Severity of Airway Obstruction May Not Correlate With Weight Gain or Failure to Thrive in Infants With Robin Sequence: A Pilot Study.

OBJECTIVE: Patients with Robin sequence (RS) can present with varying degrees of upper airway obstruction, difficulty maintaining adequate weight gain, and failure to thrive (FTT). Although inductive reasoning would suggest that these issues should be interrelated, the relationships between these factors have not been formally studied. This investigation explores the correlation between polysomnographic (PSG) findings, weight gain, and FTT in patients with RS. DESIGN: A prospective database for baseline PSG parameters and serial weight measurements in infants with RS who were admitted for airway obstruction was reviewed. The association between PSG variables and calorie intake with FTT was assessed using univariate and multivariable logistic regression. Categorical analysis of the PSG variables against FTT was explored with a Poisson regression, and linear regression was performed to evaluate the correlation between PSG parameters and percentage of weight gain. RESULTS: Univariate and multivariate logistic regression in RS patients with (n = 13) and without (n = 20) FTT showed no significant association between apnea-hypopnea index (adjusted odds ratio [aOR]: 0.99, P-value = 0.403), O2 nadir (aOR: 0.98, P-value = 0.577), time of O2 saturation below 90% (aOR: 1.03, P-value = 0.574), maximum end tidal carbon dioxide (aOR: 1.0, P-value = 0.977), and average calorie intake (OR:1.02; P-value = 0.984). Furthermore, no significant associations were identified between these variables and weight gain. CONCLUSIONS: This pilot study questions the widely held and intuitively logical belief that poor weight gain and/or FTT should correlate with the severity of upper airway obstruction in patients with RS. Large prospective investigations should be initiated to better explore the authors' findings. Our results also underscore the importance of individualized treatment for these challenging patients.

Management and Reconstruction of a Massive Neonatal Neck Teratoma.

Cervical teratoma is a rare tumor comprised of multiple tissue types. These masses can result in significant functional and aesthetic complications, and surgical intervention is the mainstay of treatment. The authors report the treatment and 8-year follow-up of a patient born with a massive cervicofacial teratoma. The mass was diagnosed in utero and required perinatal airway management. The patient underwent several procedures to enhance his appearance and function early on in life with an excellent outcome at intermediate follow-up.

Heminasal Reconstruction Utilizing Presurgical Planning, Template-Based Cartilage Reconstruction and Tissue Expansion.

Heminasal aplasia is a rare congenital nasal anomaly in which there is unilateral deficiency in both the external nasal anatomy and nasal airway. Unilateral failure in development of a nasal placode in embryogenesis is thought to be the underlying cause of this anomaly. The authors describe the reconstruction of heminasal aplasia in a teenager utilizing a templated cartilaginous framework and tissue expansion. The authors feel the satisfactory results of this technique will be of benefit to other surgeons who may encounter this rare anomaly.

Correction of Microstomia Reconstruction With the Use of Acellular Dermal Matrix for Buccal Reconstruction.

Correction of microstomia is challenging with a high rate of recurrence. We report the successful treatment of microstomia using acellular dermal matrix (ADM) as an adjunct for intraoral lining with >1 year of follow-up.A 9-year-old international patient with severe immunodeficiency presented with severe microstomia because of recurrent oral infections. She had undergone 3 previous failed attempts to re-establish an adequate oral opening and was dependent on enteral nutrition via gastrostomy tube. She underwent release of the oral commissure scar contracture and orbicularis oris and the resultant mucosal defect was lined with ADM. A postoperative splint was used for 8 weeks. One-year follow-up demonstrated maintenance of the oral aperture with complete mucosalization of the ADM; the patient was able to resume oral diet and regular dental hygiene.Mucosal reconstruction with ADM is a viable alternative to local flaps and in this case exhibited minimal soft tissue contraction.

The "Double" Tessier 7 Cleft: An Unusual Presentation of a Transverse Facial Cleft.

Congenital macrostomia, or Tessier number 7 cleft, is a rare craniofacial anomaly. We present a unique patient with bilateral macrostomia that consisted of a "double" transverse cleft on the left side and a single transverse cleft on the right side. A staged reconstructive approach was used to repair the "double" left-sided clefts. This staged technique produced a satisfactory aesthetic and functional outcome.

