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Mitchel Seruya, Albert K. Oh, Michael J. Boyajian, Jeffrey C. Posnick and Robert F. Keating

Object

Patients with delayed presentation of isolated sagittal synostosis (ISS) pose unique surgical challenges. Intracranial hypertension can be missed in the absence of overt findings. Here, an algorithm is presented for the elucidation of intracranial hypertension and approaches to surgical treatment during calvarial reconstruction.

Methods

Patients with delayed presentation (age > 15 months) of ISS between 1997 and 2009 were identified. Symptoms, signs, and radiological evidence of intracranial hypertension were noted. Intraoperative management included calvarial reconstruction in conjunction with ventriculostomy for intracranial pressure (ICP) monitoring and CSF drainage in the setting of suspected intracranial hypertension.

Results

Seventeen patients underwent calvarial reconstruction for delayed presentation of ISS. The mean surgical age was 40.5 months (16.2–82.9 months), and the average follow-up was 34.2 months (0.6–92.2 months). Eleven patients with subtle findings of intracranial hypertension underwent ICP monitoring during calvarial reconstruction. The mean opening ICP was 23.5 cm H2O (16.5–29.5 cm H2O), and the mean closing ICP was 7.0 cm H2O (3.5–17.0 cm H2O). Nine (81.8%) of 11 monitored patients demonstrated intracranial hypertension (ICP ≥ 20 cm H2O); the other 2 had borderline increased ICP. Perioperative morbidity was 5.9%, with 1 patient medically treated for transient, new-onset intracranial hypertension. The postoperative Whitaker category was I in 94.1% of patients, II in 5.9%, and III/IV in 0%. There were no reoperations or deaths.

Conclusions

In this consecutive series, 11 of 17 patients with delayed presentation of ISS underwent ICP monitoring during calvarial reconstruction as a result of subtle clinical findings of intracranial hypertension. Nine (81.8%) of 11 monitored patients demonstrated intracranial hypertension. Calvarial reshaping along with ICP monitoring and CSF drainage facilitated reconstruction and resulted in good outcomes and the resolution of intracranial hypertension.

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Benjamin C. Wood, Albert K. Oh, Robert F. Keating, Michael J. Boyajian, John S. Myseros, Suresh N. Magge and Gary F. Rogers

OBJECT

Progressive postnatal pansynostosis (PPP) is a rare form of craniosynostosis that is characterized by a normal head shape, insidious decrease in percentile head circumference, and high rates of elevated intracranial pressure (ICP). This investigation describes the clinical, radiographic, and genetic features of this entity.

METHODS

The authors’ craniofacial database for the period 1997–2013 was retrospectively culled to identify patients who had a normal or near-normal head shape and CT-confirmed multiple-suture synostosis. Patients with kleeblatt-schädel or previous craniofacial surgery were excluded. All demographic information was collected and analyzed.

RESULTS

Seventeen patients fit the inclusion criteria. Nine patients had a syndromic diagnosis: Crouzon syndrome (n = 4), Pfeiffer syndrome (n = 2), Saethre-Chotzen syndrome (n = 1), Apert syndrome (n = 1), and achondroplasia (n = 1). With the exception of 3 patients with mild turricephaly, all patients had a relatively normal head shape. Patients were diagnosed at an average age of 62.9 months. Nearly all patients had some combination of clinical, radiographic, or ophthalmological evidence of increased ICP.

CONCLUSIONS

PPP is insidious; diagnosis is typically delayed because the clinical signs are subtle and appear gradually. All normocephalic infants or children with a known or suspected craniosynostotic disorder should be carefully monitored; any decrease in percentile head circumference or signs/symptoms of increased ICP should prompt CT evaluation.

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Mitchel Seruya, Albert K. Oh, Michael J. Boyajian, John S. Myseros, Amanda L. Yaun and Robert F. Keating

Object

Intraoperative blood loss represents a significant concern during open repair of craniosynostosis, and its reliable measurement remains a serious challenge. In this study of extended sagittal synostectomies, the authors analyzed the relationship between estimated blood loss (EBL) and calculated blood loss (CBL), and investigated predictors of hemodynamic outcomes.

Methods

The authors reviewed outcomes in infants with sagittal synostosis who underwent primary extended synostectomies (the so-called Pi procedure) between 1997 and 2009. Patient demographic data, operating time, and mean arterial pressures (MAPs) were recorded. Serial MAPs were averaged for a MAPmean. The EBL was based on anesthesia records, and the CBL on pre- and postoperative hemoglobin values in concert with transfusion volumes. Factors associated with EBL, CBL, red blood cell transfusion (RBCT), and hospital length of stay (LOS) were investigated. Hemodynamic outcomes were reported as percent estimated blood volume (% EBV), and relationships were analyzed using simple and multiple linear and logistic regression models. A p value < 0.05 was considered significant.

