Uterine conservation despite severe sepsis in a case of placenta accreta first treated conservatively: 3-month delayed successful removal of the placenta.

OBJECTIVE: To report a case of successful delayed removal of a placenta accreta first treated conservatively. Secondary infectious complications can occur after conservative management in cases of placenta accreta, most often leading to hysterectomy. DESIGN: Case report. SETTING: A French teaching hospital. PATIENT(S): A 33-year-old woman. INTERVENTION(S): A healthy 33-year-old woman underwent Cesarean section for her first pregnancy. Diagnosis of placenta accreta was made at ultrasound scanning for her second pregnancy. She was first treated conservatively. Hysterectomy was planned 3 months after conservative treatment because of sepsis attributed to uterine retention. (Hysterotomy was first realized.)-?? MAIN OUTCOME MEASURE(S): Uterine conservation. RESULT(S): The placenta was easily and successfully removed with no subsequent bleeding. The uterus was sutured and conserved. CONCLUSION(S): In cases of delayed sepsis because of uterine retention after conservative treatment for placenta accreta, when medical treatment remains unsuccessful, manual removal of the placenta should be attempted. This approach might allow improved uterine conservation rates in women with placenta accreta treated conservatively.

Diaphanospondylodysostosis (DSD): confirmation of a recessive disorder with abnormal vertebral ossification and nephroblastomatosis.

We report on four patients from three families, with similar radiological findings: absent (or severely delayed) ossification of vertebral bodies and associated anomalies. The babies were stillborn or died soon after birth of respiratory insufficiency. Two patients are sibs (female and male) born to first cousin Malian parents. The two others were non-consanguineous. This perinatally lethal entity comprises short neck, short wide thorax, and normally shaped limbs. Associated, inconstant anomalies are myelomeningocele, cystic kidneys with nephrogenic rests (in the sibs), and cleft palate. Radiologically, the hallmarks are absence of ossification of the vertebral bodies and sacrum, abnormal position of the vertebral pedicles, which are lamellar and angulated, ribbon-like ribs reduced in number, narrow pelvis, upward widening of the iliac wings, and unusual tilt of the ischiopubic rami, contrasting with the normal appendicular skeleton. Maroteaux briefly described one of the patients in the 2002 edition of "Maladies osseuses de l'enfant" and three sibs with similar renal and radiological findings were reported in 2003 in this Journal. Combined with the latter cases, these four new patients allow delineation of a specific lethal AR syndrome with ossification defect of the axial skeleton and renal dysplasia. We propose to name this entity diaphanospondylodysostosis.