Umbilical Vein Calcification Associated with Double-Lumen Catheter Malpositioning in an Extremely Low-Birth-Weight Infant.

Umbilical venous (UV) catheters (UVCs) are commonly used in severely ill neonates. Complications associated with UVC often result from an inappropriate UVC position. Calcification of the UV, a rare complication, was observed in an extremely low-birth-weight infant born at 23 weeks of gestation. After birth, the infant experienced respiratory and circulatory dysfunction, followed by disseminated intravascular coagulation (DIC). A UVC was inserted, and circulatory agonists and blood transfusions were administered, as well as a calcium gluconate infusion for hypocalcemia and hyperkalemia. Ten days after birth, calcification was detected in the UV, likely due to a tunica intima injury caused by UVC, a hypercoagulable state due to DIC, and a high-dose calcium gluconate infusion. Additionally, proximal port malpositioning of the double-lumen catheter might have contributed to calcification within the UV. To prevent such complications, real-time ultrasound confirmation with agitated saline contrast during UVC placement is recommended; in the absence of the facility or skills for ultrasonography, X-rays should be performed in the lateral and anteroposterior views. Furthermore, when using multi-lumen catheters, physicians should not only verify the tip position but also ensure proper placement of proximal ports and carefully select medications administered through the ports.

Safety and accuracy of neonatal continuous glucose monitoring.

BACKGROUND: Hypoglycemia is a significant problem for all neonates and requires minimally invasive and reliable monitoring. The primary objective of this study was to verify the safety and accuracy of the continuous glucose monitoring (CGM) of full-term neonates using Freestyle Libre, a flash glucose monitoring (FGM) device. METHODS: The study was conducted on 20 neonates. Shortly after birth, we placed the FGM sensor on the outside of the neonates' thighs. We scanned the CGM values at 60, 120, 180, and 360 min after birth and simultaneously obtained blood glucose values with plantar capillaries by heel puncture. The neonates wore the sensors for up to 6 h and then they were removed. RESULTS: Of the 75 data points to be measured, 65 points (86.7%) were obtained by scan. There was no change in the sensor attachment site in 12 of 18 completed cases in this study but we observed slight induration in four cases (22.2%) and slight redness in one case (5.5%) at the sensor puncture site. A moderate correlation was observed between the CGM and blood glucose values. The CGM values tended to be low at 120, 180, and 360 min after birth, and tended to be high only at 60 min after birth. CONCLUSIONS: The CGM device was safe to wear on the neonate and the CGM data correlated well with blood glucose levels. There was dissociation between CGM data and blood glucose levels in the acute period soon after birth when the blood glucose levels changed rapidly.

Umbilical Granuloma: Frequency, Associated Factors, 10-Year Treatment Trends, and Effectiveness at a Single Hospital in Japan.

Umbilical granuloma (UG) is a common problem during the neonatal period; however, its epidemiology and etiology are poorly studied, and the best treatment option has not yet been established. We examined the medical records of neonates who were born and underwent 1-month evaluations at our hospital between 2013 and 2022 to investigate the frequency of-and factors associated with-UG, as well as the annual trends of UG treatments and their efficacy. Of the 6680 eligible neonates, 395 (5.9%) had UG. The annual incidence rate ranged from 3.8% to 7.3%. Gestational age, birth weight, and incidence of meconium-stained amniotic fluid were significantly associated with UG. Silver nitrate cauterization was the predominant UG treatment from 2013 to 2016. Silver nitrate cauterization and topical betamethasone valerate were nearly equally applied in 2017. Betamethasone application became predominant in 2018. The healing rates during the initial treatment period were 91% for silver nitrate cauterization, 97.7% for betamethasone application, 60% for ethanol disinfection, and 88% for ligation; these rates were significantly different (p < 0.001). Topical steroid application may be the most effective treatment. If steroid application is ineffective, then silver nitrate cauterization and ligation may be important treatment options.

Intestinal volvulus in utero causing torsion of dilated bowel with ileal atresia: a case report.