Factors Predicting Hospital Length of Stay Following Pharyngeal Flap for Velopharyngeal Insufficiency.

OBJECTIVE: Routine hospital admission following pharyngeal flap (PF) to correct velopharyngeal insufficiency (VPI) is the standard at most hospitals. Nevertheless, there is increasing resistance from third-party providers to approve stays longer than a "short stay" (23-hour) observation period. The purpose of the current study was to evaluate length of stay (LOS) and document potential influencing factors following PF. DESIGN: Retrospective chart review. Demographic and perioperative data were collected, and statistical analyses were performed to determine associations with hospital length of stay (LOS). Readiness for discharge was determined by oral intake, analgesic requirement, and respiratory status. SETTING: Tertiary care children's hospital Participants: All patients undergoing PF for VPI between 1990 and 2014. OUTCOME MEASURES: (1) LOS, (2) % satisfying all discharge criteria within a 23-hour observational time frame. RESULTS: Seventy-five patients were studied, with an average age of 6.8 years. Mean LOS was 65.4 hours. Only 11 patients (14.9%) met all discharge criteria by 23 hours. Multivariate predictors of shorter LOS were increasing patient age, male gender, lack of syndromic association, administration of an intraoperative antiemetic and steroids, and shorter anesthetic duration. Time to first oral intake correlated positively with LOS. Administration of intraoperative antiemetics increased the odds of meeting all discharge criteria within 23 hours by a factor of 12. CONCLUSIONS: Identification of factors associated with LOS after PF may allow providers to predict and potentially mitigate hospital stays. Nevertheless, most patients undergoing PF are not clinically ready for discharge within a short-stay (23-hour) observation period.

Conjoined Twin Separation: Integration of Three-Dimensional Modeling for Optimization of Surgical Planning.

Conjoined twinning is a rare anomaly, with an incidence of approximately 1 in 100,000 live births. There is a high perinatal mortality rate, but twins who survive pose reconstructive challenges that require meticulous preoperative planning. The authors describe the senior surgeon's career experience with conjoined twin separation, and the evolution of medical modeling and 3-dimensional imaging as a critical component in presurgical planning.The authors performed a retrospective review of all consecutive patients of conjoined twin separation at a single institution from January 2004 to December 2013. Data were collected related to patient demographics, comorbidities, operative technique, perioperative complications, survival, long-term outcomes, and the type of medical modeling system used for preoperative planning.Five sets of conjoined twins underwent separation during the 10-year study period. There were 3 sets of thoraco-omphalopagus twins, 1 set of pyopagus twins, and 1 set of ischiopagus tetrapus twins. The mean age at separation was 70 days, with a mean of 3.5 surgical procedures performed per patient during the first year of life. One set of twins experienced postseparation complications that warranted immediate return to the operating room. The overall survival rate after separation was 70%. The imaging methods used were computed tomography scan with 3-dimensional reconstruction, plaster molds, medical modeling with composite printing, and virtual surgical planning.The use of imaging and medical modeling in presurgical planning has proven to be an important element in optimizing the outcomes for patients with this rare anomaly.

Clinical Factors Affecting Length of Stay After 100 Consecutive Cases of Primary Cleft Lip Repair.

OBJECTIVE: To analyze the hospital course of 100 consecutive infants after primary cleft lip repair (PCLR) and identify factors related to length of stay (LOS). DESIGN: Retrospective analysis of 100 consecutive infants who were routinely admitted after PCLR. SETTING: Tertiary care center. PATIENTS: One hundred consecutive infants undergoing PCLR. Demographic and perioperative data were collected and analyzed. MAIN OUTCOME MEASURE: LOS, planned before data collection. RESULTS: Male:female ratio was 65:35. Seventy-two infants had unilateral cleft lip; syndromic association was documented in 15 patients. Mean age and weight at PCLR were 5.6 ± 4.0 months and 6.7 ± 1.3 kg, respectively. Mean duration of surgery was 2.5 ± 0.9 hours, and mean duration of general anesthesia was 3.4 ± 0.9 hours. A total of 3.3 ± 1.5 mL of intraoperative local anesthetic was used per patient. Intravenous fluids were necessary after transfer from the post-anesthesia care unit to the general ward in 98% of patients. Almost half (44%) of all patients received intravenous morphine 23 hours or more after hospital admission. Mean LOS was 35.8 ± 13.9 hours. No association was identified between patient demographic factors and LOS. Multivariate linear regression models identified significant positive correlation between LOS and duration of general anesthesia (P = .002). Greater volume of postoperative oral intake (P = .000) and higher acetaminophen dosage on the floor (P = .000) correlated with decreased LOS. CONCLUSIONS: This study identifies perioperative factors associated with LOS. Our findings question the safety of routine outpatient or short-stay observation after PCLR.

