ABSTRACT
We describe a case of twins with twin-to-twin transfusion syndrome (TTS) who were found to have renal tubular dysgenesis (TRD)-like lesions and hypocalvaria attributed to indomethacin treatment of the mother for acute polyhydramnios. History of pregnancy, postnatal clinical course, pathological findings of the kidneys, and the skulls are presented and discussed. These findings include incompletely differentiated proximal tubules in the kidneys and hypoplastic calvaria in both twins. The renal tubular lesions were more marked in the donor than in the transfused twin, probably due to the greater degree of ischemia in that twin. This seems to be in favor of a vascular etiology of the renal defects. However, the fact that similar renal lesions and hypocalvaria were also present in the transfused twin seems to indicate that indomethacin played a role in their onset. This so-called kidney-skull connection has never been reported in conjunction with indomethacin therapy.
Subject(s)
Fetofetal Transfusion/pathology , Indomethacin/adverse effects , Kidney Tubules, Proximal/abnormalities , Polyhydramnios/drug therapy , Skull/abnormalities , Tocolytic Agents/adverse effects , Adult , E2F6 Transcription Factor , Fatal Outcome , Female , Fetofetal Transfusion/etiology , Gestational Age , Humans , Immunoenzyme Techniques , Infant, Newborn , Polyhydramnios/complications , Pregnancy , Repressor Proteins/analysis , Transcription Factors/analysisABSTRACT
The authors report the first successful case of fetal exchange transfusion performed in Rh disease. The patient was a gravida 3, para 1; a first ultrasound guided intra-funicular transfusion was carried out at 28 gestation weeks (Coombs test: 256, Liley's chart: zone III, fetal hemoglobin: 5.7 g/l). One week later a fetal exchange transfusion was decided because of the appearance of ascites, sinusoidal heart rate pattern, and Manning's score at 4. At 29 weeks of gestation an ultrasound-guided umbilical cord puncture was performed with a 16-gauge Tuohy needle, a catheter for epidural analgesia (Perifix) was inserted through the trocar. A total exchange of +126/-96 cm3 of 50% hematocrit (Hct) concentrated and irradiated blood without leukocyte and platelet was performed increasing fetal hemoglobin concentration from 3.9 to 11.9 g/l. A 2,040-gram girl was delivered by cesarean section at 33 gestation weeks with Apgar score of 7/9/9 and she was given 4 total exchange transfusions in the Neonatal Intensive Care Unit. Two years later her development is normal. This new procedure seems easier than fetoscopy to achieve umbilical cord puncture and avoid excess blood volume while exact correction of anemia is possible. With more concentrated blood (70% Hct) the duration of the procedure is shortened.