BACKGROUND: In utero intestinal volvulus with intestinal atresia is a rare and life-threatening condition that can cause torsion of the dilated bowel. The management and outcomes of this disease remain unclear. CASE PRESENTATION: A 19-year-old woman noticed a decrease in fetal motion at 35 weeks. Fetal ultrasound showed dilated fetal bowel and the whirlpool sign. The patient was referred to our hospital for an emergency cesarean section. The neonate's abdomen was dark and severely distended, and a laparotomy was performed. Necrotic ileum and cord-type intestinal atresia (Type II) were observed in the dilated terminal ileum. The necrotic ileum was resected, and a second-look surgery was performed the following day. Then, we anastomosed the remaining intestine, and the total intestine length was 52 cm. There were no surgical complications, and the patient was discharged without requiring total parenteral nutrition or fluid infusion. The patient's height and weight were within the - 2 standard deviation range of the growth curve at 5 months. CONCLUSIONS: Emergency and appropriate management of intestinal volvulus in utero causing torsion of the dilated bowel resulted in good outcomes in a patient with intestinal atresia. Perinatal physicians should be aware of this emergency condition and plan their treatment approach accordingly.

Accuracy of transcutaneous bilirubin level measured by a JM-105 bilirubinometer.

BACKGROUND: Transcutaneous bilirubin (TcB) measurement is useful, but dissociation with total serum bilirubin (TSB) is a clinical problem in measurement. We verified the accuracy of the latest version of the JM-105 jaundice meter. METHODS: The TcB, TSB, and hematocrit (Hct) measurements obtained in the first 4 days of life in 2788 term neonates were analyzed. RESULTS: When divided into 2-mg/dL classes, the difference between the TcB and TSB measurements did not change as TcB increased, but both overestimation and underestimation of TcB increased as TcB increased. At TcB greater than 11 mg/dL, inaccurate measurements with dissociation greater than 2 mg/dL exceeded 10% of the TcB measurements. The Hct value was associated with overestimation and underestimation. CONCLUSION: To evaluate neonatal jaundice accurately, it is desirable to measure TSB by blood sampling before discharge from obstetrics or in the case of worsening jaundice on day 4 or 5 of life.

Clinical Dilemma Involving Treatments for Very Low-Birth-Weight Infants and the Potential Risk of Necrotizing Enterocolitis: A Narrative Literature Review.

Necrotizing enterocolitis (NEC) is a critical gastrointestinal emergency with substantial morbidity and mortality risks, especially for very low-birth-weight (VLBW) infants, and unclear multifactorial pathophysiology. Whether common treatments for VLBW infants increase the NEC risk remains controversial. Indomethacin (utilized for patent ductus arteriosus) offers benefits but is concerning because of its vasoconstrictive impact on NEC susceptibility. Similarly, corticosteroids used to treat bronchopulmonary dysplasia may increase vulnerability to NEC by compromising immunity and altering the mesenteric blood flow. Histamine-2 receptor blockers (used to treat gastric bleeding) may inadvertently promote NEC by affecting bacterial colonization and translocation. Doxapram (used to treat apnea) poses a risk of gastrointestinal disturbance via gastric acid hypersecretion and circulatory changes. Glycerin enemas aid meconium evacuation but disrupt microbial equilibrium and trigger stress-related effects associated with the NEC risk. Prolonged antibiotic use may unintentionally increase the NEC risk. Blood transfusions for anemia can promote NEC via interactions between the immune response and ischemia-reperfusion injury. Probiotics for NEC prevention are associated with concerns regarding sepsis and bacteremia. Amid conflicting evidence, this review unveils NEC risk factors related to treatments for VLBW infants, offers a comprehensive overview of the current research, and guides personalized management strategies, thereby elucidating this clinical dilemma.

Pitfalls in the Serological Evaluation of Maternal Cytomegalovirus Infection as a Potential Cause of Fetal and Neonatal Involvements: A Narrative Literature Review.

Cytomegalovirus (CMV) is the most common cause of intrauterine infection and serological assays are the primary tools for assessing CMV infections during pregnancy. CMV-specific immunoglobulin M (IgM) antibodies have been used as a diagnostic marker for primary CMV infection in pregnant women, although CMV-IgM has been detected in non-primary CMV infections. IgG avidity testing may aid the distinguishing of primary from non-primary CMV infection; however, there is no standardized assay for detecting this difference. Moreover, when maternal serology shows positive CMV-IgG with negative CMV-IgM findings, vertical transmission probability following primary CMV infection is often excluded. However, symptomatic congenital CMV infections in the context of negative findings for maternal CMV-IgM have been reported recently. The absence of CMV-IgM is recognized in both primary and non-primary CMV infections. Furthermore, maternal non-primary CMV infections during pregnancy may yield a greater proportion of symptomatic congenital CMV infections than previously thought. If universal prenatal screening is performed, ultrasonography for abnormal fetal findings should be conducted regardless of CMV-IgM antibody status. If not universally screened, CMV antibody screening should be performed whenever routine fetal ultrasound reveals abnormal findings. For suspected fetal CMV infection, amniotic fluid or postnatal infant urine CMV-DNA testing is required.

