First report of surgery for anencephaly in twin pregnancies: Technical notes and postoperative management.

Anencephaly, the most severe form of neural tube defect, has no known cure, and in most cases, patients die before or shortly after birth. To date, no surgical intervention has been reported in the management of anencephaly. This study presents a case of dichorionic-diamniotic twin pregnancy in which 1 twin was anencephalic and describes the surgical management of this complex case. We aimed to share the problems experienced during the follow up of a patient who survived for a long time after surgery. We also aimed to highlight several clinical issues, including the challenges of managing anencephaly in twin pregnancies, problems experienced during the follow up process in our case, diagnosis of brain death in anencephaly cases, and ethical dilemmas related to organ donation. This case is notable because of the challenging nature of the surgical procedure and complexity of postoperative care. By highlighting the difficulties encountered during the follow up period, we hope to provide insights to health professionals that can inform the management of similar cases in the future.

The Global Burden of Neural Tube Defects and Disparities in Neurosurgical Care.

BACKGROUND: Despite the success of folic acid fortification programs, neural tube defects (NTDs) such as spina bifida, encephalocele, and anencephaly remain among the most substantial causes of childhood morbidity and mortality worldwide. Although these are complicated conditions that require an interdisciplinary approach to care, definitive treatment of survivable NTDs is often neurosurgical. METHODS: Using Global Burden of Disease data, we examined the global burden of NTDs as related to a nation's wealth, health care quality, and access to neurosurgical care. We abstracted data for death by cause, years lived with disability (YLD), gross domestic product (GDP), United Nations geoscheme, Food Fortification Initiative participation, and Healthcare Access and Quality Index. We compared means using 1-way analysis of variance and proportions using Fisher exact tests, with statistical significance as α = 0.05. RESULTS: Seventeen of 20 (85%) nations with the most deaths caused by NTDs (P < 0.0001) and 15/20 (75%) nations with the highest YLD (P < 0.0001) were in the lowest GDP quartile. Deaths and YLD were negatively correlated with increasing GDP and Healthcare Access and Quality Index (P < 0.0001). The nations with the highest disease burdens also had the fewest neurosurgeons per capita. CONCLUSIONS: Despite the success of folic acid fortification programs, greater global public health efforts should be placed on improving access to neurosurgical care in low and middle-income nations through sustainable initiatives such as surgeon exchange programs and the establishment of neurosurgery residency training programs.

Dicephalus dibrachius with anencephaly.

We present a case of inferior conjunction in a rare type of conjoined twins, dicephalus in a male fetus. The male fetus was born to a 24-year-old, gravida 2, and para 0, who had medical abortion at 15 weeks of gestation due to anencephaly with meningoencephalocele revealed by ultrasound examination. The fetus was born with 2 anencephalic heads with a bifurcation of the vertebral column and presence of 2 spinal cords. The other viscera and limbs were normal in number and location as for a male singleton. This case illustrates the relationship between conjoined twining, and neural tube defect more particularly anencephaly with a male zygote, which is an unusual presentation for this type of zygote gender.

Use of a temporary vena cava filter in a woman with septic abortion and inferior vena cava thrombosis. A case report.

BACKGROUND: Ovarian vein and inferior vena cava (IVC) thrombosis are rare but life-threatening complications of severe obstetric infections. IVC filters have been used to reduce the risk of pulmonary embolus in patients who have recurrent thrombotic and embolic events or underlying risk factors. More recently, retrievable IVC filters have been used in patients in whom the risk is transient. CASE: At 18 weeks' gestation a multigravida with a fetus with anencephaly became septic after Laminaria placement and rupture of the membranes. She developed ovarian vein and IVC thrombosis and was treated successfully with a retrievable IVC filter, anticoagulation and antibiotics. The filter was removed after 9 days. CONCLUSION: In young patients at transient risk for emboli and requiring placement of an IVC filter, a retrievable filter should be considered to reduce the long-term risks of an indwelling filter.

C-section for organ donation.

KIE: A woman carrying a fetus with anencephaly requests a cesarean delivery when signs of fetal distress are detected. Her intention is to donate the organs for transplantation if the child is born alive. Commentators are asked if the woman's physician should accede to her request, and if the risks to the patient of a cesarean section are justified in this case. Berkowitz and Newman argue against surgical intervention as not medically indicated for mother or fetus, and as posing greater risks than offering benefits. Mathieu argues that while the chances are slight of obtaining transplantable organs in this case, the physician is not justified in overriding a competent patient's request for cesarean delivery.^ieng

[2 cases of anencephaly in one family]. / Dwa przypadki bezmózgowia w jednej rodzinie.

Two births of anencephalic fetuses occurred in the second and the fourth pregnancies in a family. The children born after the 1st and 3rd pregnancies were normal. Anencephaly was not associated with spina bifida, and both fetuses were female. The study failed to demonstrate the cause of the abnormality.

Intraventricular twin fetuses in fetu.