Endocortical plating of the bandeau during fronto-orbital advancement provides safe and effective osseous stabilization.

BACKGROUND: Ectocortical resorbable plate fixation has become a standard method of fixation during fronto-orbital advancement (FOA) in young children. Plate hydrolysis occurs slowly and can cause visible prominences, sterile abscesses, and osseous depressions that can persist after complete resorption. Although endocortical placement avoids contour issues, the safety and effectiveness of this technique are undocumented. METHODS: A review of our prospectively collected craniofacial database was performed. All patients undergoing FOA by a single craniofacial team at a single institution from 1997 to 2011 were examined. Inclusion criteria were as follows: (1) unicoronal, bicoronal, or metopic synostosis; (2) resorbable endocortical fixation of the bandeau; and (3) follow-up for 1 year or longer. Evaluation included patient demographic data, postoperative clinical course, and computed tomography imaging when available. RESULTS: Seventy-three patients met the inclusion criteria. Fusion involved the unicoronal (n = 26), bicoronal (n = 19), and metopic (n = 28) sutures. Mean age at operation was 8.3 months (range, 2.7-35.5 mo), and follow-up was 4.5 years (range, 1.0-9.9 y). No endocortical or ectocortical sterile abscesses were documented in our series. Postoperative complications included hematoma (n = 2), infection (n = 2), wound breakdown (n = 3), cerebral contusion (n = 2), and cerebrospinal fluid leak (n = 1); none of these issues were related to endocortical absorbable fixation. Fifty-eight patients (80%) were categorized as Whitaker classification I/II; and 15 patients (20%), Whitaker classification III/IV. Postoperative computed tomography (mean follow-up, 4.6 y) was obtained in 34 patients (47%). All plates were completely resorbed, and there were no bone or soft tissue irregularities in the region where the plates were placed. CONCLUSIONS: Endocortical resorbable fixation is a safe and effective method of osseous stabilization during FOA for craniosynostosis in young children.

Incidental findings on preoperative computed tomography for nonsyndromic single suture craniosynostosis.

Although the diagnosis of nonsyndromic single suture craniosynostosis (NSSC) can usually be made by clinical examination, computed tomography (CT) is still commonly used in preoperative evaluation. This practice has been questioned in light of recent studies that document a small, but measurable, increased risk of malignancy from CT-associated radiation. The purpose of this study was to examine whether preoperative CT for patients with NSSC provided clinically important information beyond confirmation of craniosynostosis. We performed a retrospective analysis of all patients with NSSC undergoing cranial vault remodeling at our center from March 1999 to March 2011. Only patients with complete preoperative CT scans available for review were included. Staff pediatric neurosurgeons were blinded to patient diagnosis and official radiology report, analyzed the CT images, and documented the site of synostosis and any other findings. Of the 231 patients, 80 met the inclusion criteria. Sites of synostosis included sagittal (51 patients), coronal (17 patients), metopic (11 patients), and frontosphenoidal (1 patient). Clinical diagnosis correlated with radiographic site of fusion in all patients except the patient with frontosphenoidal synostosis. Incidental findings were documented in more than 50% of the patients including prominent extra-axial cerebrospinal fluid (n = 36, 45%), ventriculomegaly (n = 5, 6.25%), choroid fissure cyst (n = 2), cavum septum pellucidum (n = 2), Chiari malformation (n = 1), and prominent perivascular space (clinically nonsignificant finding, n = 1). Incidental findings required additional follow-up or management in 5 patients (6.25%). Our findings support the use of preoperative imaging in this population to identify intracranial anomalies that cannot be discerned by clinical examination. Whereas many findings were not clinically important, some required additional attention.