Exchange Transfusion in Neonatal Sepsis: A Narrative Literature Review of Pros and Cons.

Neonatal sepsis remains a leading cause of morbidity and mortality worldwide. It is widely considered that exchange transfusion (ET) as an adjunctive treatment for neonatal sepsis has the ability to reduce mortality. This review summarizes the current knowledge regarding the efficacy of ET for neonatal sepsis. In neonatal sepsis, immune responses such as proinflammatory and anti-inflammatory cytokines play an important role in pathogenesis and can lead to septic shock, multiple organ failure, and death. Between the 1970s and 1990s several authors reported that ET was effective in the treatment of neonatal sepsis with sclerema. ET removes bacterial toxins and inflammatory cytokines from the blood by replacing it with fresh and immunologically abundant blood, thereby leading to improvement in tissue perfusion and oxygenation. Moreover, ET with fresh whole blood increases neutrophil count and immunoglobulin levels as well as enhancing neutrophil function. However, there is a lack of clear evidence for the clinical efficacy of ET. In addition, adverse events associated with ET have been reported. Although most complications are transient, ET can lead to life-threatening complications. Therefore, ET can be considered a last resort treatment to rescue neonates with severe sepsis with sclerema and disseminated intravascular coagulation.

Clinical and pathophysiologic relevance of autoantibodies in neonatal myasthenia gravis.

Between 10% and 20% of neonates born to mothers with myasthenia gravis (MG) develop neonatal MG due to the transfer of maternal autoantibodies across the placenta. Neonatal MG can occur in infants born not only from mothers with acetylcholine receptor (AChR) or muscle-specific tyrosine kinase (MuSK) antibodies but also from mothers without detectable muscle antibodies. The low incidence rate may be due to specific autoantibody characteristics that differ among individuals, but a genetic predisposition in some infants is possible. The majority of reported neonatal MG cases are anti-AChR antibody-positive (AChR-MG), and a high anti-fetal/anti-adult AChR titer ratio in the mother is predictive of its occurrence. However, patients with anti-MuSK antibody-positive MG (MuSK-MG) are more likely to experience exacerbations during pregnancy and have a higher probability of developing neonatal MG than AChR-MG patients. Moreover, maternal MuSK-MG may be associated with early-onset and more severe manifestations of neonatal MG. Although cholinesterase inhibitors have been effectively used for treating neonatal AChR-MG, neonatal MuSK-MG may be more difficult to treat with this type of medication. Maternal MuSK-MG usually greatly benefits from intravenous immunoglobulin (IVIG) and plasma exchange. In neonatal MG, IVIG is considered for severely affected infants with MuSK-MG, but the efficacy of IVIG remains unclear. Although exchange transfusion may be a management adjunct, its clinical benefits are controversial. As the therapy-induced reduction of autoantibodies may be advantageous for fetal outcomes, maternal MG should be effectively treated during pregnancy. However, caution of drug contraindication during pregnancy and lactation must be exercised to avoid unwanted effects for the fetus and neonate. In the future, MG caused by anti-lipoprotein receptor-related protein 4 or other antibodies might be also identified in pregnant women and neonates. Therefore, the determination of autoantibody specificity is essential for successful management.

Effects of Season of Birth and Meteorological Parameters on Serum Bilirubin Levels during the Early Neonatal Period: A Retrospective Chart Review.

To establish whether serum bilirubin levels vary in healthy term neonates according to seasonal variations and meteorological factors, we retrospectively studied 3344 healthy term neonates born between 2013 and 2018. Total serum bilirubin (TSB) levels were measured on the fourth day after birth. The monthly and seasonal variations in TSB levels and clinical and meteorological effects on TSB levels were assessed. In the enrolled neonates, the median TSB level was 195 µmol/L. The TSB level peaked in December and was the lowest in July, but the variation was not statistically significant. The TSB level was significantly higher in the cold (October to March) than in the warm season (April to September; p = 0.01). The comparison between seasonal differences according to sex showed TSB levels were significantly higher in the cold than in the warm season in male infants (p = 0.001), whereas no significant difference was observed in female infants. A weakly negative but significant association existed between TSB levels and the mean daily air temperature (r = -0.07, p = 0.007) in only the male population; the female population showed no significant correlation between TSB levels and meteorological parameters. The season of birth is an etiological factor in neonatal jaundice, with an additional influence from sex.