Results

Seventy-one infants with sagittal synostosis underwent primary extended synostectomies at a mean age and weight of 4.9 months and 7.3 kg, respectively. The average operating time was 1.4 hours, and intraoperative MAP was 54.6 mm Hg (21.3% lower than preoperative baseline). There was no association between mean EBL (12.7% EBV) and mean CBL (23.6% EBV) (r = 0.059, p = 0.63). The EBL inversely correlated with the patient's age (r = −0.07) and weight (r = −0.11) at surgery (p < 0.05 in both instances). With regard to intraoperative factors, EBL positively trended with operating time (r = 0.26, p = 0.09) and CBL inversely trended with MAPmean (r = −0.04, p = 0.10), although these relationships were only borderline significant. Intraoperative RBCT, which was required in 59.1% of patients, positively correlated with EBL (r = 1.55, p < 0.001), yet negatively trended with CBL (r = −0.40, p = 0.01). Undertransfusion was significantly more common than overtransfusion (40.8% vs 22.5%, p = 0.02, respectively). The mean hospital LOS was 2.3 days and was not significantly associated with patient demographic characteristics, intraoperative factors, blood loss, RBCT, or total fluid requirements.

Conclusions

In extended synostectomies for sagittal synostosis, EBL and CBL demonstrated a decided lack of correlation with one another. Intraoperative blood transfusion positively correlated with EBL, but inversely correlated with CBL, with a significantly higher proportion of patients undertransfused than overtransfused. These findings highlight the need for reliable, real-time monitoring of intraoperative blood loss to provide improved guidance for blood and fluid resuscitation.

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Tina M. Sauerhammer, Albert K. Oh, Michael Boyajian, Suresh N. Magge, John S. Myseros, Robert F. Keating and Gary F. Rogers

Object

Unilateral fusion of the frontoparietal suture is the most common cause of synostotic frontal plagiocephaly. Localized fusion of the frontosphenoidal suture is rare but can lead to a similar, but subtly distinct, phenotype.

Methods

A retrospective chart review of the authors' craniofacial database was performed. Patients with isolated frontosphenoidal synostosis on CT imaging were included. Demographic data, as well as the clinical and radiographic findings, were recorded.

Results

Three patients were identified. All patients were female and none had an identifiable syndrome. Head circumference was normal in each patient. The mean age at presentation was 4.8 months (range 2.0–9.8 months); 2 fusions were on the right side. Frontal flattening and recession of the supraorbital rim on the fused side were consistent physical findings. No patient had appreciable facial angulation or orbital dystopia, and 2 patients had anterior displacement of the ipsilateral ear. All 3 patients were initially misdiagnosed with unilateral coronal synostosis, and CT imaging at a mean age of 5.4 months (range 2.1–10.8 months) was required to secure the correct diagnosis. Computed tomography findings included patency of the frontoparietal suture, minor to no anterior cranial base angulation, and vertical flattening of the orbit without sphenoid wing elevation on the fused side. One patient underwent CT scanning at 2.1 months of age, which demonstrated a narrow, but patent, frontosphenoidal suture. The patient's condition was assumed to be a deformational process, and she underwent 6 months of unsuccessful helmet therapy. A repeat CT scan obtained at 10.7 months of age demonstrated the synostosis. All 3 patients underwent fronto-orbital correction at mean age of 12.1 months (range 7.8–16.1 months). The mean duration of postoperative follow-up was 11.7 months (range 1.9–23.9 months).

Conclusions

Isolated frontosphenoidal synostosis should be considered in the differential diagnosis of atypical frontal plagiocephaly.

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Mitchel Seruya, Albert K. Oh, Gary F. Rogers, Michael J. Boyajian, John S. Myseros, Amanda L. Yaun and Robert F. Keating

Object

Controlled hypotension is routinely used during open repair of craniosynostosis to decrease blood loss, although this benefit is unproven. In this study the authors analyzed the longitudinal relationships between intraoperative mean arterial pressure (MAP) and calculated blood loss (CBL) during frontoorbital advancement (FOA) for craniosynostosis.

Methods

The authors reviewed the records of infants with craniosynostosis who had undergone primary FOA between 1997 and 2009. Anesthesia records provided preoperative and serial intraoperative MAP. Interval measures of CBL had been determined during the course of the operation. The longitudinal relationships between MAPmean, MAPchange, and CBLchange were assessed over the same time interval and compared between adjacent time intervals to determine the directionality of associations.

Results

Ninety infants (44 males and 46 females) underwent FOA at a mean age and weight of 10.7 ± 12.9 months and 9.0 ± 7.0 kg, respectively. The average intraoperative MAP was 56.1 ± 4.8 mm Hg, 22.6 ± 12.1% lower than preoperative baseline. A negative correlation was found between CBLchange and MAPmean over the same interval (r = −0.31, p < 0.05), and an inverse relationship was noted between CBLchange of the previous interval and MAPchange of the next interval (r = −0.07, p < 0.05). Finally, there was no significant association between MAPchange of the previous interval and CBLchange of the next interval.

Conclusions

Calculated blood loss demonstrated a negative correlation with MAP during FOA. Directionality testing indicated that MAP did not affect intraoperative blood loss; instead, blood loss drove changes in MAP. Overall, these findings challenge the benefit of controlled hypotension during open craniofacial repair.