The authors report a rare case of multiple intracranial fetuses in fetu, fulfilling Willis' traditional criteria, which include an axial and appendicular skeleton with surrounding organized tissue. This case was ascertained from studies of a full-term female neonate who presented with ventriculomegaly. A CT scan showed intracranial calcifications that were suggestive of an axial skeleton. Her birth weight was 3.176 kg (50th-75th percentile), length was 52 cm (90th percentile), head circumference was 35 cm (50th-75th percentile), and Apgar scores were 7 and 8 at 1 and 5 minutes, respectively. Prenatal ultrasonography studies performed at 12 weeks and 5 days, and 19 weeks and 6 days revealed normal findings. A 37-week prenatal ultrasonography study showed ventriculomegaly and obstructive hydrocephalus, with a possible intracranial teratoma. Cranial imaging at birth with ultrasonography, CT and MR imaging, and MR angiography demonstrated 2 complex intraventricular masses with cystic, solid, and bony elements. A craniotomy with resection of the masses was performed at 3 months of age. The infant survived and is now 12 months old with some developmental progress. Two axial skeletons, with accompanying rib cage and extremities, including well-formed feet and toes, were noted. Both anencephalic structures had skin with hair, fat, skeletal and smooth muscle, and bony structures with bone marrow and focal areas of calcification. Multiple viscera were present and included thymus, bowel, stomach, salivary gland, kidney, adrenal gland, lung, and presumed adnexal structures. A diagnosis of fetuses in fetu was rendered. Chromosomal studies of the child and tissue from the 2 fetuses in fetu showed normal female karyotypes. A single nucleotide polymorphism array analysis from the proband infant and tissue from the 2 identified fetuses in fetu appeared to be genetically identical. These results are consistent with a monozygotic twin embryonic origin of the fetus in fetu tissue, which is a mechanism that has been suggested in previous reports in which karyotypes, blood types, and limited genetic loci have been studied. This is the first report of a rare example of intracranial intraventricular twin fetuses in fetu for which a genome-wide single nucleotide polymorphism assay has confirmed their genetic identity.

[Reflections about the obstetric attitude towards anencephalic fetuses]. / Reflexiones sobre la conducta obstétrica en la embarazada con feto anencefálico.

Pregnancy of an anencephalic fetus generates deep thoughts about its diagnosis, treatment and management of maternal risk, having in mind the irreversibility of the fetal situation. At the present moment, there are no guidelines for labor care in these cases, probably because in most developed countries in which abortion is legal, these pregnancies are interrupted earlier. In Chile, where abortion is illegal we must deal with these situations at the end of the pregnancy period. Of 35,682 labors attended at our hospital, 14 were anencephalic fetuses. In 50% of these a cesarean section was done and in one, a hysterectomy was required due to uterine inertia.

[Renal transplants from anencephalic donors]. / Trasplante renal de donador anencéfalo.

Kidney transplants are carried out as any other surgical procedure at specialized third level hospitals. For over 20 years, in Mexico, transplants have been done with kidneys originating from cadavers. Even though this has been of tremendous help, there is still a shortage of donors. Two successful cases of kidney transplants are presented where the donor was an anencephalic neonate and where segments of the aorta and vena cava were used to keep alive the kidney tissue enough for the corporal surface of a child and also that of an adult.

A cohort study of neural tube defects (NTD) in Denmark covering the first seven years of life.

In the County of Fyn, Denmark, which is a well-delimited region comprising a 10% representative sample of the total population, ten complete one year cohorts of newborn were scrutinized for neural tube defects (NTD). Among 66,000 births, 71 cases with NTD were observed. The total prevalence at birth was 1.08/1,000; 0.29 for anencephaly; 0.20 for encephalocele, and 0.59 for spina bifida (SB). This is at the same level as in other Scandinavian countries. Considering only neonates born alive, 57% of the total survived until the age of 7 years, disregarding anencephaly, but only 4 of 18 SB cases and 6 of 11 encephalocele cases had no handicaps at age 7. On the average, SB patients spent 9% of their life before the age of 7 years in hospitals and encephaloceles 2%. The SB cases occupied 25 times more beds per year than the comparable age group in general and many were likely to need continued medical and social care.

Reflexiones sobre la conducta obstétrica en la embarazada con feto anencefálico / Reflections about the obstetric attitude towards anencephalic fetuses

Pregnancy of an anencephalic fetus generates deep thoughts about its diagnosis, treatment and management of maternal risk, having in mind the irreversibility of the fetal situation. At the present moment, there are no guidelines for labor care in these cases, probably because in most developed countries in which abortion is legal, these pregnancies are interrupted earlier. In Chile, where abortion is illegal we must deal with these situations at the end of the pregnancy period. Of 35,682 labors attended at our hospital, 14 were anencephalic fetuses. In 50 percent of these a cesarean section was done and in one, a hysterectomy was required due to uterine inertia.