A Retrospective Review of Patient-reported Outcomes after Postaxial Polydactyly Ligation and Surgical Excision.

Background: Interventions for type B postaxial polydactyly include suture ligation and surgical excision, yet there is a paucity of literature comparing the outcomes of these procedures. This study sought to compare patient-reported long-term outcomes of postaxial digit excision. Methods: A six-question survey was distributed from January 2021 to March 2022 to patients who underwent treatment for type B postaxial polydactyly at a single pediatric institution from 2010 to 2016. Patients were queried about the incidence of pain sensitivity, keloid healing, and/or persistent presence of bump ("nubbin") at the treatment site. Results: A total of 158 responses accounting for 258 digits were attained for a 53% response rate. The majority of digits (67.4%, n = 174) were surgically excised. Median age at procedure was 49 days: 13.0 days for ligation, 63.0 days for surgical excision. Median age at survey was 8 [IQR 5.4-10.2] years. Short-term (<30 days after procedure) complications rate was 1.6%. The rate of a raised or sensitive scar was 39.5% (ligation 51.5% versus surgery 35.4%, P < 0.05). The likelihood of postoperative sensitivity (P = 0.80) was similar among groups. However, the odds of a residual bump or raised scar at the surgical site was significantly higher in the ligation group (P = 0.001). These findings remained significant in the adjusted analysis. Conclusion: This study suggests that suture ligation can be used in select cases without increasing the prevalence of long-term pain or sensitivity, albeit with greater risk of a bump or raised scar at the excision site compared with surgical excision.

Age at initial consultation for craniosynostosis: comparison across different patient characteristics.

BACKGROUND: The severity and dysmorphology that results from the premature fusion of one or more cranial sutures is not uniform. Less striking phenotypes may be more easily missed on routine screening, possibly leading to delayed diagnosis and treatment. The purpose of this study was to compare the age at initial presentation for the different forms of craniosynostosis. METHODS: The authors reviewed the records of all patients who underwent open craniofacial repair of craniosynostosis at a single institution from 1996 to 2009. Relationships between type of suture fusion and age at initial consultation were compared. RESULTS: Two hundred eleven patients (136 males, 75 females) were identified. Indications included sagittal (n = 96), metopic (n = 39), unicoronal (n = 33), bicoronal (n = 24), multisutural (n = 15), bilambdoidal (n = 3), and unilambdoidal (n = 1) synostoses. Seventeen patients (8.1%) had a craniosynostosis syndrome and 5 (2.4%) had a syndrome or disorder not typically associated with craniosynostosis [X-linked hypophosphatemic rickets (n = 3), achondroplasia (n = 1), and Beckwith Wiedemann (n = 1)]. Median age at initial consultation was 4.1 months; there was no gender difference. Patients with X-linked hypophosphatemic rickets presented at a significantly older age than nonsyndromic patients or those with a known craniosynostosis syndrome. Those with multisutural synostosis presented at a significantly older age than patients with sagittal or bicoronal synostosis. CONCLUSIONS: Patients with multisutural involvement or X-linked hypophosphatemic rickets had a significant delay in presentation for craniosynostosis. The latter group of patients may especially benefit from routine surveillance for craniosynostosis given their advanced age at diagnosis.

Factors related to blood loss during fronto-orbital advancement.