Effects of fetal involvement of inadvertent radioactive iodine therapy for the treatment of thyroid diseases during an unsuspected pregnancy.

Radioactive iodine (131I) therapy is absolutely contraindicated in pregnancy, but reports of inadvertent exposure continue to appear in the literature. Radiation-induced effects on the embryo/fetus are highly dependent on the stage of pregnancy, the dose absorbed by the embryo/fetus, and the manifestations of the pathological condition that develops as a result of the irradiation. Prior to implantation, the major concern is death of the embryo when exposed to radiation greater than the 100 mGy threshold. At this very early stage of pregnancy, exposure to 131I is unlikely to cause major malformations or thyroid dysfunction in surviving embryos. Exposure during organogenesis of the thyroid (from 10 weeks of gestation onward) and that of other organs at radiation thresholds of 100-300 mGy may result in fetal thyroid ablation, malformations, growth restriction, and in later life, mental retardation (MR). In addition, any dose of radiation exposure may increase the risk of cancer many years after the in utero exposure. Fetal and neonatal hypothyroidism due to in utero 131I exposure may require lifelong thyroxine replacement therapy and result in severe MR if the condition is not recognized immediately. Therefore, thyroid function must be evaluated and replacement therapy should be started without delay even before birth. Physicians treating women of childbearing age with 131I need to be aware of the risks of in utero 131I exposure and take all measures to avoid inadvertent exposure during pregnancy. Nevertheless, in case of accidental exposure, the clinician should confirm the gestational age at exposure, evaluate the risks to the fetus by estimating the radiation dose delivered, and discuss the subsequent management plan with the pregnant woman. This review aimed to summarize the current knowledge regarding the risk of harm to the developing fetus after in utero 131I exposure, especially focusing on the effects on thyroid function. This study also evaluated the most significant new findings regarding the prevention and in utero and peripartum management of fetal exposure to 131I.

Hemodynamic changes in neonates born to mothers with Graves' disease.

PURPOSE: Cardiac insufficiency is a major morbidity in neonatal hyperthyroidism. It is important to assess the hemodynamics in neonates born to mothers with Graves' disease (GD). This study prospectively evaluated the hemodynamic changes in neonates born to mothers with GD. METHODS: Overall, 80 newborns were enrolled. Thirty-six neonates were born to mothers with GD who were positive for thyroid-stimulating hormone (TSH) receptor antibody (TRAb), and 44 were born to mother negative for TRAb. The serum levels of TSH, free triiodothyronine (FT3), free thyroxine (FT4), and N-terminal-pro-B-type natriuretic peptide (NT-proBNP), the cardiac output, and cardiac index (CI) evaluated by echocardiography were compared between the two groups at several postnatal points (day of delivery and 5, 10, and 30 days of life). RESULTS: The TRAb-positive newborns had higher FT4 levels and CI on Day 5 (both p < 0.05) and higher FT3 (p < 0.05) and FT4 levels (p < 0.01) and CI (p < 0.01) but lower TSH levels (p < 0.05) on Day 10 than the TRAb-negative newborns. The TRAb-positive newborns had significantly higher NT-proBNP levels on Days 5 (median 752 vs. 563 pg/mL, p = 0.034) and 10 (median 789 vs. 552 pg/mL, p = 0.002) than the TRAb-negative newborns. CONCLUSIONS: Hemodynamic changes in neonates born to TRAb-positive mothers with GD resulted in a higher CI and NT-proBNP levels than in those with TRAb-negative mothers from postnatal days 5 to 10.

The association between congenital heart disease and small for gestational age with regard to the prevalence and outcomes.

