A Treatment Algorithm for Patients Presenting with Sagittal Craniosynostosis after the Age of 1 Year.

BACKGROUND: Sagittal craniosynostosis typically presents shortly after birth, with a scaphocephalic head shape, and is addressed surgically for functional and aesthetic concerns. This study highlights the authors' experience with a challenging patient population: those with phenotypically mild, missed, and late-developing sagittal craniosynostosis. METHODS: A prospective cohort study was conducted for all cases of sagittal craniosynostosis presenting to the authors' institution between July of 2013 and December of 2015. Patients older than 1 year with isolated sagittal craniosynostosis were included. All children were evaluated by craniofacial surgery, neurosurgery, and ophthalmology departments. All patients had dilated fundus examinations and visual evoked potentials. RESULTS: Fifty-two patients met inclusion criteria. Only nine patients have been treated surgically (17.3 percent). Two patients underwent operative correction for obvious scaphocephaly. Four patients who presented with concerning ophthalmologic evaluations and another patient with classic intracranial hypertension-related headaches underwent cranial vault expansion. Eight patients presented with inconclusive ophthalmologic evaluations. These patients were admitted for intracranial pressure monitoring, of which two were found to have elevated levels (25 percent) and underwent operative intervention. Thirty-seven other patients presented with isolated sagittal craniosynostosis in the setting of overall normocephaly without any signs concerning for intracranial hypertension. These patients continue to undergo serial evaluation. CONCLUSIONS: The authors describe their treatment protocol for a large series of patients presenting with the delayed diagnosis of sagittal craniosynostosis. Based on the early experience of the authors' center with this protocol, the risk of intracranial hypertension appears to be low in this population. The majority of patients to date have been managed nonsurgically without invasive monitoring. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Improving speech outcomes after failed palate repair: evaluating the safety and efficacy of conversion Furlow palatoplasty.

BACKGROUND: Velopharyngeal insufficiency occurs in a nontrivial number of cases following cleft palate repair. We hypothesize that a conversion Furlow palatoplasty allows for long-term correction of VPI resulting from a failed primary palate repair, obviating the need for pharyngoplasty and its attendant comorbidities. METHODS: A retrospective review of patients undergoing a conversion Furlow palatoplasty between 2003 and 2010 was performed. Patients were grouped according to the type of preceding palatal repair. Velopharyngeal insufficiency was assessed using Pittsburgh Weighted Speech Scale (PWSS). Scores were recorded and compared preoperatively and postoperatively at 3 sequential visits. RESULTS: Sixty-two patients met inclusion criteria and were grouped by preceding repair (straight-line repair (n = 37), straight-line repair with subsequent oronasal fistula (n = 14), or pharyngeal flap (n = 11). Median PWSS scores at individual visits were as follows: preoperative = 11, first postoperative = 3 (mean, 114.0 ± 6.7 days), second postoperative = 1 (mean, 529.0 ± 29.1 days), and most recent postoperative = 3 (mean, 1368.6 ± 76.9 days). There was a significant difference between preoperative and postoperative PWSS scores in the entire cohort (P < 0.001) with overall improvement, and post hoc analysis showed improvement between each postoperative visit (P < 0.05) with the exception of the second to the most recent visit. There were no differences between postoperative PWSS scores in the operative subgroupings (P > 0.05). Eight patients failed to improve and showed no differences in PWSS scores over time (P > 0.05). Patients with a PWSS score of 7 or greater (n = 8) at the first postoperative visit (0-6 months) displayed improvement at the most recent visit (P< 0.05). CONCLUSIONS: Conversion Furlow palatoplasty is an effective means for salvaging speech. Future studies should elucidate which factors predict the success of this technique following failed palate repair.

Divided parietal bone in plagiocephaly.

Supernumerary cranial sutures represent a rare cause of plagiocephaly. In the case of an extra suture dividing the parietal bone, the presenting features are often inconsistent with the typical presentation of deformational or synostotic plagiocephaly. Disagreement exists as to how the presence of this suture affects the shape of the skull. We present a case of a supernumerary suture in the parietal bone leading to plagiocephaly and discuss the role this suture plays in cranial growth.