct a sternum by incising the lateral border of each sternal bar, thereby creating flaps that we sutured together at midline. The sternal bars were then approximated by loops of nonabsorbable suture around their circumference.

The patient had an uncomplicated course, and at the 12-month follow-up visit, her sternal appearance was normal. (Tex Heart Inst J 2002;29:206–9)

Objective:

Sirenomelia (Mermaid syndrome) is a rare anomaly of caudal region of the body, presented with fusion of the lower limbs. Genito-urinary, gastro-intestinal, neural tube and vertebral anomalies are found in most cases.

Methods:

We present a case of sirenomelia diagnosed in the first tri-mester, associated with dextrocardia, and omphalocele

Conclusion:

First trimester diagnosis of sirenomielia is possible and early diagnosis gives the parents the option of early pregnancy termination