BACKGROUND: Blood loss during fronto-orbital advancement (FOA) remains a significant potential source of morbidity. This study explored variables that might correlate with calculated blood loss (CBL) during this procedure. METHODS: The authors reviewed infants with craniosynostosis who underwent primary FOA (1997-2009). Patient demographics, operative time, and mean arterial pressure (MAP) were recorded. Serial MAPs were averaged for a MAPmean and subtracted from preoperative baseline to calculate MAP%decrease. This provided indicators of both absolute and relative hypotension, respectively. Calculated blood loss was based on preoperative/postoperative hemoglobin values and transfusion volumes and accounted for hemodilutional effects. RESULTS: Ninety infants underwent FOA at an average age of 10.7 ± 12.9 months and mean weight of 9.0 ± 7.0 kg. Average operative time was 4.2 hours, and intraoperative MAP was 56.1 mm Hg, 22.6% lower than baseline. Mean CBL was 259.3 mL, or 39.3% of estimated blood volume, negatively correlating with surgical age (r = -0.033, P < 0.05) and positively trending with operative time (r = 0.55, P < 0.05). Absolute hypotension was associated with greater blood loss, as demonstrated by an inverse relationship between CBL and MAPmean (r = -0.19, P < 0.05). From the perspective of relative hypotension, no association was found between CBL and MAP%decrease. CONCLUSIONS: Greater operative efficiency and deferring operative correction to a later age may diminish blood loss during FOA. The study results also raise serious concerns regarding the hemodynamic benefits of controlled systemic hypotension.

Blood loss estimation during fronto-orbital advancement: implications for blood transfusion practice and hospital length of stay.

BACKGROUND: Reliable measurement of intraoperative blood loss remains a serious challenge during correction of craniosynostosis. This study analyzed the relationship between estimated blood loss (EBL) and calculated blood loss (CBL) in fronto-orbital advancement and its implications on blood transfusion practice and hospital length of stay (LOS). METHODS: The authors reviewed infants who underwent primary fronto-orbital advancement for craniosynostosis (1997-2009). Estimated blood loss was based on anesthesia records and CBL by preoperative/postoperative hemoglobin. Perioperative red blood cell transfusion (RCT) and hospital LOS were recorded. RESULTS: Ninety infants were included. Mean EBL was 42.2% of estimated blood volume (% EBV), and CBL was 39.3% EBV, without significant difference (P = 0.23). Bland-Altman analysis revealed that EBL was greater than CBL at lower levels of blood loss (≤47.0% EBV) and less than CBL at higher levels (>47.0% EBV). Mean intraoperative RCT was 45.8% EBV; overtransfusion was more frequent at lower levels of bleeding, and undertransfusion at higher levels. Postoperative RCT occurred more frequently with greater blood loss. Mean LOS was 3.7 days, increasing with CBL (hazard ratio of discharge, HR(discharge) = 0.988, P < 0.01), postoperative RCT (HR(discharge) = 0.96, P < 0.05), total RCT (HR(discharge) = 0.991, P < 0.05), and total intraoperative fluid (HR(discharge) = 0.999, P < 0.05). CONCLUSIONS: Estimated blood loss is a less accurate marker for CBL at the extremes of blood loss during fronto-orbital advancement. The tendency to overestimate blood loss with less intravascular volume loss can result in unnecessary transfusion, whereas underestimation with greater actual blood loss can lead to delay in resuscitation and longer hospitalization.

Pediatric Ectopic Nail Formation following Fingertip Trauma: A Case Report and Literature Review.

Post-traumatic ectopic nail is an uncommon entity that is occasionally observed after trauma to the fingertip and nail, resulting in aesthetic and functional morbidity. We report a case of post-traumatic ectopic nail in a 3-year-old girl following trauma to her index finger and subsequent surgical intervention to remove an inclusion cyst. The unusual clinical sequence is presented to highlight the etiology and treatment of this rare lesion.

Fistula after 2-flap palatoplasty: a 20-year review.

Oronasal fistula formation is a recalcitrant complication following palatoplasty, resulting in nasal emission during speech and deglutition. We review our series to identify factors associated with fistula incidence. A retrospective review of all children with nonsyndromic cleft palate who underwent 2-flap palatoplasty by the senior author from July 1983 to August 2004, was performed. Patient demographics, cleft characteristics, and operative techniques were recorded for each patient. The incidence rates of fistula, pharyngeal flap, and reoperation were used as primary outcomes. Statistical comparisons of frequencies were performed using Fisher exact test. Comparisons of means were performed using chi2 analysis. A total of 332 consecutive children met inclusion criteria. Mean age at palatoplasty was 10.8 months, and mean follow-up was 74.1 months. Eight children (2.4%) were found to have fistulae postoperatively, ranging in size from 2 to 15 mm. Four palatal fistulas occurred in the soft palate, 2 at the junction of the hard and soft palate, 1 in the hard palate, and 1 at the incisive foramen. Symptomatic nasal emission requiring reoperation occurred in 5 children. Two of these 5 children required a second operation to achieve fistula closure. Forty pharyngeal flaps were required for correction of velopharyngeal incompetence (12.0%). Two-flap palatoplasty remains a highly successful technique for closure of a variety of palatal clefts, with low fistula incidence. Surgical technique and experience are factors associated with low fistula incidence.