AIM: To evaluate the association between small for gestational age (SGA) and the prevalence of congenital heart disease (CHD) and the association of the SGA status with the outcomes among infants with CHD. METHODS: Echocardiography was performed within the first 5 days of life in 5664 consecutive infants. Infants were classified into four groups according to the presence or absence of SGA and CHD. All CHD infants were followed up until either spontaneous resolution of all cardiac lesions, invasive intervention or death. All newborns without CHD were followed for mortality until the final follow-up date. RESULTS: A total of 303 infants were diagnosed with CHD, while 610 were diagnosed with SGA. Among the CHD infants, 56 were SGA, and 247 were not. A multivariable logistic regression analysis showed that the adjusted odds ratio of SGA (9.71, P < .001) was significantly higher than that of other parameters concerning predictors of invasive intervention or death. The mortality rate in the presence of both SGA and CHD (hazard ratio: 33.6, P < .001) was markedly higher than in the absence of both. CONCLUSION: SGA was a significant predictor of invasive intervention for CHD. The combination of CHD and SGA carried a high risk of death beyond that of either alone.

Suicide attempt using potassium tablets for congenital chloride diarrhea: A case report.

BACKGROUND: Congenital chloride diarrhea (CCD) is a rare inherited disorder of intestinal electrolyte transport that results in a large wastage of electrolytes and water. Advances in substitution therapy using sodium chloride (NaCl) and potassium chloride (KCl) have dramatically improved survival for patients with CCD. Slow-release KCl is widely prescribed as a potassium supplement; however, it has also occasionally been used in suicide attempts, as potassium poisoning can generate life-threatening hyperkalemia. CASE SUMMARY: A 26-year-old female presented to the emergency department (ED) with self-poisoning, having taken 30 tablets of slow-release KCl (total: 240 mmol potassium) following an auditory hallucination. The patient had been undergoing substitution therapy with NaCl and KCl for CCD and been followed up in the pediatric department. One month prior, she developed insomnia and anxiety and had consulted a psychiatrist. At the ED, although her general condition was good, she appeared agitated. Her serum potassium level was 7.0 mmol/L, indicating hyperkalemia, and electrocardiographic changes showed tenting of the T-waves. She responded to the administration of calcium gluconate, sodium bicarbonate, and insulin with glucose, and the serum potassium level improved. Finally, she was diagnosed with schizophrenia. CONCLUSION: In CCD management, physicians should pay careful attention to patients' extraintestinal issues, including psychological disorders that may emerge in adulthood.

Pitfalls in the assessment of gestational transient thyrotoxicosis.

Gestational transient thyrotoxicosis (GTT) is associated with direct stimulation of the maternal thyroid gland by human chorionic gonadotropin (hCG). It is characterized by slightly higher thyroid hormone and lower thyroid-stimulating hormone (TSH) levels in early pregnancy and mild or no symptoms. While GTT must be distinguished from Graves' disease (GD), which is associated with maternal and fetal complications, treated GD and new-onset GD in pregnancy are occasionally challenging to distinguish. Evaluating serum hCG levels and TSH receptor antibody (TRAb) titers can help, but the results are not irrefutable due to pregnancy-related immunosuppression. Moreover, GTT can follow unusual clinical courses in relation to some pregnancy complications. Excessive hCG production can cause severe GTT symptoms in patients with hyperemesis gravidarum, trophoblastic disease, or multiple pregnancies. Thyrotoxicosis can emerge beyond the second trimester in patients with gestational diabetes mellitus and mirror syndrome, because of delayed elevations in the hCG levels. Detailed knowledge about GTT is necessary for correct diagnoses and its appropriate management. This review focuses on the diagnosis of GTT, and, particularly, its differentiation from GD, and unusual clinical conditions associated with GTT that require comprehensive management.

Seasonal and meteorological associations of vitamin K-dependent coagulation factors in 1-month-old infants: assessment of Normotest values.

: We aimed to determine the presence of seasonal and meteorological associations of the activity of vitamin K-dependent coagulation factors to explain the seasonal variation in vitamin K deficiency-related bleeding. Seasonal and monthly changes in Normotest values in 1759 healthy 1-month-old infants were retrospectively accessed, and the impact of meteorological parameters on Normotest values was analyzed. Normotest values peaked in winter and were the lowest in summer, with statistically significant differences among the seasonal values (P < 0.001). Comparing monthly variations, the values peaked in January and were the lowest in August (P < 0.001). Only the average daily air temperature significantly correlated with the Normotest values on multiple linear regression (P < 0.001) and with low Normotest values on multiple logistic regression analysis (odds ratio, 1.023; P = 0.002). Seasonal and monthly variations in Normotest values were observed in 1-month-old infants, possibly due to fluctuations in daily air temperature.

