Separation of Thoraco-Omphalo-Ischiopagus Conjoined Twins: Surgical Planning, Management, and Outcomes.

BACKGROUND: Conjoined twins are a rare medical phenomenon that offers a unique challenge for medical professionals. The complex anatomy of conjoined twins dictates their survival and amenability to separation, making each case different in terms of medical management, surgical planning, and patient outcomes. Thoraco-omphalo-ischiopagus twins, joined from the thorax to the pelvis, are one of the rarest orientations recorded in the medical literature, and successful separation of this subset of conjoined twins has not been documented. This report presents a novel case of thoraco-omphalo-ischiopagus tetrapus twins who were successfully separated at 10 months of age. The preoperative planning, operative details, and postoperative course are discussed as they relate to the reconstructive effort. METHODS: Three-dimensional medical modeling was pursued early in the planning process and was used to estimate the soft-tissue requirements for reconstruction and to design custom tissue expanders. RESULTS: The reconstructive effort required postponement until respiratory status was optimized. Even with elaborate preoperative planning, primary closure of the abdomen was limited because of tissue edema and other less predictable patient factors. Delayed closure of the abdominal wall was made possible with negative-pressure wound therapy and secondary flap advancements. CONCLUSION: Preoperative coordination with necessary vendors, a multidisciplinary surgical effort, and optimal timing of the surgical intervention all contribute to the successful separation and long-term survival of thoraco-omphalo-ischiopagus conjoined twins. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Assessment of quality of life outcomes using the pediatric quality of life inventory survey in prenatally diagnosed congenital diaphragmatic hernia patients.

INTRODUCTION: Congenital diaphragmatic hernia patients can have significant postnatal morbidity. To date, there has been no quality of life (QOL) study in a prenatally diagnosed CDH patient population, assessing prenatal disease severity and QOL. The purpose of this pilot study was to assess the QOL in a single institution fetal center CDH experience. METHODS: The study was a retrospective cohort study of CDH patients diagnosed by fetal ultrasound and MRI at a fetal center between March 2002-March 2014. Parents of children ≥2years were contacted by telephone to participate in the validated Pediatric Quality of Life Inventory (PedsQL™, Version 4.0). RESULTS: Of 95 CDH survivors, 68 met inclusion criteria, of which 28 (42%) completed the survey (mean age, range: 5.5, 2.3-11.7years). Based on prenatal markers of disease severity, there were no differences in performance between those with mild or severe forms of CDH. Overall, patients had minimal QOL limitations, but those with limitations had a higher risk for oxygen dependence at 30days of life (71%v. 29%, OR 0.16, CI, 0.031-0.82, p=0.02). ECMO was not associated with significantly worse QOL in physical or psychosocial functioning. Cronbach's alpha reliability coefficient yielded a correlation of 0.951 for the overall survey, 0.911 for physical functioning, and 0.901 for psychosocial functioning questions. CONCLUSIONS: Patients that are prenatally diagnosed with severe forms of CDH have similar QOL outcomes across all categories of physical and psychosocial functioning. This pilot study is encouraging and may allow improved counseling for expectant parents of CDH patients.