Upper Extremity Friction Burns in the Pediatric Patient: A 10-year Review.

BACKGROUND: Upper extremity friction burn due to powered home equipment is a growing problem in the pediatric population. The purpose of this study was to review the etiology, presentation, characteristics, and treatment of this particular type of pediatric mechanism of injury. METHODS: A retrospective chart review using International Classification of Diseases, version 9, codes for patients treated at a large tertiary care, free-standing children's hospital was performed to identify all patients presenting with an upper extremity friction burn from 2003 to 2012. RESULTS: Sixty-nine patients sustained upper extremity friction burns. The average age at the time of injury was 3.3 years (range, 0.7-10.6) with presentation to our center occurring 16.6 days (range, 0-365 days) following injury. Mean follow-up was 23.3 months (range, 2-104). Mechanism of injury included treadmills (n = 63) and vacuum cleaners (n = 6). Twenty-eight operations were performed on 21 patients (30%). All patients requiring a surgical intervention sustained injury via treadmill mechanism (P = 0.0001). Unlike treadmill burns, vacuum cleaner injuries affected the dorsal hand or a single digit (P = 0.00004). Scar hyperpigmentation was more prevalent in these patients compared with the treadmill group (P = 0.003). All vacuum-induced burn patients had full range of motion and function with conservative treatment alone, whereas only 55.6% of treadmill burn patients had full recovery of range of motion and 50.8% recovery of full hand function. CONCLUSIONS: Friction burns from vacuum cleaners are less prevalent, have different injury patterns, and can be treated conservatively with excellent functional outcomes. Treadmill friction burns result in more significant injury and risk for dysfunction, requiring surgical intervention.

Solitary orbital infantile myofibroma: a case report and review of the literature.

Infantile myofibromatosis is a benign condition involving the skin, viscera, or bone that presents rarely in the orbit where rapid growth and bony destruction can mimic malignant tumors. Lesions can be solitary or scattered throughout the body. Growth in viscera such as the lungs can lead to death. Herein, we present a case of a solitary orbital myofibroma in a young child.

Evaluating the Need for Routine Admission following Primary Cleft Palate Repair: An Analysis of 100 Consecutive Cases.

BACKGROUND: Routine admission following primary cleft palate repair is the standard of care at most institutions. Insurance companies have demonstrated increasing resistance to hospitalization longer than a "short stay"(23 hour) observation period following palatoplasty. The purpose of this study was to identify factors related to length of stay following palate repair. METHODS: Retrospective chart review was conducted for 100 consecutive patients undergoing primary cleft palate repair from May 2009 to February 2013. Demographic and perioperative data were collected and two-sample Student t-test, univariate and multivariable linear regression models were performed to assess for correlation with longer length of stay. RESULTS: Mean age at the time of surgery was 12.6 months. Median length of stay was 39 hours; all 100 patients had >23 hours length of stay. Seventy-three percent of patients required intravenous fluids greater than 23 hours after admission. Postoperative intravenous narcotics were required in 92 percent of patients after transfer to the post-surgical floor, and the last dose was given on average 19.8 hours after completion of surgery. There were 13 patients who required postoperative supplemental oxygen for greater than 23 hours following admission. Multivariable predictors of increased length of stay included female gender, syndromic diagnosis, longer operative and anesthetic durations, longer time to postoperative oral intake, and lower dose of postoperative intravenous narcotic. CONCLUSIONS: Factors identified in association with increased length of stay may guide opportunities for reducing postoperative hospitalization; however, these findings would oppose the safety of routine ambulatory surgery or short-stay observation following primary cleft palate repair. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

Progressive postnatal pansynostosis: an insidious and pernicious form of craniosynostosis.

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.