De novo ZBTB7A variant in a patient with macrocephaly, intellectual disability, and sleep apnea: implications for the phenotypic development in 19p13.3 microdeletions.

Interstitial microdeletions at chromosome 19p13.3 are frequently associated with a constellation of clinical features including macrocephaly, characteristic face, intellectual disability, and sleep apnea. Previous studies in 25 patients with 19p13.3 microdeletions have revealed loss of MAP2K2 in 24 patients and that of PIAS4 and ZBTB7A in 23 patients, suggesting that these three adjacent genes are candidate genes for the phenotypic development in 19p13.3 microdeletions. We identified a de novo likely pathogenic heterozygous missense variant of ZBTB7A (NM_015898.3:c.1152C>G, p.(Cys384Trp)) in a Japanese boy with macrocephaly, intellectual disability, and sleep apnea. This variant affects the conserved cysteine residue forming the coordinate bond with Zn2+ ion at the first zinc finger domain, and is predicted to exert a dominant-negative effect because of the generation of homo- and hetero-dimers with the wild-type and variant ZBTB7A proteins. The results argue for a critical relevance of ZBTB7A to the development of most, but probably not all, of the 19p13.3 microdeletion phenotype.

Late-onset glucocorticoid-responsive circulatory collapse in premature infants.

Late-onset glucocorticoid-responsive circulatory collapse (LGCC) in infants is characterized by sudden onset of hypotension and/or oliguria, which is resistant to volume expanders and inotropes but responds rapidly to intravenous glucocorticoids. LGCC occurs after the first week of life mainly in relatively stable very low birth weight (VLBW) infants. In Japan, the incidence of LGCC is reported to be 8%. Relative adrenal insufficiency (AI) is considered the most likely cause of LGCC, but its detailed pathophysiology remains unclear. Intrinsic and extrinsic factors may affect the pathophysiological mechanism. LGCC should be recognized as one of the high-risk complications in VLBW infants and managed promptly and properly, because if it is not, it may cause life-long neurological problems. To diagnose relative AI, an accurate evaluation of adrenal function is necessary; however, the interpretation of basal serum cortisol levels is difficult in preterm infants after 7 days of life. To recognize LGCC, it is recommended that blood pressure and urine volume be carefully monitored, even outside of the transitional period. If no underlying causes are documented or volume expansion and inotropic support fail, intravenous hydrocortisone should be initiated, and an additional dose of hydrocortisone is required when the response is inadequate. There are few reports to verify or characterize LGCC and this phenomenon has not been recognized worldwide to date. This review summarizes the current knowledge about LGCC in premature infants and evaluates the most significant new findings regarding its pathophysiology, treatment, and prognosis.

Current knowledge about the in utero and peripartum management of fetal goiter associated with maternal Graves' disease.

Maternal Graves' disease is the most common cause of fetal goiter. Fetal goiter can cause complications attributable either to the physical effects of the goiter itself or to thyroid dysfunction, which can be life-threatening and cause neurological impairment. Determining whether a goiter is caused by fetal hyperthyroidism or hypothyroidism is the main clinical problem, and in utero evaluations and management are essential. Ultrasonography combined with color Doppler and magnetic resonance imaging are helpful for the initial diagnosis and monitoring, but these imaging techniques have a limited ability to discriminate between fetal hyperthyroidism and hypothyroidism. To determine the fetal thyroid status, fetal blood sampling using cordocentesis is reliable but hazardous, and the indications must be considered carefully. Amniocentesis is an easier and safer alternative, but the correlations between the amniotic fluid and fetal serum thyroid hormone levels remain unclear. If a fetal goiter is accompanied by hypothyroidism, administering thyroid hormone intra-amniotically may be effective and relatively safe. However, the wide variety of approaches to treatment exemplifies the lack of guidelines, and no systematic studies have been conducted to date. Therefore, intrauterine treatment should be reserved for selected patients at a high risk of complications. Moreover, when intrauterine treatment fails and a fetal goiter can cause airway obstruction, intrapartum management, such as ex utero intrapartum treatment, may be required; however, reports describing the use of this procedure for fetal goiter are limited. This review summarizes the current knowledge about fetal goiter associated with maternal Graves' disease and evaluates the most significant new findings regarding its in utero and peripartum management.